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Competing Interests: The authors declare that they have no conflict of interest.

Extramammary Paget's disease (EMPD) is an uncommon cutaneous neoplasm almost exclusively located in the vulvar, perianal, and male genitalia regions. Evaluation and management are complicated given the average delay in diagnosis is two years and approximately 30% of cases are associated with underlying malignancies. The axilla is a unique location for EMPD. We report a rare case of a 78-year-old male with axillary EMPD associated with an underlying adenocarcinoma. A 1-cm tender and pruritic erythematous plaque with surrounding erythema appeared in the patient's axilla. An irritated seborrheic keratosis secondarily impetiginized along with irritant contact dermatitis was suspected. Treatment of cefdinir and topical hydrocortisone failed and a biopsy was taken. Microscopic and immunohistochemical examination showed ulceration with an underlying proliferation of atypical glands (Figure 2A) and a nested intraepidermal proliferation with pagetoid spread (Figure 2B). The atypical cells were positive for gross cystic disease fluid protein 15 (Figure 2C), epithelial membrane antigen (Figure 2D), cytokeratin 5/6, and cytokeratin 7. These findings were supportive of an apocrine adenocarcinoma arising in association with EMPD. Wide location excision was performed. Screening for associated malignancies or lymphatic spread is the primary goal during evaluation. Outcomes are favorable when the primary neoplasm is of limited distribution. The accepted treatment for primary lesions is wide local excision, although anatomic tissue constraints necessitate further research into other treatment modalities. To our knowledge, this is the 14th reported case of axillary EMPD with an underlying adenocarcinoma which may help with identification and management of future cases., (Copyright© South Dakota State Medical Association.)

Background: Extramammary Paget disease is a relatively rare and less malignant intraepithelial adenocarcinoma. t is found in areas with abundant distribution of apocrine sweat glands such as the external genitalia, external genitalia, and perianal area, with fewer armpits. The disease progresses slowly and is prone to misdiagnosis in clinical practice., Methods: We retrospectively analyzed a female patient. She had a left axillary mass for more than 2 years. Recently, the mass increased and the surface skin was ulcerated. Then she went to Jiangxi Provincial Dermatology Hospital for left axillary lesion resection, and the postoperative pathology showed Paget disease outside the breast. For further diagnosis and treatment, she came to our hospital. We diagnosed a tumor with uncertain or unknown dynamics in the left axillary breast. Under general anesthesia, left subaxillary mass resection, freezing and left breast cancer breast conserving surgery was performed., Results: The postoperative pathology of the left axillary mass combined with morphological and immunohistochemical results was consistent with Paget disease. Postoperative immunohistochemistry showed estrogen receptor (+, 20%), progesterone receptor (-), human epidermal growth factor receptor-2 (3+), Ki-67 (30%), cytokine7 (+), and p63 (-). Following up for 22 months, there has been no local recurrence, no swelling of the right axillary lymph node, no distant metastasis found on follow-up, and no complications such as upper limb lymphedema, upper limb sensory abnormalities, or motor disorders have been observed., Conclusion: Paget disease outside the axillary breast is relatively rare, and surgical resection is the best choice. The prognosis is good, and the recurrence rate is low., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Paget's disease was first described in 1874 as an eczematoid changes of the nipple associated with underlying breast carcinoma. Extra-mammary Paget's disease (EMPD) is rare with involvement of the male genitalia described in small case series and management options varying according to location and extent. The diagnosis of EMPD requires a high index of clinical suspicion and close liaison with the multidisciplinary team, particularly histopathology. We present two cases of EMPD affecting the male external genitalia that highlight important learnings in the presentation, diagnosis, and management of EMPD.

Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma. To increase awareness and knowledge regarding this rare dermatosis, we hereby reported a case of ulcerated plaque on genitalia.

Aim: Perianal Paget's disease (PAPD) is a rare disorder with a predisposition to anal and colorectal malignancies and an unclear prognosis. Our previous 25-year series demonstrated a non-aggressive nature. This study aims to describe our updated institutional experience., Methods: This is a retrospective review of all patients diagnosed with primary PAPD from 1991 to 2021. A prospectively maintained institutional database was searched which included demographics, clinical and pathological manifestations, treatment methods, recurrence, oncological outcome and mortality., Results: Thirty patients were diagnosed with PAPD. Fifteen were women (50%); the average age at diagnosis was 71 ± 10.7 years, and the average lesion size was 3.7 ± 2.6 cm. At diagnosis, 12 (40%) were harbouring invasive anal adenocarcinoma. Eight (27%) developed adenocarcinomas concurrent with PAPD recurrence at a mean interval of 9 ± 4.4 years (range 1.9-14.8). The Kaplan-Meier curve estimated overall survival of 93%, 86%, 82%, 65% and 56% at 1, 3, 5, 10 and 15 years, respectively. Median survival was 16 years. Six (20%) had disease-related mortality. Initially, nine (30%) were treated with abdominoperineal resection (APR), 15 (50%) underwent local resection, three (10%) were treated with radiotherapy, two (7%) received only topical therapy and one (3%) chose observation. Fifteen (50%) experienced recurrence of PAPD, two after undergoing APR. Five (17%) had persistent disease until death. Only 10 (33%) did not experience PAPD recurrence, seven of whom underwent APR. The mean follow-up time was 9.2 ± 6.2 years., Conclusions: Perianal Paget's disease is an aggressive entity with high rates of synchronous anal adenocarcinoma at diagnosis and development of metachronous adenocarcinoma later in life., (© 2023 Association of Coloproctology of Great Britain and Ireland.)

Extramammary Paget's disease is a rare cutaneous malignancy affecting areas with a high concentration of apocrine glands. The authors present a 77-year-old male followed in the General Surgery consultation due to anal pruritus complaints with an evolution of around 20 years and recent lower gastrointestinal bleeding. A colonoscopy was requested and the patient was observed by Gastroenterology team in this context. The objective examination showed an erythematous-whitish plaque with well-defined, slightly exudative and verrucous limits in the perianal region. Definitive diagnosis was possible by biopsy of the referred lesion carried out by Dermatology, and the histological findings were compatible with Paget's disease: intraepithelial infiltration by large atypical neoplastic cells and hypochromic halo and positive immunostaining for cytokeratin 7. Perianal Paget's disease, often underdiagnosed, may be associated with colorectal neoplasms. We present this case due to its rarity, in order to draw attention to this diagnosis.

Introduction: AS an uncommon neoplasm, goblet cell adenocarcinoma (GCA) is characterized by mixed endocrine-exocrine features. It is almost exclusively found in the appendix. Primary GCA of the anal canal is extremely rare., Case Presentation: Herein we describe a novel rare case of 74-year-old Chinese female who is diagnosed with GCA in the anal canal with perianal Paget disease, including a brief review of the literature. In the lesion of anal canal, the tumor was composed of signet-ring-like cells on confluent growth model and copious mucin was produced as well. Simultaneously, the results of immunohistochemistry showed signet-ring-like cells were positive for CK20, CDX2, synaptophysin (Syn), CD56, carcinoembryonic antigen (CEA) and Villin. Meanwhile, the Ki67-labeling index reached 40%. In the lesion of perianal Paget disease, the small groups of atypical neoplastic cells were present in the epidermis. Immunohistochemically, the neoplastic cells were positive for CK20, CDX2 and epithelial membrane antigen, but negative for CK7, GCDFP15, S100, HMB45, and P63. The Ki67-labeling index reached 60% in the most concentrated spot., Conclusions: Extra-appendiceal GCA was rare and easily under-recognizable. The diagnosis of GCA was seldom made preoperatively. Occasionally, GCA could occur in the anal canal accompanied by perianal Paget disease. So careful rectal examination was important in the patient with perianal Paget disease for avoid missing diagnosis of GCA on anal canal. GCA may show aggressive clinical behavior compared with typical well-differentiated neuroendocrine tumors. Therefore, we should pay more attention on the recognization of this rare disease., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Vulval extramammary Paget disease (vEMPD) is an uncommon epithelial malignancy that may arise within the vulva (primary vEMPD) or represent vulval skin involvement by a noncutaneous carcinoma (secondary vEMPD). Primary vEMPD is most often an in situ carcinoma arising within the epidermis but may be associated with dermal invasion (invasive vEMPD) or represent intraepidermal spread of an adenocarcinoma originating in vulval skin adnexa or anogenital mammary-like glands. The latter, termed mammary gland-like adenocarcinoma (MGLA), exhibits morphologic, immunohistochemical, and molecular features of various breast carcinomas but, as far as we are aware, the metaplastic variant of MGLA has not been reported on the vulva. We report 2 cases of metaplastic MGLA of the vulva with associated Paget disease and postulate that some cases of vulval MGLA may arise from Paget disease rather than originating in mammary-like glands. We also report a unique case of secondary vEMPD resulting from spread of urothelial carcinoma in situ that subsequently progressed to invasive urothelial carcinoma within the vulva., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 by the International Society of Gynecological Pathologists.)

Extra-mammary Paget's disease is a rare cancer affecting the anogenital region and can present with a myriad of symptoms. In women, the diagnosis of Paget's disease can be challenging as it mimics several other vulval conditions. It is important to promptly recognise this disease because of its potential association with synchronous tumours, such as colorectal adenocarcinoma. The mainstay of treatment is with immunomodulating therapies or surgery, but unfortunately the risk of recurrence is high and appears to be independent of treatment choice and tissue margin status. There is growing evidence to move away from traditional surgical excision to using topical therapy, such as imiquimod, as surgery can often be extensive and disfiguring with a prolonged recovery time. There is considerable psychosexual and physical morbidity associated with Paget's disease, largely owing to multiple surgical recurrences. As recurrences can occur several years after the initial presentation, long-term follow up of patients is recommended.

Primary adenocarcinoma of the anorectum, compared with squamous cell carcinoma, is a rarer and more aggressive malignant neoplasm. Infection with human papillomavirus (HPV) has been identified as a causal agent in a variety of tumors, including those of the cervix, head and neck, and anogenital region, especially squamous cell carcinoma. However, the relationship between HPV and anorectal adenocarcinoma has not been well studied. In this article, we report an HPV-related anorectal adenocarcinoma arising in a tubulovillous adenoma in a 76 years old female who presented initially with lower gastrointestinal bleeding. The carcinoma cells were positive for cytokeratin 7 and p16 by immunohistochemistry. High-risk HPV RNA in situ hybridization was positive. A follow-up examination of the anal area showed perianal plaques. Histologically, the excision of the perianal lesion showed intraepithelial infiltration by sheets and clusters of large atypical neoplastic cells. The neoplastic cells showed the same immunoprofile compared with the anorectal adenocarcinoma with p16 and high-risk HPV positivity. The findings are consistent with extramammary perianal Paget's disease secondary to anorectal adenocarcinoma. HPV-related adenocarcinoma in the anorectum is a newly recognized entity and was previously considered clinically indolent. Our case uniquely exhibits adenoma-carcinoma-perianal Paget's disease sequence, which has not been reported before. Our findings suggest that evaluation of the patient's lower genital tract for any HPV-associated lesions and long-term follow-up are required to monitor the disease progression in this type of malignancy.

Paget's disease (PD) denotes an initially intra-epidermal adenocarcinoma that can later invade the dermis and metastasise. Among the extramammary forms of PD (EMPD), penoscrotal presentations are rarer than the vulvar and perianal forms. Once diagnosis has been confirmed by histopathological examination, a search for associated neoplasia must be conducted, although penoscrotal EMPD is less frequently associated with underlying neoplasia than mammary PD (MPD). The associated cancer most often involves a neighbouring organ, with prostate cancer being the most common, or in some cases consists of underlying cutaneous adnexal tumours. First-line therapy consists of surgical excision. Alternatives to surgery (imiquimod, CO 2 laser vaporisation, dynamic phototherapy) may be considered in certain cases., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background and Objective: Extramammary Paget disease (EMPD) has seldom been studied in Mediterranean populations. We aimed to review the characteristics of our patients with EMPD, the presence of a neoplasm in continuity, and the long-term course of the disease., Patients and Methods: Retrospective observational study of 27 patients diagnosed with EMPD between 1990 and 2015. All clinical and pathology findings related to clinical course and outcomes were retrieved for analysis., Results: Twenty patients were women and 7 were men. Ages ranged from 42 to 88 years (median, 76 years). Lesions were in the following locations: vulva (16 cases), pubis-groin (5), perianal region (4), and axilla (2). Time from onset to diagnosis ranged from 1 to 60 months (median, 12 months) and maximum lesion diameter from 20 to 140mm (median, 55mm). In 3 cases (11.1%) EMPD was a secondary condition. None of the lesions developed on a previous cutaneous adnexal adenocarcinoma. Ten of the 24 primary EMPDs (41.7%) invaded the dermis. Eight of the 27 patients (29.6%) experienced local recurrence after the initial surgical treatment.Three patients (11.1%) died as a consequence of metastasis from the EMPD., Conclusions: The presence of an underlying cutaneous adnexal adenocarcinoma is uncommon, but it is not unusual to find an extracutaneous adenocarcinoma in continuity. Although EMPD is a slow-growing tumor, dermal invasion is frequent and metastasis is not uncommon. Local recurrence is common even after excision with wide margins and may be delated, so long term follow-up is essential., (Copyright © 2020. Publicado por Elsevier España, S.L.U.)

Vulvar Paget disease (VPD) is an unusual and rare adenocarcinoma that can be challenging to diagnose. Although the condition has a high treatment success rate, it can be associated with underlying malignancy, recurrence rates can be high, and patients often need regular and frequent follow-up. The article describes a woman whose nonresolving, pruritic vulvar lesion was found to be VPD on biopsy.

A man aged 83 years with vague perirectal symptoms had a delayed diagnosis of Paget's disease of the anus. A lack of thorough digital rectal examination failed to diagnose a mixed adenonueroendocrine tumour of the rectum in a timely matter., (2017 BMJ Publishing Group Ltd.)

Extramammary Paget disease (EMPD) is a rare malignant neoplasm of apocrine sweat glands that is morphologically and histologically identical to Paget disease of the breast. The primary lesion is usually a solitary, well-demarcated, erythematous, scaly plaque that may contain crust, erosions, or ulcerations. The vulva is the most common site, but any area containing apocrine sweat glands may be involved. We present a case of triple extramammary Paget disease of the groin and bilateral axillae in a diabetic patient whose axillary lesions appeared consistent with acanthosis nigricans. This case demonstrates the need to consider EMPD in the evaluation of acanthosis of the axilla given its ability to mimic more common conditions.

Mammary-like adenocarcinoma of the vulva associated to Paget's disease is exceedingly rare. So, it is very important to perform all the pathological and immunohistochemical investigations to achieve differential diagnosis from both a metastatic lesion from an orthotopic breast cancer and a vulvar adnexal tumor. This report describes a case of vulvar Paget's disease associated with underlying mammary-like adenocarcinoma diagnosed in the Department of Obstetrics and Gynecology of Farhat Hached university hospital of Sousse in Tunisia. We also review previously reported cases of primary breast-like carcinoma of the vulva with or without Paget's disease.

Extramammary Paget disease (EMPD) often develops in external genitalia. Paget cells can, however, adopt an invasive phenotype and metastasize to regional lymph nodes and beyond, leading to poor patient outcomes. Based on this clinical observation, multiple lymphadenopathy may represent an initial sign of EMPD. To address the potential significance of multiple lymph node swelling in EMPD, we report two patients with cutaneous primary EMPD who showed multiple lymphadenopathy as an initial sign during the clinical course of the disease as well as tumour metastasis. Significantly, marked lymphatic vessel growth was observed in regional lymph nodes that underwent massive tumour cell invasion. Therefore, nodal lymphangiogenesis may promote tumour cell invasion and metastasis to distant organs, including the lymph nodes, emphasizing the clinical relevance of multiple lymphadenopathy., (© 2010 The Authors. BJD © 2010 British Association of Dermatologists 2010.)

Perianal Paget's disease is categorized as Paget's disease, which is epidermotropic neoplasm arising from the apocrine glands of perianal region, or Pagetoid spread invaded from rectal or anal canal cancer. It has been reported that the immunohistochemical staining of GCDFP15, the marker of the apocrine epithelium, and CK20 is used to be distinguished between Paget's disease and Pagetoid spread. Five patients with perianal Paget's disease who underwent a surgical operation had been treated in our department between 1997 and 2006. We analyzed the clinical findings and the treatment of these patients and investigated the expression of GCDFP15 and CK20 by immunohistochemical staining. All cases presented the redness around perianal regions, and 2 cases were recognized a tumor at the anal canal. We preoperatively diagnosed these cases as Pagetoid spread and others without tumor regions as Paget's disease. Surgical treatment was performed for all patients. As a result of immunohistochemical staining, 2 cases of Pagetoid spread were negative for GCDFP15, and positive for CK20. It was compatible with the preoperative diagnosis. Only one of 3 Paget's disease cases was positive for GCDFP15 and negative for CK20 resulting in the diagnosis of perianal Paget's disease. Based on the expression of negative for GCDFP15 and positive for CK20, others were seemed to be Pagetoid spread. A treatment strategy including surgical operation and chemotherapy is different between patients with Paget's disease and those with Pagetoid spread. Therefore, it is essential to investigate the expression pattern of GCDFP15 and CK20 using the tissue from the biopsy to identify the disease for appropriate treatment.

Background: Extramammary Paget disease (EMPD) is categorized into 2 groups: primary EMPD or EMPD secondary to underlying malignancy. Primary EMPD has a better prognosis, and the ability to distinguish between the 2 subsets has clinical relevance. Recent studies have suggested that immunostains, including cytokeratin (CK) 7, CK20, and BRST-2, distinguish between the 2 groups. We analyzed a large series of EMPD with an expanded immunohistochemical panel to assess its value in distinguishing primary from secondary disease., Design: Formalin-fixed, paraffin-embedded sections of 98 EMPD specimens from 61 patients (45 primary and 16 secondary) were immunostained with CK7, CK20, HER-2/neu, BRST-2, CDX2, androgen receptor (AR), and cyclin D1. The study included 44 women and 17 men (median age: 73 years). Median follow-up time was 47 months., Results: All EMPD specimens were vibrantly positive for CK7. The frequency of positivity for all EMPD samples was CK20 (28%), BRST-2 (40%), HER-2/neu (64%), CDX2 (10%), AR (16%), and cyclin D1 (76%). For primary EMPD, the frequency of positivity was CK20 (22%), BRST-2 (48%), HER-2/neu (65%), CDX2 (2%), AR (21%), and cyclin D1 (84%). For secondary EMPD, the frequency of positivity was CK20 (50%), BRST-2 (25%), HER-2/neu (60%), CDX2 (33%), AR (0%), and cyclin D1 (53%). Notably, all 6 of 7 cases of EMPD secondary to an anorectal adenocarcinoma tested were HER-2/neu negative and 5 of those 6 cases (80%) were CDX2 positive., Conclusions: The role of CK7, CK20, and BRST-2 in distinguishing primary and secondary EMPD is limited because CK20 and BRST-2 were positive in large subsets of both groups. An expanded immunohistochemical panel, including HER-2/neu and CDX2, may be useful in discriminating primary EMPD from EMPD secondary to anorectal adenocarcinoma but fails to distinguish primary EMPD from EMPD secondary to urothelial or prostatic malignancy. The consistent overexpression of HER-2/neu in primary EMPD suggests a role for trastuzumab therapy in patients with recurrent disease.

Apocrine carcinoma is a rare malignant sweat-gland neoplasm with apocrine differentiation. There have been some reported cases of apocrine carcinoma with apocrine naevus. We report a case of a 78-year-old man with a painless tumour of the left axilla. Positron emission tomography (PET) showed slight fluorodeoxyglucose (FDG) uptake in both axillae. The patient underwent radical excision of the left axilla with left axillary lymph-node resection. The resected specimen showed apocrine adenocarcinoma with extramammary Paget's disease and apocrine naevus. Two years later, the patient noted enlargement of the right axilla, and PET showed increased FDG uptake. On resection of this enlarging right axilla, an apocrine naevus was found. FDG-PET is a useful method for detecting precancerous lesions, allowing monitoring of abnormal foci that are not suspicious for cancer and have no clinically apparent cause for concern.

Perianal Paget's disease is rare. It usually represents intraepidermal extension of an invasive carcinoma from an adjacent internal organ, but some cases represent primary intraepithelial cutaneous apocrine adenocarcinomas. Here, we report a unique case, which we interpret as synchronous primary perianal Paget's disease and lower rectal adenocarcinoma. Immunohistochemical stains demonstrated that the Paget's cells were CK7+/ CK20-/GCDFP+, whereas the rectal adenocarcinoma was CK7+(variable)/CK20+/GCDFP-. This discordant immunoprofile supported our impression that the Paget's disease in this patient was of cutaneous apocrine origin rather than a pagetoid extension from the patient's nearby rectal adenocarcinoma-to our knowledge a hitherto undocumented occurrence.

Objective: The aim of our study was to review our experience with Paget's disease of the vulvar relative to initial examination, treatment and oncological outcome., Methods: Ten women with extramammary Paget's diseases of the vulva were treated during the 10-year period. The charts of these patients were reviewed and data were collected regarding patient demographics, symptoms, previous Paget's disease, surgical treatments and time to recurrence., Results: Eight women (80%) were treated with wide local excision or partial vulvectomy, and two patients (20%) required radical resection for invasive adenocarcinoma. The group of women who had invasive diseases also underwent inquinofemoral lymphadenectomy and no lymphatic metastases were noted. Three women (30%) experienced recurrence. The mean time to relapse was 30 months (range 3-88 months)., Conclusion: Recurrence is very common and long-term monitoring is recommended with careful examination of any abnormal vulvar lesion.

We report a case of extramammary Paget's disease with underlying adenocarcinoma simulating breast carcinoma of the vulva. An 82-year-old woman was found to have a 5 x 3-cm bulky tumor located in the left labium major, infiltrating to the clitoris, left labium minor, and left lateral tissue of the vulva. Small biopsy of the vulva showed intraepidermal proliferation of Paget cells. The patient underwent wide local excision of the vulvar tumor and dissection of left inguinal lymph nodes. Histopathological examination of the resected specimens revealed that Paget cells were distributed singly or tended to form small nests in the epidermis, and that association of these cells with the underlying carcinoma invading to the subcutis could be seen. The underlying carcinoma was composed of squamoid solid nests with central necrotic debris, mimicking 'comedocarcinoma' of the breast. In other areas, the tumor cells were present in tubular formations and solid cords reminiscent of invasive ductal carcinoma of the breast. Immunohistochemically, the Paget cells and the underlying carcinoma cells were positive for carcinoembryonic antigen, epithelial membrane antigen, estrogen receptors, and glandular keratins except for CK 20. We speculate that our case is vulvar Paget's disease presenting as a manifestation of underlying breast carcinoma of the vulva, which might have arisen from either the ectopic breast tissue or anogenital mammary-like glands.

Background: Extramammary Paget's disease (EMPD) is a rare clinical entity and can be associated with other malignancies. We analyzed our experience for prognosis and impact of therapy on outcomes., Study Design: We conducted a retrospective review of all patients (n = 33) with EMPD treated at a tertiary care center from 1971 to 1998. Pathologic features of EMPD, concurrent secondary malignancies, and the effect of operations on recurrence were analyzed. Overall survival was compared with that of the general population., Results: Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p = 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population., Conclusions: EMPD is an infrequently diagnosed disease that is preferably managed with complete local excision and reexcisions if needed. A thorough search for frequently occurring secondary malignancies might be beneficial to provide the best outcomes for these patients., (Copyright 2003 by the American College of Surgeons)

Background: There have been no reports of the efficacy of primary radiotherapy with concurrent chemotherapy for vulvar adenocarcinoma associated with extramammary Paget's disease. In this report we discuss the differential diagnosis and efficacy of treatment for this rare tumor., Case: The patient was diagnosed as having vulvar carcinoma stage IVb. A dose of 40 Gy in 20 fractions was administered to the vulva, including the tumor mass, and the inguinal-femoral lymph nodes with concurrent systemic chemotherapy. Outside the radiated field, bone scintigraphy showed an increase in abnormal uptake images. However, in the radiated field, the tumors disappeared macroscopically, and bone scintigraphy also showed a disappearance of the abnormal uptake images., Conclusion: Within the radiated field, the efficacy was brought about by either radiotherapy only or by radiotherapy together with the sensitizing effects of chemotherapy. As for the chemotherapy itself, it was not effective in this case.

A case of extramammary Paget's disease of the vulva giving rise to an invasive microacinar adenocarcinoma is presented. Extramammary Paget's disease of the vulva is rare and documented detailed cases associated with unequivocal dermal invasion exceptional. We have reviewed the literature with particular reference to such cases.