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Aim: The significance of lymphadenectomy and its indications in patients with inguinal lymph node metastasis (ILNM) of anorectal adenocarcinoma is unclear. This study aimed to clarify the surgical outcomes and prognostic factors of inguinal lymphadenectomy for ILNM., Method: This study included patients who underwent surgical resection for ILNM of rectal or anal canal adenocarcinoma with pathologically positive metastases between 1997 and 2011 at 20 participating centres in the Study Group for Inguinal Lymph Node Metastasis from Colorectal Cancer organized by the Japanese Society for Cancer of the Colon and Rectum. Clinicopathological characteristics and short- and long-term postoperative outcomes were retrospectively analysed., Results: In total, 107 patients were included. The primary tumour was in the rectum in 57 patients (53.3%) and in the anal canal in 50 (46.7%). The median number of ILNMs was 2.34. Postoperative complications of Clavien-Dindo Grade III or higher were observed in five patients. The 5-year overall survival rate was 38.8%. Multivariate analysis identified undifferentiated histological type (P < 0.001), pathological venous invasion (P = 0.01) and pathological primary tumour depth T0-2 (P = 0.01) as independent prognostic factors for poor overall survival., Conclusion: The 5-year overall survival after inguinal lymph node dissection was acceptable, and it warrants consideration in more patients. Further larger-scale studies are needed in order to clarify the surgical indications., (© 2024 Association of Coloproctology of Great Britain and Ireland.)

Background: In transitioning from the 7th edition of the tumor-node-metastasis classification (TNM-7) to the 8th edition (TNM-8), colorectal cancer with peritoneal metastasis was newly categorized as M1c. In the 9th edition of the Japanese Classification of colorectal, appendiceal, and anal carcinoma (JPC-9), M1c is further subdivided into M1c1 (without other organ involvement) and M1c2 (with other organ involvement). This study aimed to compare the model fit and discriminatory ability of the M category of these three classification systems, as no study to date has made this comparison., Methods: The study population consisted of stage IV colorectal cancer patients who were referred to the National Cancer Center Hospital from 2000 to 2017. The Akaike information criterion (AIC), Harrell's concordance index (C-index), and time-dependent receiver operating characteristic (ROC) curves were used to compare the three classification systems. Subgroup analyses, stratified by initial treatment year, were also performed., Results: According to TNM-8, 670 (55%) patients had M1a, 273 (22%) had M1b, and 279 (23%) had M1c (87 M1c1 and 192 M1c2 using JPC-9) tumors. Among the three classification systems, JPC-9 had the lowest AIC value (JPC-9: 10546.3; TNM-7: 10555.9; TNM-8: 10585.5), highest C-index (JPC-9: 0.608; TNM-7: 0.598; TNM-8: 0.599), and superior time-dependent ROC curves throughout the observation period. Subgroup analyses were consistent with these results., Conclusions: While the revised M category definition did not improve model fit and discriminatory ability from TNM-7 to TNM-8, further subdivision of M1c in JPC-9 improved these parameters. These results support further revisions to M1 subcategories in future editions of the TNM classification system., (© 2021 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background and Aim: Although surveillance colonoscopy is recommended by several guidelines for Crohn's disease (CD), the evidence is insufficient to support the validity of this recommendation. Moreover, the efficacy of surveillance colonoscopy for anorectal cancer remains unclear. Therefore, we performed a systematic review of cancer in patients with CD before considering the proper surveillance methods., Methods: We conducted a systematic review and meta-analysis examining the incidence of intestinal cancer and a literature review to clarify the characteristic features of cancer in CD. We performed the systematic literature review of studies published up to May 2019., Results: Overall, 7344 patients were included in eight studies. The standardized incidence ratios (95% confidence intervals) of colorectal cancer (CRC) and small bowel cancer (SBC) were 2.08 (1.43-3.02) and 22.01 (9.10-53.25), respectively. The prevalence of CRC and SBC was 57/7344 (0.77%) and 17/7344 (0.23%), respectively, during a median follow-up of 12.55 years. Additionally, 54 studies reporting 208 anorectal cancer cases were identified. In patients with anorectal cancer, the prognosis for survival was 2.1 ± 2.3 years, and advanced cancer greater than stage T3 occurred in 46/74 patients (62.1%). Many more reports of anorectal cancer were published in Asia than in Western countries., Conclusion: Although we were unable to state a recommendation for surveillance for SBC, we should perform cancer surveillance for CRC in patients with CD. However, the characteristics of cancer may differ according to geography or race. We must establish proper and effective surveillance methods that are independently suitable to detect these differences., (© 2020 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Background: Patients with Crohn's disease (CD) are generally known to be at an increased risk of cancer. The anorectal area is the most frequent cancer site in Japanese CD patients. However, the risks are not well defined. The aim of this study was to clarify the clinicopathological characteristics of Japanese CD-associated anorectal carcinoma patients and to explore screening methods for the detection of cancer at earlier stages., Methods: A systematic review of case series and reports of Japanese CD-associated anorectal cancer patients published between 1983 and 2016 was conducted., Result: There were 144 cases of cancer arising from anorectal lesions of CD. The median duration from the onset of CD to the cancer diagnosis was approximately 17 (0-39) years. The most prevalent histological type of cancer was mucinous carcinoma (49.3%), and 82.1% of patients were over T3 invasion. There were only 15.6% cases with early stage disease. A total of 82% patients had enhanced symptoms, whereas 56.3% of the early cancer cases had no symptomatic changes. Approximately 90% of cases were diagnosed preoperatively, and almost all early cancer patients were diagnosed with colonoscopy. The 5-year overall survival rate was 35.8%., Conclusion: CD-associated anorectal carcinoma had a poor prognosis due to the advanced stage of the cases and aggressive histological features. As earlier-stage cancer is associated with a better prognosis than advanced stage disease, and it is also typically diagnosed by colonoscopy, surveillance colonoscopy may therefore help to improve the prognosis in cases without any symptomatic changes.

Purpose: Anal adenocarcinoma (AA) represents a rare condition, and little is known about the predictive factors of the outcomes or the optimal TNM staging system for curable AA. Using population-based data, we preliminarily sought to determine the prognostic factors and evaluate the existing T and N staging criteria of AA., Methods: We analyzed the Surveillance, Epidemiology, and End Results 18 database to identify patients 20-80 years old who were diagnosed with AA or rectal adenocarcinoma (RA) and underwent abdominal perineal resection between 2004 and 2012. The difference between Kaplan-Meier survival curves was estimated by a log-rank test. A Cox proportional hazard regression model was used to adjust the effects of other covariates on survival in the propensity score-matched cohort, including age, gender, race, marital status, histology, grade of differentiation, tumor size, number of positive lymph nodes, radiotherapy, and chemotherapy., Results: Compared to patients with RA, patients with AA had a worse CSS after controlling for other covariates (hazard ratio [HR], 1.96; 95% confidence interval [CI], 1.25-3.07; P<0.01). For AA, the increasing tumor size (2-5 cm: HR, 0.62; 95% CI, 0.29-1.32; P>0.05; >5 cm: HR, 1.01; 95% CI, 0.49-2.07; P>0.05) had no significant influence on survival. The number of positive lymph nodes (1-3: HR, 2.93; 95% CI, 1.55-5.53; P<0.01; ≥4: HR, 4.24; 95% CI, 2.08-8.62; P<0.01) significantly influenced survival., Conclusions: AA confers a worse prognosis than RA does. The T staging criteria of anal carcinoma, dominated by tumor size, seem to be invalid for AA, while the number of positive lymph nodes is a prognostic factor., Competing Interests: The authors have declared that no competing interests exist.

Background: The 8th edition of the tumor-node-metastasis (TNM) classification classifies inguinal lymph nodes as regional lymph nodes for anal canal carcinoma but non-regional lymph nodes for rectal carcinoma. This difference might reflect the different prognosis of inguinal lymph node metastasis from anal canal carcinoma and rectal carcinoma. However, long-term outcomes of inguinal lymph node metastasis from rectal or anal canal adenocarcinoma are unclear, which we aimed to investigate in this study., Methods: The study population included 31 consecutive patients with rectal or anal canal adenocarcinoma who underwent inguinal lymph node dissection with curative intent at the National Cancer Center Hospital from 1986 to 2017. Long-term outcomes were assessed and clinicopathologic variables analyzed for prognostic significance., Results: Of the 31 patients, 12 patients had rectal adenocarcinoma and 19 patients had anal canal adenocarcinoma. Synchronous metastasis were observed in 14 patients and metachronous metastasis in 17 patients. After dissection of inguinal lymph node metastasis with curative intent, the 5-year overall survival rate was 55.2%, with 12 patients surviving for more than 5 years. Median survival time was 66.6 months. Multivariate analyses revealed that location of primary tumor (rectum versus anal canal) was not a prognostic factor, whereas lateral lymph node metastasis and histological findings were independent prognostic factors., Conclusion: Given the good prognosis, inguinal lymph node metastasis in patients with rectal or anal canal adenocarcinoma appears to be regional rather than distant. If R0 resection can be achieved, inguinal lymph node dissection may be indicated for these patients.

Primary melanoma involving the anorectal region is rare, accounting for <1% of all melanomas in most Western countries. It characteristically presents at an advanced clinical stage and is associated with poor clinical outcomes. Preliminary reports suggest that response rates to immunotherapies in patients with advanced stage mucosal melanoma are much lower than in cutaneous (or acral) melanoma patients but reasons for this are unclear. Comprehensive characterisation of the immune microenvironment in anorectal melanoma has not previously been performed. A single-institution cohort of 43 primary anorectal melanoma patients was examined to describe clinicopathological features and characterise the immune microenvironment to provide insights into the behaviour of this rare melanoma subtype. The tumours displayed multiple adverse prognostic attributes including deep thickness (median 11.5 mm), ulceration (81%) and high mitotic rate (median 12/mm 2 ). The median overall survival was 24 months and the median recurrence-free survival was 9 months. Tumour-infiltrating lymphocytes (TILs) were absent or mild in most tumours (75%); PD-L1 positive staining (>1% of tumour cells) was present in 44% of cases, however in 86% of positive tumours the percentage of positive cells was ≤10%. Four tumours underwent whole genome sequencing; no ultraviolet signature was identified, and there was a lower mutational load but higher structural chromosomal variant load compared with cutaneous melanomas. Poor responses of anorectal melanomas to immunotherapy may be caused by lower immunogenicity of these tumours as characterised by low mutation burden (and therefore low neoantigenicity), low TILs infiltrates and low PD-L1 expression. Further investigation is required to determine whether TILs and PD-L1 expression predict response to immunotherapy in patients with mucosal melanoma., (Copyright © 2018 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

Background: Because anal and rectal squamous cell carcinomas (R-SCCs and A-SCCs) share a common histology and an excellent response to chemoradiation, we hypothesized that R-SCC and A-SCC may represent a similar biological entity, and location would not affect clinical presentation and prognosis., Methods: Patients diagnosed with R-SCC (n = 2881) and A-SCC (n = 21,854) were identified in the Surveillance, Epidemiology, and End Results database (1998-2013). R-SCCs were staged based on American Joint Committee on Cancer classification for A-SCC, and impact of location was analyzed accordingly., Results: Compared to A-SCC, R-SCCs were more common in females (65% versus 48%, P < 0.001) and older patients (62 versus 56 yrs, P < 0.001). R-SCC presented with more advanced disease than A-SCC: mean size 4.2 versus 3.6 cm; T4 14% versus 5%; nodal involvement 20% versus 15%; and metastases 13% versus 6% (all P < 0.001). In multivariable analysis, R-SCCs and A-SCCs had similar disease-specific survival (DSS) for stages 0, I, and III; however, stage II R-SCC had significantly worse DSS than A-SCCs (P = 0.002). This was due to a greater proportion of T3 (>5 cm) R-SCC tumors (36% versus 27%, P < 0.001), which had a lower DSS than T2 (2-5 cm) tumors. Within T3 and T4 tumors, R-SCCs had lower DSS than A-SCCs., Conclusions: R-SCC presented with higher stages than A-SCC, suggesting a delayed diagnosis. Larger R-SCC (T3-T4) had worse survival compared to T3-4 A-SCC, which may be due to a combination of more advanced disease within-stage as well as the use of less efficacious therapeutic regimens. Therefore, location may represent a significant prognostic factor for SCC of the anorectal region., (Copyright © 2018 Elsevier Inc. All rights reserved.)

To investigate the clinicopathological features and prognostic factors of primary anorectal melanoma, we described the clinical presentations, histopathology, and prognosis of 60 cases in China. Patients' age ranged from 17 to 86 years, with a female-to-male ratio of 2.33:1. The prevalent anatomic site of tumor was the anorectum. The mean tumor thickness was 11.5 mm, and mean tumor size was 2.9 cm. Abdominoperineal resection was performed on 38 (63.3%) patients, whereas wide local excision was performed on 22 (36.7%) patients. Thirty-three (55.0%) patients were pathologically confirmed to have lymph node metastasis, and 9 (15%) patients had clinically distant metastasis at diagnosis. Histologically, epithelioid cell (91.7%) was the predominant cell type, followed by spindle (31.7%), pleomorphic (25.0%), and small round cell (5.0%). Solid sheet, nest, pseudopapillary, and pseudoalveolar growth patterns were noted in 45 (75.0%), 22 (36.7%), 13 (21.7%), and 6 (10.0%) cases, respectively. After a median follow-up of 44 months, the 5-year disease-specific survival rate was 33.3%. Age, tumor size, depth of invasion, tumor thickness, lymphatic metastasis, and lymphovascular and perineural invasion were significantly correlated with survival in univariate analysis. Multivariate analysis revealed that age greater than 70 years and tumor invasion beyond deep muscular layer/sphincter ani externus were independent poor prognostic factors. As the largest single-institution study of anorectal melanoma in an Asian population, we concluded that anorectal melanoma is a rare and lethal malignant neoplasm with morphologic diversity. Large population-based studies are still needed to establish an efficient staging system in evaluation of prognosis and facilitation of treatment for anorectal melanoma patients., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Melanoma metastatic to the large bowel (colon, rectum, and anus) is rarely diagnosed, with more than 95% of large bowel metastases identified post-mortem. The incidence, natural history, and survival rates of patients with large bowel melanoma metastases are poorly documented in the literature., Objective: This study aimed to identify the incidence, clinical characteristics, and survival of patients with large bowel melanoma metastases., Methods: A review was undertaken of all patients with melanoma treated over a 50-year period (1964-2014) at a tertiary referral center. Cases selected for study were those diagnosed with melanoma metastases in the colon, rectum, and anus. Primary colorectal and anal melanomas were excluded. Data were retrieved relating to patient demographics, clinical features, and survival., Results: Of 38,279 patients with primary melanoma, 106 patients (0.3%, mean age 51.0 years [standard deviation 16.3], 64 males) developed large bowel metastases. The median interval between diagnosis of primary melanoma and large bowel metastasis was 62.8 months (range 1-476). The most common symptom was rectal bleeding (29.2%), and the large bowel was the sole site of metastasis in 47.2% of patients. Median survival from diagnosis of large bowel metastasis was 31.7 months (range 1-315), and overall survival at 1, 2, and 5 years was 68.1, 45.9, and 26.5%, respectively., Conclusion: Our study provides insights into melanoma metastatic to the colon, rectum, and anus, which had an incidence of 0.3%. There are potentially long intervals between diagnosis of primary melanoma and large bowel metastasis. The most common symptom was rectal bleeding, although some patients were asymptomatic.

Introduction Mucosal malignant melanoma of the anorectum is a rare and aggressive disease, in which early diagnosis is difficult. The prognosis remains extremely poor, irrespective of the treatment. We share our experience in treating this malignancy at our centre in South India. Methods This study describes a retrospective analysis of 31 cases of anorectal melanoma presented to our centre between January 2001 and December 2013. Results Twenty-two patients (71%) presented with metastasis and had a median overall survival of nine months. None of the 22 patients survived for two years. Nine patients (29%) had curative surgery, in the form of abdominoperineal resection (six patients), abdominoperineal resection with bilateral inguinal node dissection (one patient), abdominoperineal resection with liver resection (one patient) and posterior exenteration (one patient). In patients who underwent curative surgery, the median overall survival was 15 months and disease-free survival was nine months, with a two-year overall survival of 22%. Conclusions Anorectal melanoma is an aggressive disease with a poor prognosis. The majority of patients present with distant metastases. Prognosis depends on stage at presentation. Early diagnosis and surgical resection may improve the overall outcome. Newer modalities such as immunotherapy and targeted therapies such as anti-CTLA4 monoclonal antibody and anti-programmed cell death protein 1 monoclonal antibodies have radically changed the management of mucosal melanoma and may, in the future, improve the overall prognosis of anorectal melanoma.

Background: Squamous cell cancers of the anus are rare GI malignancies for which neoadjuvant chemoradiation is the first-line treatment for nonmetastatic disease. Squamous cancers of the rectum are far less common, and it is unclear to what degree chemoradiotherapy improves their outcomes., Objective: The purpose of this study was to compare stage-specific survival for anal and rectal squamous cancers stratified by treatment approach., Design: This was a retrospective cohort study., Settings: The study was conducted at Commission on Cancer designated hospitals., Patients: Patients (2006-2012) identified in the National Cancer Database with pretreatment clinical stage I to III cancers who underwent chemoradiotherapy, with and without subsequent salvage surgical resection (low anterior resection or abdominoperineal resection), ≥12 weeks after chemoradiotherapy were included in the study., Main Outcome Measures: Overall survival and the need for salvage surgery were measured., Results: Anal cancers (n = 11,224) typically presented with stage II (45.7%) or III (36.3%) disease, whereas rectal cancer stages (n = 1049) were more evenly distributed (p < 0.001). More patients with rectal cancer underwent low anterior or abdominoperineal resections 12 weeks or later after chemoradiotherapy versus those undergoing abdominoperineal resection for anal cancer (3.8% versus 1.2%; p < 0.001). Stage I and II rectal cancer was associated with poorer survival compared with anal cancer (stage I, p = 0.017; stage II, p < 0.001); survival was similar for stage III disease. Salvage surgery for anal cancer was associated with worse survival for stage I to III cancers; salvage surgery did not significantly affect survival for rectal cancer., Limitations: This was a retrospective study without cancer-specific survival measures., Conclusions: Squamous rectal cancers are associated with significantly worse survival than squamous cancers of the anus for clinical stage I and II disease. Despite both cancers exhibiting squamous histology, rectal cancers may be less radiosensitive than anal cancers, as suggested by the greater incidence of salvage surgery that does not appear to significantly improve overall survival. See Video Abstract at http://links.lww.com/DCR/A422.

Purpose: Anorectal gastrointestinal stromal tumors (GISTs) are exceedingly rare, and management remains controversial in regard to local resection (LR) and preoperative chemotherapy., Methods: The National Cancer Data Base was queried from 1998 to 2012 for cases of GIST resection in the rectum or anus. Patient demographics, type of surgery (LR vs. radical excision [RE]), short-term outcomes, and overall survival (OS) were analyzed. Preoperative chemotherapy was recorded following the US FDA approval of imatinib in 2002., Results: Overall, 333 patients with resection of anorectal GISTs were included. Mean age at presentation was 62.3 years (range 22-90), and median tumor size was 4.0 cm (interquartile range 2.2-7.0). Five-year OS for all patients was 77.6%. In a multivariable survival analysis, only age and tumor size >5 cm (hazard ratio 2.48, 95% confidence interval 1.50-4.01; p = 0.004) were associated with increased mortality. One hundred and sixty-three (49.0%) patients underwent LR, compared with 158 (47.4%) who underwent RE. For tumors smaller than 5 cm, no difference in 5-year survival by surgical approach was observed (LR 82.3% vs. RE 82.6%; p = 0.71). Fifty-nine patients (17.7%) received preoperative chemotherapy; for patients undergoing RE with tumors >5 cm, there was decreased mortality in the group who received preoperative chemotherapy (5-year OS with chemotherapy 79.2% vs. no chemotherapy 51.2%; p = 0.03)., Conclusions: Size is the most important determinant in survival following resection. Local excision is common, with resection split between LR and RE. For smaller tumors, LR may be adequate therapy. Preoperative chemotherapy may result in improved survival for large tumors treated with radical resection, but the data are imperfect.

Background: Anorectal malignant melanomas (ARMM) are rare tumors, characterized by an early lymphatic spread and distant metastasis, resulting in an extremely poor overall survival. The objective of this study was to determine the pattern of regional lymph node metastasis (LNM) by computed tomography (CT) and 18F-FDG-PET/CT in patients undergoing abdominoperineal resection (APR) and its impact on oncologic outcome., Methods: A retrospective analysis of six consecutive patients who underwent APR due to primary ARMM was performed. Patients were staged by CT and PET/CT., Results: Four out of six patients had preoperative LNM involvement (two patients inguinal and perirectal, one iliacal, one perirectal), with two of them presenting with distant metastases additionally. Inguinal/iliacal LNM in two patients as well as liver metastasis in one patient was seen in PET/CT and missed by CT. The three patients with initial inguinal/iliacal LNM died during the observation period (overall survival: 10 (6-18) months). The three patients without inguinal/iliacal LNM involvement are currently alive, one patient showing a slowly progressive disease since 5 years, and two patients are tumor-free since 8.5 and 1.5 years (the patients had initial perirectal LNM)., Conclusions: In ARMM, PET/CT is superior to CT in detection of LNM and distant metastasis. APR is possibly a curative approach if the PET/CT shows exclusively perirectal LNM despite locally advanced tumor growth.

Background: Anal adenocarcinoma (AA) represents 5% to 10% of anal cancer. Little is known about its natural history and prognosis. Using population-based data, we defined the outcomes of AA relative to other anorectal malignancies., Patients and Methods: We analyzed the Surveillance, Epidemiology, and End Results 18 database to identify patients ≥ 18 years old with AA, squamous cell carcinoma of the anus (SCCA), and rectal adenocarcinoma (RA) diagnosed between 1990 and 2011. Median overall survival (OS), 1-year, 3-year, 5-year, and 10-year OS were computed using actuarial methods. The log rank test was used to estimate the difference between Kaplan-Meier survival curves. A Cox proportional hazard regression model was used to adjust the effects of other covariates on survival, including age, year diagnosed, sex, stage, surgery, and radiation., Results: Of 57,369 cases, 0.8% (n = 462) were patients with AA, 87.8% (n = 50,382) were patients with RA, and 11.4% (n = 6525) were patients with SCCA. The median age for AA was 69 years (range, 20-96 years), 66 years (range, 18-103 years) for RA, and 66 years (range, 14-104 years) for SCCA. The median OS was significantly lower for AA (33 months), compared with SCCA (118 months) and RA (68 months) (P < .01). In multivariate analysis, AA had a worse prognosis compared with SCCA (hazard ratio [HR], 0.66; 95% confidence interval [CI], 0.59-0.75; P < .01) and RA (HR, 0.68; 95% CI, 0.61-0.77; P < .01), after adjusting for age, sex, race, stage, grade, radiation, and surgery. There was a strong trend for improved survival among patients who received radical surgery (HR, 0.71; 95% CI, 0.51-1.00; P = .05)., Conclusion: AA confers a significantly worse prognosis than SCCA and RA., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: To determine whether the extent of surgery is associated with survival in anorectal malignant melanoma (ARMM)., Background: ARMM is a rare and highly malignant neoplasm with unfavorable prognosis. The optimal surgical management, abdominoperineal resection (APR) or local excision (LE), has been long debated, but conclusive evidence has not been obtained., Methods: A comprehensive electronic literature search was performed to identify studies evaluating survival between APR and LE for ARMM. The main outcome measures were overall survival, relapse-free survival, and local recurrence. A meta-analysis was performed using the random-effects models to calculate the odds ratios (ORs) and 95% confidence intervals (CIs)., Results: Thirty-one studies, with a total of 1006 patients [544 (54.1%) APR and 462 (45.9%) LE], were included. Meta-analyses showed that overall survival (OR, 1.14; 95% CI, 0.74-1.76; P = 0.54) and relapse-free survival (OR, 0.95; 95% CI, 0.43-2.09; P = 0.89) did not differ significantly between the APR and LE groups. APR significantly reduced local recurrence compared with LE (OR, 0.18; 95% CI, 0.09-0.36; P < 0.00001)., Conclusions: Although several limitations, such as inclusion of only retrospective studies with relatively small sample size and selection biases for surgical procedure, are involved, this meta-analysis suggested that APR has no survival benefit; however, APR confers better local control than LE. Given that local failures after LE could be managed by salvage surgery, minimizing morbidity and maximizing quality of life should be the focus in surgical treatment of ARMM.

Anorectal melanoma is an uncommon cancer with a poor prognosis. We aim to describe the clinical presentation, treatment and outcome of patients with anorectal melanoma in our center. Retrospective study of patients with anorectal melanoma treated between 2000 and 2011 at a cancer center in Lisbon. Ten patients were identified, eight females and two males, with median age 70.5 years (32-79). Symptoms at presentation were rectal bleeding (8), anal pain (4) and discomfort (3). Tumor location was anal (6), rectal (3) and anorectal transition (2). Seven patients had surgery: abdomino-pelvic resection (5) and local resection (2). Among the two patients who underwent local resection, one was an incidental finding in a hemorrhoidectomy specimen. This patient had further adjuvant chemotherapy (dacarbazine). Three patients had distant metastasis at diagnosis, one had chemotherapy and two had best supportive care. Six of the seven operated patients relapsed in a median time of 5.4 months: distant metastasis (4), local recurrence (1), both (1). The two local relapse patients had surgical widening of resection margins (1) and radiotherapy (2). One-year survival was 30 %; 3-year survival was 20 %. Anorectal melanoma has a poor prognosis due to advanced disease at presentation and aggressive course, with relapse in almost all operated patients. Treatment guidelines have not been established due to the lack of randomized studies. However, recent studies show that sphincter-sparing surgical procedures along with low dose intensity radiotherapy seem to achieve a local control similar to abdomino-pelvic resection. No systemic therapy is considered standard of care for advanced disease, and regimens are extrapolated from cutaneous melanoma experience.

Introduction and Aim: Neuroendocrine carcinomas (NEC) of the large intestine are rare and aggressive neoplasms. This study was designed to review the experience at a single institution and analyze the outcomes to improve our understanding of these tumors., Methods: The patients with NEC (high-grade neuroendocrine tumor) of the colon, rectum, and anal canal were identified from June 1993 to April 2011. Clinical features studied were patient demographics, presenting symptoms, tumor location, tumor stage, treatment status and length of follow-up., Results: Twenty-five patients were identified. Stages of the diseases were I (n=5), II (n=1), III (n=10) and IV (n=9). Locations of the tumors were: colon (36%); rectum (28%) and anal canal (36%). Rectal bleeding (36%) and pain (32%) were the most common symptoms but 16% of patients were asymptomatic. Among the patients with local or locally advanced disease, only five patients had surgery alone. The remainder of the patients underwent chemotherapy with/without radiation. Mean follow-up was 33.7 ± 8.4 months after diagnosis. NEC of the large bowel and anal canal has very distinctive pathologic features and a very poor prognosis. More than one third of the patients with NEC had metastatic disease at the time of diagnosis. When these tumors are in an advanced stage none of the treatment modalities impact on survival., Conclusion: The present study showed the poor prognosis of these rare tumors., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Purpose: Anorectal malignant melanoma (AMM) is a rare tumor with a poor prognosis. The aim of this study was to investigate the clinicopathological characteristics and treatment outcomes in patients with AMM., Methods: The study included 21 patients diagnosed with AMM between 2000 and 2010 that were evaluated with regard to age, sex, disease stage, treatment modality, and survival. Stage I, II, and III were defined as localized primary malignant melanoma, regional lymph node metastasis, and distant metastasis, respectively., Results: In all, 12 (57%) patients were female and 9 (43%) were male ; median age was 61 years (range : 30-84 years). Among the 21 patients, 7 (47%) underwent abdominoperineal resection and 8 (53%) were treated using wide local excision. Four (19%) patients were classified as stage I, 10 (48%) as stage II, and 7 (33%) patients as stage III. In total, 10 patients received adjuvant therapy. Median overall and progression-free survival was 12 and 9 months, respectively. The 1-year and 5-year overall survival estimates were 59% and 42%, and progression free survival were 49% and 7%, respectively. Patients aged > 60 years (P = 0.145), female patients (P = 0.076), patients with localized disease (P = 0.045), patients that underwent wide local excision (P = 0.619), and patients that received adjuvant therapy (P = 0.962) had longer survival., Conclusions: The prognosis of AMM remains very poor and disease stage is the only predictor of survival. Abdominoperineal resection does not confer an advantage, in terms of survival, in patients with AMM.

Aim: Anorectal melanoma is a rare neoplasm with poor prognosis. The aim of this study was to investigate what clinicopathological factors predict lymph node metastases and to investigate their association with survival., Method: Patients undergoing surgery with curative intent for primary anorectal melanoma in Fudan University Shanghai Cancer Center between 1989 and 2011 were studied retrospectively. The associations between clinicopathological factors and lymph node metastases and prognosis were determined., Results: Forty-three patients underwent a potentially curative resection with a median follow-up of 20 months; the 5-year overall survival rate was 29.6% with median overall survival of 28 months. Tumour diameter > 3 cm was associated with mesorectal and mesenteric lymph node metastases (P = 0.013). Perineural invasion (hazard ratio 5.683; 95% CI 1.978-16.328; P = 0.001) was the only factor that independently predicted survival., Conclusion: Tumour diameter was associated with mesorectal and mesenteric lymph node metastases; therefore, wide local excision may not be appropriate for surgery with curative intent for patients with a tumour diameter ≥ 3 cm. Perineural invasion was an important prognostic factor for anorectal melanoma., (Colorectal Disease © 2013 The Association of Coloproctology of Great Britain and Ireland.)

Background: Anatomic site is a predictive factor in subtypes of cutaneous and mucosal melanoma., Objective: The aim of this study was to examine the clinical relevance of location of origin of anorectal melanoma as a prognostic factor., Design: With the use of a prospectively maintained database, clinical characteristics, management, and outcomes were compared according to the site of origin. SETTINGS, PATIENTS, INTERVENTIONS: A retrospective review was conducted of patients diagnosed with anorectal melanoma from 1994 to 2010. Tumors were defined as anal, anorectal, or rectal melanoma according to their anatomic relationship to the dentate line., Main Outcome Measures: Clinicopathologic factors were compared by χ2 test. Time-to-event analysis was performed by Kaplan-Meier analysis., Results: Of the 96 patients included (41 with anal melanoma, 32 with anorectal melanoma, 23 with rectal melanoma), patients with rectal and anorectal mucosal melanoma had advanced primary tumors (median Breslow thickness, 12 mm and 8 mm, p = 0.002), whereas anal lesions could be found at earlier depths (median thickness, 6.5 mm). Patients with anal tumors more commonly underwent transanal excision (p < 0.02) and sentinel lymph node biopsy (p = 0.004) versus anorectal and rectal tumors. Patterns of recurrence were also distinct; nearly two-thirds of anorectal and rectal tumors recurred systemically, whereas anal melanoma more often recurred within the lymph nodes first (63%; p < 0.02). Recurrence occurred in 24 (59%) patients with anal tumors, 23 (72%) patients with anorectal tumors, and 16 (70%) patients with rectal tumors. Median overall survival was 22 months for anal melanoma, 28 months for anorectal melanoma, and 27 months for rectal melanoma. Recurrence and survival were not statistically different between the groups., Limitations: This study is limited by small sample size and its retrospective nature., Conclusions: This study represents the only series describing the outcomes of anorectal melanoma by anatomic location. Lesions at or proximal to the dentate line present with more advanced disease, possibly related to a delay in diagnosis. Lesions distal to the dentate line more commonly recur within lymph nodes, which may represent differences in nodal drainage. Irrespective of location, the long-term prognosis remains poor for all cases of anorectal melanoma.

Background/aims: To evaluate outcome and prognostic factors of patients with anorectal melanoma., Methodology: Twenty-two consecutive patients with anorectal melanoma received operation from 1993 through 2011 were reviewed. The definitions of stage I, II and III are local disease, locoregional lymphadenopathy and metastatic disease, respectively., Results: The patients included 8 men and 14 women, aged from 36 to 83 years (mean, 58.4 years). At the end of the follow-up period, 19 patients died of disease and only 3 patients were alive with disease. Only two patients were alive longer than 5 years after operation. For stage I and II tumors that underwent clinical curative resection (n=17), stage II (p=0.04), tumor size >3 cm (p=0.008) and invasion depth to muscle (p=0.021) all showed poorer prognosis in overall survival. Though wide local excision (WLE) were performed in the patients with earlier tumors, there was no statistical difference in overall (p=0.063) and disease-free survival (p=0.333) between WLE and radical surgery. Furthermore, patients with WLE had more chance of local recurrence than radical surgery (6/7 vs. 3/10, p=0.050). Four salvage radical operations could be performed after local recurrence in WLE group., Conclusions: WLE increases the chance of local recurrence more than radical surgery. Care should be taken to avoid microseeding during performed WLE.

Small cell carcinomas represent <1% of colorectal/anal carcinomas and have a poor prognosis. Anorectal squamous cell carcinomas are often associated with high-risk human papillomavirus (HPV) infection, similar to squamous and small cell carcinomas of the uterine cervix. In HPV infection, the oncoprotein E7 inactivates the tumor suppressor Rb, leading to p16 upregulation; however, in small cell carcinomas, the Rb pathway is often blocked by other mechanisms; thus, increased p16 may not indicate HPV infection. P16 immunohistochemistry (IHC) may have a limited role in evaluating small cell carcinomas for HPV infection. Formalin-fixed, paraffin-embedded tissue sections of previously diagnosed small cell carcinomas of the anus (n=5) and rectum (n=11) were subjected to IHC for p16, CDX2, and p63, followed by in situ hybridization for high-risk HPV. All (100%) cases of anal and rectal small cell carcinomas were positive for p16, and 100% of anal and 82% of rectal small cell carcinomas were positive for high-risk HPV. The majority of cases showed low or very low HPV copy numbers. In 6 cases, HPV was detected only by using the HPV-16 genotype-specific assay detecting very low copy numbers (1 to 2 viral copies). The majority of tumors expressed p63, which was more pronounced in the anal tumors. CDX2 expression was observed predominantly in rectal tumors. High-risk HPV can be detected using in situ hybridization in the majority of anorectal small cell carcinomas, which are uniformly p16 positive by IHC. HPV-targeted therapy could result in better control of these aggressive neoplasms.

Background: Primary anorectal melanoma is a rare and aggressive disease. It accounts for 0.5% of all rectal tumors. They are very agressive tumors with poor prognosis. The aim of this study is to report the clinical and evolutionary profile and therapeutical approach of these tumors., Patients and Methods: A retrospective study of 17 patients with anorectal melanoma diagnosed between January 1998 and December 2007 was performed. The signs and symptoms, diagnostic study, and surgical and medical treatments were analyzed., Results: The average age was 58 years. Sex ratio was 12 men per five women. Patients had symptoms present for an average of 6 months. The most common symptom was rectal bleeding. According to Slingluff classification, five patients had stage I (localized tumor), four cases had stage II (regional nodes metastasis), and eight cases had stage III (distant metastasis). Seven patients have radical surgery. Only two patients received adjuvant immunotherapy. Eight patients received palliative chemotherapy based on dacarbazine or cisplatinum. The median survival was 8 months., Conclusion: Prognosis of anorectal melanoma is still very poor. However, some patients when treated by radical resection may experience long-term survival. The use of adjuvant immunotherapy needs large collaborative studies in view of the rarity of the tumor.

Objective: To study the outcomes of solid tumors of the colon and rectum in pediatric patients., Methods: The Surveillance, Epidemiology, and End Results (SEER) database (1973-2005) was queried for patients less than 20 y of age., Results: Overall, 270 patients with malignant tumors of the lower gastrointestinal tract were identified. The annual incidence was 1.04 cases per million in 2005. Overall, equal distribution between boys (50.7%) and girls (49.3%) was observed. The majority of tumors arose in adolescents (68.1% were older than 15 y). Tumors were more commonly seen in white (77.8%) and non-Hispanic (78.9%) patients. Tumors were identified in the right colon (45.9%), transverse colon (9.3%), left colon (20.4%), rectum (15.2%), and anal canal (1.1%). The most common histology of these tumors was adenocarcinoma (35.6%), followed by carcinoid (34.1%). Disease specific 5- and 10-y-survival for the entire cohort was 61.0% and 57.9%, respectively. Multivariate analysis of the cohort identified tumor stage (HR 8.39, P < 0.001 for distant disease), tumor type (signet ring HR 2.12, P = 0.025, and carcinoid HR = 0.14, P = 0.001), and surgical resection (no surgery HR 2.98, P = 0.010) as independent predictors of worse outcome., Conclusion: In the pediatric population, solid tumors of the colon and rectum occur more frequently in the right side of the colon in teenagers. Surgical resection significantly improves survival for most pediatric tumors of the lower gastrointestinal tract., (Copyright 2010 Elsevier Inc. All rights reserved.)

Background: Anorectal melanoma is rare and surgery is the recommended primary treatment. There has been some debate whether abdominoperineal resection (APR) or local excision is most appropriate. The aim of this study was to provide a population-based analysis of symptoms, treatment and outcome., Methods: From the Swedish National Cancer Registry, 251 patients with anorectal melanoma were identified from 1960 to 1999. Medical reports were collected and reviewed retrospectively. R0 resection was defined by clear macroscopic margins and a pathology report showing a margin greater than 10 mm. Survival was compared with the log rank test, and Cox multivariable analysis was performed., Results: APR and local excision was performed in 66 and 86 patients respectively. Median survival after surgery was 14 months, with no statistically significant difference between the two groups. Seventy-two patients in whom an R0 resection was achieved, irrespective of approach, had a significantly better overall 5-year survival rate than patients with involved margins (19 versus 6 per cent; P < 0.001). Multivariable analysis showed resection status and tumour stage to be independent prognostic variables., Conclusion: Both APR and LE seem appropriate for anorectal melanoma provided clear margins can be achieved; prognosis is poor regardless of surgical approach., (Copyright 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.)

Aims: This study describes the experience of the National Cancer Institute of Milano in the treatment of anorectal melanoma over the last 32 years., Methods: The influence of different surgical approaches on local care and final outcome was investigated on 40 completely evaluable patients, followed for a median follow-up time of 75 months. The analysis was carried out by calculating and comparing overall survival, disease-free survival and cumulative incidence curves of disease recurrence., Results: Thirty-one patients underwent radical surgery: nine abdominoperineal resections, four total rectal resections and coloendoanal anastomosis, and 18 local excisions. The remaining nine patients received palliative treatments. Median overall survival time for patients receiving non-radical treatments was poor: only 6 months. However, even when a radical surgery was undergone, the prognosis of patients with anal melanoma remains dismal. Local relapse incidence was 45.8% for the limited surgery group, but non-existent for the extended-surgery group (p = 0.007). However, the median disease-free survival time was 7 and 9 months for patients receiving limited or major surgery (p = 0.97). Overall survival was 17 months, irrespective of the adopted surgery., Conclusion: Prognosis of anal melanoma remains poor. Final outcome is not influenced by modality of surgery. A limited but radical excision can be considered whenever possible while a major demolitive surgery should be applied only for therapy of advanced or bulky lesions.

Introduction: Anorectal malignant melanoma (AMM) is a relatively rare disease. Because of its poor prognosis, the optimal surgical treatment for AMM is still controversial and difficult to determine. In this paper, we report five cases of AMM that have been treated by surgery and/or other methods at Shinshu University Hospital within the last decade. We also review the present five cases along with 74 other Japanese cases reported between 1997 and 2006 and discuss the role of surgery in the treatment of AMM., Results and Discussion: Among our AMM patients, two who underwent radical abdominoperineal resection had long survival, while the other three patients who underwent palliative surgery had a poor outcome. On the total of 79 AMM patients, those who underwent curative surgery had a better outcome than those who underwent palliative surgery (p < 0.0001). Furthermore, the outcome of AMM patients at stages 0 and I was better than that of AMM patients at stages II, III, and IV (p < 0.0001). There was no significant difference in survival between AMM patients with and without adjuvant chemotherapy., Conclusion: In conclusion, AMM patients treated by curative surgery can expect long-term survival, although the usefulness of adjuvant chemotherapy for AMM patients is controversial.

Objective: Anorectal melanoma is a rare, highly malignant tumour with a poor 5 year survival of 10%. Most anorectal melanomas have gross and/or histologic pigmentation, however about 30% of anorectal melanomas are amelanotic., Method: We report three cases of amelanotic anorectal melanomas and integrate our data with six case reports of amelanotic malignant melanoma from the literature. Further we compare clinicopathological data and clinical outcome with large series of anorectal melanomas (both, amelanotic and pigmentated)., Results: There were seven females and two males, of median age 62 years (range: 45-75 years). Rectal bleeding was the leading symptom in all cases with a mean duration of 4 months before diagnosis. Eight of nine patients developed distant metastases. Median survival was 14 months (range: 3-60 months). A tumour thickness of < 4 mm was correlated with long-term disease-free survival, whereas tumour thickness of 4 mm or more was correlated with systemic recurrence., Conclusion: Early diagnosis is key for efficient treatment and improved survival rate for patients with this unusual variant of melanoma. There is no difference in terms of age, time of diagnosis, stage and survival between pigmented and amelanotic anorectal melanoma.

Background: Wide local excision (WLE) of anorectal melanoma is associated with a high incidence of local recurrence. There is a paucity of literature on adjuvant radiation in this malignancy., Aim: To identify the optimal method of local treatment in anorectal melanoma., Settings and Design: Retrospective study in a tertiary cancer centre., Materials and Methods: Records of 63 patients who presented between 1980 and 2004 were reviewed., Results: Of the 63 patients, 18 were treated by either surgery with or without adjuvant radiation, or by radiation alone. The remaining had advanced disease and were offered only symptomatic treatment. The median overall survival in stage I patients was 12 months, while it was seven and four months in those with stage II and III disease respectively. The median survival in patients treated by WLE with adjuvant radiation (RT), WLE alone or Abdominoperineal resection (APR) was 34, 12 and 10 months respectively. Patients in whom the disease was confined to the mucosa had a better median overall survival than those in whom it had infiltrated beyond the mucosa (102 vs 11 months). The pattern of recurrence following WLE with adjuvant RT or APR was similar. None of the patients who received adjuvant RT after wide excision had a local or nodal recurrence., Conclusion: Local treatment of anorectal melanoma should be individualized. WLE with adjuvant radiation seems to offer good locoregional control without reducing the survival and may be an option of treatment for patients with small, superficial anorectal melanoma. However, APR should be offered for patients with locally advanced disease or as a salvage following recurrence.

The aim of this study was to investigate the effects of tumour and patient characteristics on trends in the survival of patients with cancer of the anus or rectum in England and Wales. A total of 132,542 adults (15-99 years) who were diagnosed during the 14 years 1986-1999 were followed up to 2001 through the National Health Service Central Register. Relative survival up to 5 years after diagnosis was estimated, using deprivation-specific life tables. Generalised linear models were used to estimate relative excess risks of death, adjusted for patient and tumour characteristics. The results showed that 5-year relative survival was higher in women, younger patients and more affluent patients, and higher for anal cancer than rectal cancer. Survival improved by more than 10% from the late 1980s (around 38%) to the late 1990s (49%). This trend was not explained by changes in the distribution of age, anatomical site, morphology or deprivation. The trend was more marked in younger and more affluent patients, and for adenocarcinoma and epidermoid carcinoma than for tumours with other morphology. The inequality in survival between affluent and deprived patients widened. It is concluded that improvements in survival may reflect improvements in disease stage, diagnostic technique or treatment. Which of these factors contribute to the widening socioeconomic inequalities in survival remains to be elucidated.

Objective: Colorectal cancer mortality predictions for Ireland are presented for all ages and for the age group 0-64 years., Methods: Log and log-linear Poisson regression modelling was fitted to colorectal cancer mortality data for each year between 1950 and 2002 to predict average European age standardized mortality rates (EASMRs) per 100,000 person years (100,000 PY(-1)) and average numbers of deaths., Results: EASMRs for colon cancer are predicted to fall to 17.3 100,000 PY(-1) (95%PI 14.4, 20.2) in men and to 8.4 100,000 PY(-1) (95%PI 6.9, 10.2) in women by 2013-17. EASMRs for anorectal cancer are also predicted to fall to 8.0 100,000 PY(-1) (95%PI 6.6, 9.5) in men and to 3.0 100,000 PY(-1) (95%PI 2.3, 3.8) in women by this time. Truncated EASMRs for colon cancer for those aged between 0 and 64 years are predicted over the next 15 years to fall from their current levels to 4.4 100,000 PY(-1) (95%PI 3.0, 5.8) in men and to 2.3 100,000 PY(-1) (95%PI 1.5, 3.2) in women. For anorectal cancer, truncated EASMRs for those aged between 0 and 64 years are also predicted over the next 15 years to fall from their current levels to 2.9 100,000 PY(-1) (95%PI 2.0, 3.8) in men and to 0.9 100,000 PY(-1) (95%PI 0.4, 1.3) in women., Conclusions: Ireland currently has one of the highest colorectal mortality rates in the developed world but recent downward trends, the advent of new screening tests and well targeted public health promotion programmes may lead to further reductions.

Background and Aims: This study compared outcome following the two common surgical procedures for anorectal melanoma: wide local excision and abdominal perineal resection. We also examined the utility of endoluminal ultrasound to guide therapy., Patients and Methods: Records of 19 patients surgically treated at our institution were studied. In addition to type of surgical procedure, we noted age, metastatic disease spread, sphincter involvement, tumor size and thickness, and mode of diagnosis. Survival after diagnosis and after recurrence of disease were also recorded. Ultrasound was used in seven, with the lesion delineated in six (all had therapy guided by the ultrasound). Regarding surgery ten had wide local excision, seven had abdominal perineal resection, and two had other procedures., Results: The most common sites of recurrence were distant in 31.6% and regional lymph nodes in 26.3%. Mean survival after recurrence was 13 months (range 5-29). Two patients who had wide local excision are disease free and alive 135 and 29 months after diagnosis. Neither surgical treatment conferred obvious benefit on survival., Conclusion: Ultrasound can guide management by delineating lesions amendable to wide local excision. Since the mortality rate is high, wide local excision offers the advantage of avoiding a permanent colostomy and should be considered the procedure of choice when excision is feasible.

Background: Posteriorly fixed anorectal cancer is often considered incurable, but may be resectable using transsacral approaches., Methods: We reviewed 45 patients undergoing transsacral exenteration for this problem since 1983 to determine outcome of such surgery., Results: The group consisted of 38 men and 7 women; 17 had primary tumors, 28 had recurrent cancer: local excision, 1; low anterior resection, 11; or abdominoperineal resection, 16. Thirty-nine had prior XRT. Operative mortality was 4%. Severe pain was relieved in 16 of 22 (72%) patients. Crude recurrence rates are local 22%, local plus distant 11%, distant 16%. Five-year disease-free survival is 31% for primary disease, 32% for recurrence. Median survival is abdominoperineal resection recurrence, 24 months; primary cancer, 30 months; low anterior resection recurrence, 37 months., Conclusions: Transsacral exenteration relieved pain in 70% of patients with fixed anorectal cancer, and led to long-term survival in 31% to 32%. Prognosis trended toward benefit for recurrence after low anterior resection.

Background: In order to individualize the therapy in patients with anal cancer, we evaluated the applicability of the sentinel lymph node (SLN) concept for the staging of inguinal lymph nodes in these patients., Patients and Method: SLN mapping using the radiocolloid technique was performed in 12 patients with histopathologically proven anal cancer. Mean age of the 4 male and 8 female patients was 62 years (range: 37-83 years). All patients underwent injection of (99m)Tc-colloid (Nanocis) in 4 portions around the tumor followed by scintigraphy after 17 h and selective lymph node biopsy in case of nuclide enrichment. The nuclide-enriched lymph node was intraoperatively identified by a hand-held gamma-camera. Histopathological assessment of the harvested SLNs included serial sections and immunohistochemical staining., Results: Enrichment of radiocolloid in lymph nodes was seen in 10 of the 12 patients (detection rate: 83%). SLN biopsy was performed in 9 patients, one patient refused the SLN biopsy (SLNB). 4 patients revealed tumor-infiltrated sentinel lymph nodes including one patient with bilateral biopsy, who showed metastases unilaterally. The remaining 5 patients had no evidence of metastases in the excised SLNs., Conclusion: It is feasible to evaluate the nodal status of the groin in patients with anal cancer using the radiocolloid technique. Preliminary results indicate a refined diagnostic work-up for anal cancer patients, potentially improving the results of clinical and sonographical examinations. Further application of the method may lead to an individualized treatment of patients with anal cancer., (Copyright 2003 S. Karger GmbH, Freiburg)

Introduction: Anorectal malignant tumours are increasing in frequency for unknown reasons. Surgery is the principal treatment, and the role of adjuvant therapy has not been defined. We therefore decided to review the experience of the Princess Margaret Hospital in Toronto, a large tertiary care cancer hospital, with respect to the surgical management of anorectal melanoma., Methods: We reviewed the charts of all registered patients with anorectal malignant melanoma (AMM) treated with surgery or radiotherapy, or both, at the hospital between 1980 and 1999, paying particular attention to survival, and local and distant recurrences., Results: There were 14 patients, all of whom were followed up to the time of death or for a minimum of 28 months for surviving patients. The mean ages at diagnosis were 56 years for men and 68 years for women. Clinical staging was as follows: local, 10 patients; locoregional, 3 patients and metastatic disease, 1 patient. Local therapy included local resection alone in 7 cases and abdominoperineal resection in 7. Seven patients received pelvic irradiation at some time during their disease, using different doses and fractionation schemes. Three of them had concomitant chemotherapy and radiotherapy with no tumour regression. In all 3 patients the lesions was reclassified as AMM and the patient underwent surgery. The other 4 patients had a short course of radiotherapy for palliation after the original lesion recurred. The overall median survival was 12 (range from 3-51) months. Two patients remained alive at last follow-up. Patients managed by local excision had a median survival of 12 (range from 3-51) months, and those managed by abdominoperineal resection had a median survival of 7 (range 5-51) months. Of the 10 patients treated initially with local excision, 6 required reoperation. Three underwent salvage abdominoperineal resection. Six patients were alive 1 year after treatment (median survival 32.5 mo [range from 21-51 mo]). Eight patients had a rapid evolution of their disease with a median survival of 5.5 (range from 3-12) months. Eleven of the 12 patients who died had metastatic disease., Conclusions: Systemic dissemination is almost universal in patients with AMM. The overall survival was poor regardless of local treatment. There was a 60% failure rate of local excision, which necessitated further surgery. Improving local control is important since some patients will survive up to 3 years.

Background: Anorectal melanoma is a rare but highly lethal malignancy. Historically, radical resection was considered the "gold standard" for treatment of potentially curable anorectal melanoma. The dismal prognosis of this disease has prompted us to recommend wide local excision as the initial therapeutic approach. The purpose of this study was to review our results in patients who underwent wide local excision or radical surgery (abdominoperineal resection [APR]) for localized anorectal melanoma., Study Design: We reviewed the charts of all patients referred for resection of anorectal melanoma between 1988 and 2002. Endpoints included overall survival, disease-free survival, and local, regional, or systemic recurrence., Results: Fifteen patients underwent curative-intent surgery; four underwent APR and 11 underwent wide local excision. Eight patients (53%) are alive; 7 (47%) are disease-free (followup 6 months to 13 years). Of 12 patients who have been followed for more than 2 years, 4 are alive (33%) and 3 are disease-free (25%). Seven patients have been followed for more than 5 years and two are alive and disease-free (29%). All of the longterm survivors underwent local excision as the initial operation. There were no differences in local recurrence, systemic recurrence, disease-free survival, or overall survival between the APR group and the local excision group. Local recurrence occurred in 50% of the APR group and 18% of the local excision group; regional recurrence occurred in 25% versus 27%. Distant metastases were common (75% versus 36%)., Conclusion: In patients who have undergone resection with curative intent for anorectal melanoma, most recurrences occur systemically regardless of the initial surgical procedure. Local resection does not increase the risk of local or regional recurrence. APR offers no survival advantage over local excision. We advocate wide local excision as primary therapy for anorectal melanoma when technically feasible.

A multimodal approach including preoperative external beam radiation, surgical resection, and intraoperative electron radiation was used in 23 patients with locally advanced anal or recurrent rectal cancers involving the sacrum. The proximal extent of complete sacral resection was S2 in three patients, S3 in 12 patients, S4 in two patients, and S5 in one patient. The tumor was confined to the anterior sacral cortex in five patients. The resection was marginal in 10, contaminated marginal in 11, and intralesional in two patients. At 19 to 54 months of followup, five patients are alive without evidence of disease and four are alive with disease. Twelve patients died of their disease, and two died of other causes. There was a mean survival of 32.9 months for the patients who were alive at followup. Kaplan-Meier survival for all patients was 82% at 1 year and 73% at 2 years, with death of disease as an endpoint. Thirteen (57%) patients had another local recurrence develop at a mean of 17.2 months. Eight (35%) patients had metastatic disease develop at a mean of 16.3 months. Proper patients selection is important in ensuring a favorable outcome from this aggressive surgery.

Individuals who are seropositive for the human immunodeficiency virus (HIV) often suffer from disorders affecting the anorectum, but unfortunately the best kind of treatment frequently seems questionable. In a retrospective investigation we reviewed 29 HIV-positive patients with anorectal lesions who had experienced a proctologic operation. Our impression correlates with other findings according to which HIV-positive patients in advanced stages have a poorer post-operative outcome in terms of morbidity and survival than patients in an early CDC disease stage. This implies that the indication for a proctologic operation in an immunocompromised state has to be considered carefully.

Purpose: The aim of this study was to determine the outcome of patients with leiomyosarcoma of the rectum or anus treated with local excision and brachytherapy., Methods: Eight patients with leiomyosarcoma of the rectum (7 patients) or anus (1 patient) were treated with a transanal excision followed by a temporary iridium-192 interstitial implant to 4,500 cGy. Median tumor size was 4.2 (range, 1.5-5) cm. Margins were positive in six patients, negative in one patient, and close in one patient., Results: With a median follow-up of 53 months, median survival time was 53 months and the three-year actuarial survival rate was 71 percent. The cumulative incidence of failure as a component of failure for local was 25 percent (2/8), for abdominal was 0 percent (0/8), and for distant was 25 percent (2/8). Four patients eligible for functional analysis all had excellent sphincter function (1-2 bowel movements per day, no soilage)., Conclusion: In selected patients the use of conservative surgery followed by brachytherapy is a reasonable alternative to an abdominoperineal resection. However, more experience and longer follow-up are needed before this approach can be recommended routinely.

Purpose: There have been 49 cases of adenosquamous carcinoma of the colon, rectum, and anus reported in the English literature. We have reviewed 145 cases of adenosquamous carcinoma to better define epidemiologic and survival characteristics of this extremely rare colon carcinoma., Methods: The National Cancer Institute's Surveillance, Epidemiology, and End Results program public use CD-ROM file for the years 1973 through 1992 were reviewed. This represents approximately 9.5 percent of the United States population. Adenosquamous carcinomas arising in the colon, rectum, and anus were identified using the International Classification of Diseases-O codes. The Astler-Coller tumor classification was used for staging. Two-tailed Student's t-test, Mantel-Haenszel chi-squared tests, and generalized Wilcoxon's tests were used for comparisons of means, proportions, and actuarial survival rates, respectively. Survival curves were calculated by the Kaplan-Meier method., Results: One hundred forty-five cases of adenosquamous carcinoma were identified, representing 0.06 percent of all colorectal malignancies. The mean age of patients was 67 years. Eighty-four percent of patients were Caucasians, 15 percent were Afro-Americans, and 1 percent were other races. Afro-Americans were diagnosed at a significantly younger age (median age, 62 years; P = 0.03). Fifty-three percent of the carcinomas were located in the sigmoid colon, rectum, and anus, 28 percent in the right colon, and the rest in the middle segment. Seventy-four percent of distal cases were staged A through C, compared with 44 percent of proximal cases. Patients with adenosquamous carcinoma of the sigmoid colon, rectum, and anus survived longer than all other patients (P = 0.001). Patients with adenosquamous carcinoma Stages A and B1 had survival rates similar to patients with comparably staged adenocarcinomas. Fifty percent of the patients, including most of the patients with D stage, died in the first year. Patients with Stages B2, C, and D adenosquamous carcinomas had a significantly shorter survival than the comparably staged adenocarcinomas (P < or = 0.02). The overall adjusted five-year survival rate was 30.7 percent. In those patients who survived more than 24 months, the five-year survival was 84 percent., Conclusions: The survival rates for patients with adenosquamous carcinoma Stages A and B1 are similar to patients with comparably staged colorectal adenocarcinomas. However, we found that patients with colorectal and anal adenosquamous carcinomas staged B2 through D have significantly poorer survival than patients with comparably staged adenocarcinomas, supporting the previous reports of a poor prognosis associated with adenosquamous carcinomas.

Purpose: To evaluate the results of interstitial radiotherapy of anorectal tumors., Patients and Methods: From 1972 to 1993, one of the authors treated 45 patients by an interstitial implant for anorectal tumors. Of these, 33 patients suffered from primary tumors, 19 from squamous carcinoma, 2 from basaloid carcinoma of the anus and the other 12 from primary adenocarcinoma of the rectum. Of 12 patients treated for local recurrence, 10 had adenocarcinoma and 2 squamous cell carcinoma. Of the 33 patients with primary tumors, 27 received a course of external-beam radiotherapy before the implant. The median follow-up was 35 months., Results: Local response depended on the tumor volume treated. All 21 anal tumors showed complete response, 5 patients developed local recurrence and 4 distant metastases: 3 died from their disease. Of 12 rectal adenocarcinomas, 9 responded completely, 4 patients developed local recurrence and 4 distant metastases; 6 died from active disease. In the last group of 12 patients who were treated for recurrent tumors, 7 responded completely. One patient developed local recurrence and 9 distant metastases, only 4 are alive., Conclusions: A combination of external-beam and interstitial radiotherapy is a relatively simple, non-mutilating, but well-tolerated and very effective method of treatment for early carcinoma of the lower rectum and anus.

Aim: Retrospective analysis of a series of 287 anal canal carcinomas seen during a 15-year period in the Department of Radiotherapy Oncology of Hospices Civils de Lyon., Material and Method: Between 1980 and 1995, 287 patients were managed in the radiotherapy department of Hospices Civils de Lyon. In 25 cases, the patients were referred for recurrence. Post or preoperative irradiation was performed in 12 and 23 cases. Palliative treatment was given to 15 cases and simple follow-up in 7 cases. Radiotherapy was given to 205 patients. In 71 cases irradiation alone and in 134 concomitant radiochemotherapy was given., Results: The 5-year overall survival of the group treated by radiotherapy (205 patients) was 71.5% and the 10 year-overall survival was 60.8%. The specific survival at 5 and 10 years was 81.9% and 74.7% respectively. At 5 years the overall survival was 78% for the group treated by concomitant radiochemotherapy and 60% for the group treated by irradiation alone., Conclusion: Radiotherapy is the standard treatment for anal canal carcinoma. Radiochemotherapy seems to improve results in advanced cases. The best irradiation of technique has yet to be defined.

Purpose: To review and to update the results of Total Anorectal Reconstruction with Electrostimulated Graciloplasty (ES-TAR) at the same time as or following abdominoperineal resection (APR)., Setting: A university hospital in Italy., Methods: Retrospective study., Population: A series of 98 consecutive anorectal cancer patients who had undergone ES-TAR (in 88 cases at the same time as APR; in 10 cases following APR), 61 of whom are still evaluable in respect of continence (median follow-up period 55 months)., Results: There was no mortality. Thirty-seven percent of patients had postoperative complications with no impact on survival or functional outcome. The 5-year survival rate in 50 patients was 61% and the 5-year estimated cumulative probability of survival in 81 patients was 65%. Local recurrence rate was 16%. Continence was achieved in 87% of patients with a chronically stimulated TAR, and in 69% of patients with short-term stimulation., Conclusion: ES-TAR is a safe and effective method for both curing anorectal cancer and restoring continence. It may be considered a reliable alternative to sphincter-saving procedures in lower rectal cancer patients.

Background: Melanoma occurring in the anorectal region is recognized to be an uncommon tumour with a poor prognosis. Queensland has the highest incidence rate of cutaneous melanoma in the world. The purpose of the present study was to determine if the very high incidence of cutaneous melanoma in Queensland has any bearing on the incidence of anorectal melanoma, and to compare this with published studies from other parts of the world. An assessment of the authors' results in the treatment of anorectal melanoma was made by a review of patients presenting during the last decade throughout Queensland., Methods: All cases of melanoma occurring in Queensland are accumulated in the Melanoma Register, administered by the Queensland Melanoma Project, and data from this were used to derive the results in the present study. Queensland population data were provided by the Australian Bureau of Statistics., Results: During the study period 1985-95 in Queensland there were 21,391 cases of cutaneous melanoma which occurred in an average population, during that time, of 2.89 million. During the same time there were nine cases of anorectal melanoma, giving a relative incidence therefore of 0.04%. This contrasts with published relative incidences of 0.2% for white people in North America, 11% for Southwestern Native Americans, and 14.2% for Northern Pakistan (P < 0.0001). However, the population-based incidence rates of anorectal melanoma for Queensland, United States white people, Southwestern Native Americans and Northern Pakistan are not significantly different from each other. All patients in the present series presented with large tumours, and none survived beyond 25 months despite standard surgical and adjuvant therapy., Conclusions: The protection against cutaneous melanoma afforded by skin pigmentation is not apparent in the anal area. Anorectal melanoma is no more or less frequent in Queensland, even with the very high incidence rate of cutaneous melanoma. Sun exposure appears to be neither protective nor a risk factor. The outcome of therapy for this condition remains disappointing in the authors' experience.

Purpose: This study was designed to describe recurrence and survival rates after operative treatment for anorectal melanoma and to identify predictive factors for recurrence., Methods: Records of 50 patients with anorectal melanoma from 1939 to 1993 were reviewed., Results: Overall five-year survival and disease-free survival were 22 and 16 percent, respectively. At the time of diagnosis, 26 percent of patients had metastatic disease, and all died within 12 (mean, 6.3) months. Five-year survival and recurrence rates were identical after either abdominoperineal resection (APR) or wide local excision, both with curative intent. Gender, size of tumor, presence of melanin, positive perirectal lymph nodes, or treatment were not predictive of recurrence. Anorectal melanoma was found incidentally after hemorrhoidectomy or polypectomy in five patients. Three other patients underwent an excisional biopsy of a lesion measuring less than 2 cm. Of these eight patients, five underwent APR and three underwent wide local excision with no microscopic residual tumor at pathology. All developed regional or systemic recurrence at a mean of 21 (range, 4-88) months, and all died of their disease at a mean of 29 (range, 5-98) months., Conclusion: Prognosis for anorectal melanoma is poor, irrespective of surgical treatment performed. No predictive factors for recurrence were identified in this series. Wide local excision with a negative margin of a least 1 cm is suggested as the treatment of choice. APR should be reserved for tumor not amenable to local excision or for palliative treatment of large obstructive lesion until effective adjuvant therapies are available.

Objective: The aim of this study was to determine the significant prognostic factors for primary anorectal melanoma and propose a standard surgical procedure., Patients and Methods: From 1975 to 1995, 19 patients with primary melanoma of the anus were treated. Five patients were given palliative care, 6 had abdominoperineal amputation and 8 had curative local transrectal tumoral excision. Two patients also underwent inguinal node dissection., Results: Overall 5-year survival was 21%. Univariate analysis showed a significantly poorer prognosis when: inguinal or pelvic nodes were invaded (p = 0.01), surgical margins were invaded (p = 0.003), visceral metastases were present (p = 0.01) and the tumor measured over 20 mm thick (p = 0.01). Prognosis was unchanged by the type of surgical exeresis., Conclusions: Local transrectal excision is the first ling choice for primary anorectal melanoma. Amputation of the rectum should be reserved for cases where complete tumor resection is technically impossible transrectally. Node dissection is indicated in cases with clinical invasion of the inguinal nodes.

33 Cases of anorectal malignant melanoma (ARMM) diagnosed pathologically were treated in Tianjin Cancer Hospital from 1996 to 1994. Some of these patients were treated with abdominoperineal resection (Mile's operation) and local resection plus chemotherapy, biotherapy and radiotherapy, others were given chemotherapy only. 25 cases were followed up from six months up to six years. The results showed that the median survival time was 23.5 months in the group of Mile's, 17.5 months of local resection and 5.5 months in chemotherapy alone respectively. The authors considered that early diagnosis and treatment are the key to raise the survival rate. For the cases diagnosed pathologically the Mile's operation should be performed and groin dissection is necessary for cases of groin lymphatic metastasis.

Background: Great controversy exists with regard to the best surgical therapy for anorectal malignant melanoma., Materials and Methods: Between 1980 and 1996, 15 patients with anorectal malignant melanoma were treated. The recurrence pattern after therapeutic intervention and their survival were evaluated., Results: There were nine females and six males, their mean age was 66.3 years. At diagnosis their disease stages were: I (n=7), 11 (n=3) and III (n=5). Patients with stage I disease were treated with abdominoperineal resection (APR) (n=6) and local excision (n=1); their average tumour size and thickness were: 4.7 cm and 6.4 mm respectively; their median follow-up and disease-free survival were 11 months and 7 months respectively; their recurrence pattern was: local (n=6), inguinal (n=4) and distant (n=6). Those patients with stage II and III disease were treated with transverse colostomy (n=6); two of them received 50 Gy of radiotherapy and local excision plus interferon alpha-2b (n=2), all had progressive distant disease. Patients with stage I disease had a median survival of 12 months compared with 5 months for those with stages II and III (P=0.10). The overall 5-year survival was 0%., Conclusion: The recurrence pattern in anorectal malignant melanoma is mainly at distant sites. The role of APR in maintaining local control over tumours larger than 4 cm or thicker than 5 mm remains elusive.