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A 57-year-old man was incidentally diagnosed with a 7 cm left-sided retroperitoneal mass. The mass was presumed to be arising from the left adrenal gland on a CT scan of the adrenal glands. Thus, a thorough workup ensued, looking into the possible functionality of the lesion. All investigations resulted in the negative. Subsequently, the patient was referred for surgery to fully characterise the tumour. Histology proved that the lesion was a benign retroperitoneal schwannoma with no relation to the adrenal gland.

Retroperitoneal tumours arising from the inferior vena cava (IVC) are rare tumours often requiring large vessel resection for complete surgical excision. Limited exposure to such tumours often discourages surgeons from offering surgical resection to these patients, depriving them of the only potentially curative modality. We present here the surgical technique for resection of a large IVC sarcoma without IVC reconstruction.A 53-year-old lady presented with a large retroperitoneal sarcoma encasing the infra-hepatic IVC with tumour thrombus extension into the hepatic cloaca as well as the left renal vein. Surgical resection was planned as the disease remained stable after 2 cycles of neoadjuvant chemotherapy with adriamycin and ifosfamide.Complete surgical excision of the tumour was achieved by performing a resection of the entire length of infra-hepatic IVC and right kidney, without IVC reconstruction. Left renal vein was divided after careful preservation of a draining collateral. Tumour thrombus was extracted from the hepatic cloaca, and proximal IVC stump closure was achieved with preservation of right hepatic vein insertion. Total blood loss during the procedure was 2300 mL, and the patient recovered without compromise of renal function or development of lower limb oedema.IVC resection without reconstruction can be safely performed for large retroperitoneal sarcomas involving major vascular structures. Familiarity with the retroperitoneal, retro-hepatic and supra-hepatic anatomy is paramount to achieving good surgical outcomes.

Objective To evaluate factors impacting survival outcomes in patients with uterine serous carcinoma (USC). Methods Data of consecutive patients diagnosed with USC undergoing surgery between 2000 and 2020 at Fondazione IRCCS Istituto Nazionale Tumori of Milan (Italy) were reviewed. Progression-free (PFS) and overall survival (OS) outcomes were evaluated using Kaplan-Meier and Cox proportional hazard models. Results Records of 147 consecutive patients meeting the inclusion criteria were analyzed. Stage distribution was: 67 (45.6%) patients with early-stage with uterine confined disease and 80 (54.4%) with advanced stages disease. Minimally invasive surgery was performed in 43 patients (29.5%). The median follow-up period was 78.6 months (IQ range = 35.7–117.3 months). The overall recurrence rate was 41% (60 patients): 19/67 patients (28.4%) with early-stage disease and 41/80 patients (51.3%) with advanced stage. The 5-year PFS rate was 35.0% (95% confidence interval [CI]: 27.5–44.7%). In multivariate analysis, age, BMI, depth of myometrial invasion, cytology, and optimal cytoreduction with postoperative residual tumor absent significantly impacted on PFS. The 5-year OS rates were 46.5% (95% CI: 38.1–56.8). The result of multivariate analysis showed that there was significant difference in OS based only on optimal cytoreduction and accuracy of retroperitoneal surgery. Conclusions In apparent early-stage USC, peritoneal and retroperitoneal staging allows to identify patients with disease harboring outside the uterus. Optimal cytoreduction is the most significant prognostic factor. Further collaborative studies are warranted in order to improve outcomes of USC patients.

Background We report two rare cases of retroperitoneal schwannoma completely resected by a laparoscopic medial-retroperitoneal approach aided by virtual navigation. Three-dimensional images have been used in liver and lung surgery, but there are few prior reports on retroperitoneal surgery. Case presentation These two case reports are of a 60-year-old man and a 40-year-old man with asymptomatic retroperitoneal schwannoma. In both cases, the tumors were located in the right renal hilum and were close to the duodenum, right ureter, and inferior vena cava. Simulation using three-dimensional images was performed before surgery, and a medial-retroperitoneal approach was performed to secure a wide surgical field. During the operation, we confirmed the location of the main feeder and the relationship between the tumor and organs with those shown on the three-dimensional images and performed total laparoscopic resection. Conclusion The medial-retroperitoneal approach provides operative safety. Preoperative simulation and intraoperative navigation with three-dimensional images, which can be freely rotated and interactively visualized from any angle, are useful methods to enhance the surgeon’s understanding of a patient’s specific anatomy and are especially effective when resecting a retroperitoneal tumor that is located in an anatomically deep and complex location.

Purpose The aim of this study was to create and validate dynamic nomograms to predict overall survival (OS) and disease-free survival (DFS) at different time points during follow-up in patients who had undergone resection of primary retroperitoneal sarcoma (RPS). Methods Patients with primary RPS operated upon between 2002 and 2017 at four and six referral centres comprised the development and external validation cohorts, respectively. Landmark analysis and multivariable Cox models were used to develop dynamic nomograms. Variables were selected using two backward procedures based on the Akaike information criterion. The prediction window was fixed at 5 years. Nomogram performances were tested in terms of calibration and discrimination on the development and validation cohorts. Results Development and validation cohorts totalled 1357 and 487 patients (OS analysis), and 1309 and 452 patients (DFS analysis), respectively. The final OS model included age, landmark time (TLM), tumour grade, completeness of resection and occurrence of local/distant recurrence. The final DFS model included TLM, histologic subtype, tumour size, tumour grade, multifocality and the interaction terms between TLM and size, grade and multifocality. For OS, Harrell C indices were higher than 0.7 in both cohorts, indicating very good discriminative capability. For DFS, Harrell C indices were between 0.64 and 0.72 in the development cohort and 0.62 and 0.68 in the validation cohort. Calibration plots showed good agreement between predicted and observed outcomes. Conclusion Validated nomograms are available to predict the 5-year OS and DFS probability at different time points throughout the first 5 years of follow-up in RPS survivors.

A 47-year-old man presented with problems of progressive abdominal distension for 3 months, associated with dull aching abdominal pain, increasing with food intake. He gave a 1-month history of shortness of breath on exertion and swelling of the lower limbs. On examination, he was pale and

Local recurrences (LR) and distant metastases (DM) are common in retroperitoneal soft tissue sarcoma (RPS). Longer time to recurrence and resection of the recurrent lesion have been identified as beneficial prognostic factors for overall survival (OS) upon first tumor relapse. However, prognostic factors concerning OS upon subsequent recurrences are scarcely defined. In this study, we aimed to identify prognostic factors for post-relapse outcome in multiple recurrent RPS.Patients undergoing resection of primary and recurrent RPS at the University Hospital Heidelberg were retrospectively analyzed. Multivariable Cox regression analyses were performed to identify predictors of overall, LR- and DM-free survival. Subgroup analyses were performed for liposarcoma and leiomyosarcoma patients.201 patients with primary disease, 101 patients with first, 66 patients with second and 43 patients with third LR as well as 75 patients with DM were analyzed. More than 12 months to recurrence and resection of recurrence were associated with improved OS after resection of first and second LR (5-year OS for first/second LR; resection: 64%/62%, no resection: 20%/46%). Gross macroscopic incomplete resection of first (p 0.001), second (p = 0.001), and third recurrences (p 0.001) was an independent prognostic factor for poor OS.Development of LR and DM is frequent in RPS. Once a tumor relapsed, patients benefit from tumor resection not only in case of first, but also in case of subsequent recurrences.

Introduction The incidence and patterns of local recurrence of colon cancer are not well reported. The aim of this study was to investigate the contemporary rates and patterns of local recurrence after sigmoid cancer resection, comparing pre and post-operative biomarkers in predicting local disease recurrence. Materials and methods A multi-centre, retrospective analysis of 414 patients undergoing resectional surgery for sigmoid colon cancer was conducted. Multivariable Cox Proportional Hazard models were created to identify variables associated with local disease recurrence. Patterns of recurrence and prognostic significance of pre and post-operative variables were identified. Results In 414 patients, the local recurrence rate was 12.6%. The R1/R2 rate was 2.4%. Local recurrence occurred most commonly within the peri-anastomotic region (50%), followed by the peritoneum (33%). On multivariate analysis, local recurrence was predicted by pathological T stage (HR 1.15) and R1 resection (HR 2.95), but also computerised tomography (CT) identified tumour deposits (HR 2.40) and local peritoneal infiltration (2.70). Conclusions Contemporary local recurrence rates for sigmoid cancer are high at 12.6%. Outcomes may be improved if local recurrence is reduced at the most common sites such as the peri-anastomotic area or peritoneum. Extra-nodal CT-imaging biomarkers of local peritoneal infiltration and tumour deposits were prognostically significant on multivariate analysis in addition to pathology staging variables.

Background and objectives Prognostic nomograms for patients undergoing resection of retroperitoneal sarcoma (RPS) include the Sarculator and Memorial Sloan Kettering (MSK) sarcoma nomograms. We sought to validate the Sarculator and MSK nomograms within a large, modern multi-institutional cohort of patients with primary RPS undergoing resection. Methods Patients who underwent resection of primary RPS between 2000 and 2017 across nine high-volume US institutions were identified. Predicted 7-year disease-free (DFS) and overall survival (OS) and 4-, 8-, and 12-year disease-specific survival (DSS) were calculated from the Sarculator and MSK nomograms, respectively. Nomogram-predicted survival probabilities were stratified in quintiles and compared in calibration plots to observed survival outcomes assessed by Kaplan-Meier estimates. Discriminative ability of nomograms was quantified by Harrell's concordance index (C-index). Results Five hundred and two patients underwent resection of primary RPS. Histologies included leiomyosarcoma (30%), dedifferentiated liposarcoma (23%), and well-differentiated liposarcoma (15%). Median tumor size was 14.0 cm (interquartile range [IQR], 8.5-21.0 cm). Tumor grade distribution was: Grade 1 (27%), Grade 2 (17%), and Grade 3 (56%). Median DFS was 31.5 months; 7-year DFS was 29%. Median OS was 93.8 months; 7-year OS was 51%. C-indices for 7-year DFS, and OS by the Sarculator nomogram were 0.65 (95% confidence interval [CI]: 0.62-0.69) and 0.69 (95%CI: 0.65-0.73); plots demonstrated good calibration for predicting 7-year outcomes. The C-index for 4-, 8-, and 12-year DSS by the MSK nomogram was 0.71 (95%CI: 0.67-0.75); plots demonstrated similarly good calibration ability. Conclusions In a diverse, modern validation cohort of patients with resected primary RPS, both Sarculator and MSK nomograms demonstrated good prognostic ability, supporting their ongoing adoption into clinical practice.

Background The preoperative evaluation is crucial for diagnosis and surgical plan of retroperitoneum ganglioneuroma (GN). In this study, we reviewed a relatively large series of histopathological proved retroperitoneum GN cases, summarized the imaging features and further depicted risk factors of increased surgical blood loss. Methods A total of 35 (18 male, 17 female) patients were retrospectively enrolled from January 2012 to June 2019 at our institution. Among them, 24 patients had undergone CT scans and 19 patients had undergone MR examination before treatment. The clinical and radiological features were analyzed and the relationships between image features and surgical blood loss were evaluated. Results The media age of the involved 35 patients was 40 years (range, 14–66 years). The histological tumor size was 10.12 ± 4.56 cm for average. Retroperitoneum GN was relatively low density on unenhanced CT images and showed delayed progressive enhancement on enhanced CT and MR images. The whorled sign could be seen in 14 patients. The vessel encasement sign could be found in 17 patients. Univariate analysis revealed maximal tumor size measured on axial image, maximal tumor size measured on coronal image, encasing one or both renal pedicles, encasing the aorta and/or vena cava and whorled sign on MRI showed significant difference between the blood loss ≥ 400 ml and blood loss P = 0.023) and encasing one or both renal pedicles (OR: 22.39; 95% CI: 1.35–372.99; P = 0.030) were independently correlated with surgical blood loss. Conclusions Preoperative CT and MR imaging analysis was valuable for both diagnosis and surgical risk prediction of retroperitoneum GN.

The pure retroperitoneal lipoma is a sporadic benign tumor originating from the mesenchymal tissue. There are only a few data in the literature. The technique available at this moment cannot establish a clear distinction between a benign adipose mass and a low-grade liposarcoma. This study presents the case of a 53-year-old man presenting computed tomography (CT) and magnetic resonance imaging (MRI) findings that revealed a giant retroperitoneal fatty tumor of 365∕210∕165 mm dimensions, suspected of malignancy. The patient's medical history, clinical data, and blood tests are more likely inclined to a benign condition. Liposarcoma couldn't be excluded preoperatively. The patient underwent an open surgical procedure respecting the oncological principles, with complete resection of 6400 g of retroperitoneal adipose tumor that also affected the surrounding organs' normal abdominal anatomy. The tumor was relatively well delimited without any signs of local invasion or infiltration. Three solid masses described on the MRI were identified upon dissection and sent separately for histological examination. The result indicated a giant retroperitoneal lipoma with benign characteristics. Facing imagistic difficulties to distinguish lipoma from liposarcoma clearly, and the inconclusiveness of fine-needle biopsies, oncological resection remains the only option as a diagnostic method and curative treatment.

Objective This investigation aimed to explore the relationship between sarcopenia and severe postoperative complications, relapse-free survival (RFS), and overall survival (OS) in patients with retroperitoneal liposarcoma (RLPS). Material and methods This retrospective study included 72 RLPS patients (47 men, 25 women; mean age, 57.49 years, SD 10.92) who had abdominal CT exams. Clinical information was recorded, including RLPS characteristics (histologic subtypes, grade, size), laboratory assessment (ALB, PALB, A/G, Hb, SCr), relapse-free survival, overall survival, and postoperative complications. The relationships between those variables and RFS and OS were analyzed using Cox proportional hazard models. Results There were 8 severe postoperative complications (Clavien-Dindo grade > 2). The chi-square test showed sarcopenia was associated with severe postoperative complications (P = 0.011). In multivariate analysis, sarcopenia was not associated with relapse-free survival (P = 0.574) and overall survival (P = 0.578). Conclusions Sarcopenia predicts worse surgical complications but does not affect relapse-free survival and overall survival.

Sclerosing angiomyolipoma (sAML) is a rare variant of the perivascular epithelioid tumors exhibiting distinct morphology with extensive stromal hyalinization, which makes it challenging to recognize. It often lacks an adipose tissue component and melanocytic markers may be expressed only focally, further posing a diagnostic challenge. Here, we report a case of sAML of the left pararenal retroperitoneum in a 52-year-old woman with 92 months of clinical follow up and discuss the histologic features, immunoprofile, molecular alterations, and differential diagnoses that can aid in the diagnosis of this unique and rare entity.

Objective: This retrospective study aimed to present our surgical experience in patients with primary retroperitoneal tumors (PRTs) who underwent laparoscopic surgery and to compare the results wit...

Background and purpose Locoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify clinicopathological factors predictive of overall survival (OS) and disease specific survival (DSS) after reoperation for recurrent RPS. Patients and methods We retrospectively collected data from the medical records of 800 patients who underwent resection for sarcoma at our Institution, from 1983 to 2015. Among these patients, 120 were treated for retroperitoneal sarcoma and 55 had a locoregional recurrence (LR). Four of them did not undergo surgery and thus were excluded from this study leaving 51 cases available for data analysis. Univariate and multivariate survival analyses were performed to identify prognostic factors. Results Median overall survival was 33 months. The 1-year, 3-year and 5-year OS rates were 75.5%, 47.1% and 31.6% respectively. Multivariate Cox regression analysis suggested that extension of surgery (P = 0.026), surgical margin status (P = 0.015) and histological grade of recurrent tumor (P = 0.047) were independent prognostic factors for OS. Median DSS was 48 months. The 1-year, 3-year and 5-year DSS rates were 79.2%, 53.1% and 40.9%, respectively. At multivariate analysis, predictors of DSS were extension of surgery (P = 0.004), margin status (P = 0.011), histological grade of recurrent tumor (P = 0.008), and disease free interval (DFI) (P = 0.020). As regards histological subtype of recurrent RPS, at univariate analysis, well-differentiated liposarcoma (WDLS) was associated with better OS and DSS (P = 0.052 and P = 0.016 respectively) compared to dedifferentiated liposarcoma (DDLS). Conclusions According to our findings, surgery is more beneficial in patients with low-grade sarcoma, WDLS and long DFI. The achievement of clear resection margins, rather than performing a multivisceral resection, appears to be a key factor to improve OS and DSS.

Background Most retroperitoneal sarcoma (RPS) operations require combined multi-organ resection, and the proportion of unplanned reoperation is high. However, there are no relevant studies on reoperation for RPS. Methods Patients who underwent at least once unplanned reoperation at Shanghai Public Health Clinical Center, Fudan University, China, from August 2009 to December 2021 were retrospectively analyzed. The baseline characteristics, primary surgery, and reoperation information, postoperative complications, and survival were analyzed. Results A total of 51 patients were included. Among them, 21 (41.2%) were male and 30 (58.8%) were female. The median age was 51 (interquartile range [IQR], 49-63) years. Most (88.3%) had a history of abdominal surgery. Dedifferentiated liposarcoma, well-differentiated liposarcoma, leiomyosarcoma, and others accounted for 50.9%, 21.6%, 15.7%, and 11.8%, respectively. The conditions of the primary operation were as follows: 35 (68.6%) patients achieved complete surgical resection, 48 patients had combined organ resection, and a median of 3 (IQR, 2–4) organs was removed, of which 5 (9.9%) were combined with pancreaticoduodenectomy. The median operative time was 330 (IQR, 245–440) min, and the median estimated blood loss was 1500 (IQR, 500–2600) ml. The median postoperative hospital stay was 42 (IQR, 23–82) days. For reoperation, the most common reasons were bleeding (31.3%), complications related to intestinal anastomosis (27.4%), and intestinal perforation (19.9%). The mortality rate after reoperation was 39.2% (20/51). Twelve (23.5%) patients underwent reoperation at least twice. Conclusions Unplanned reoperation among retroperitoneal sarcoma correlates with established measures of surgical quality.

As the population ages, more elderly patients are receiving surgery for retroperitoneal sarcoma (RPS). However, high-quality data investigating associations between ageing and prognosis are lacking. Our study aimed to investigate whether ageing is associated with inferior short-term survival outcomes after RPS surgery.Patients undergoing surgery for primary RPS between 2008 and 2019 at two tertiary sarcoma centres were analysed. The primary outcome was 1-year mortality, and the primary explanatory variable was patient age, classified as:55, 55-64, 65-74 or 75+ years.The 692 patients undergoing surgery (mean age 60.8 ± 13.8 years) had a 1-year mortality rate of 9.4%, which differed significantly by age (p0.001), with rates of 7.2%, 6.9%, 8.7% and 22.8% for the55, 55-64, 65-74 and 75+ years groups, respectively. The distribution of causes of death also differed significantly by age (p = 0.023), with 22% and 28% of deaths in the 65-74 and 75+ years groups caused by post-operative complications, versus none in the55 and 55-64 years groups. On multivariable analysis, age of 75+ years (versus55 years) was a significant independent predictor of 1-year mortality [odds ratio (OR) 7.05, 95% confidence interval (CI) 2.63-18.9, p0.001]; no significant increase in risk was observed in the 55-64 (OR 0.72, 95% CI 0.28-1.87) or 65-74 (OR 0.89, 95% CI 0.37-2.15) years groups.Post-operative complications are an important cause of deaths in elderly patients. These findings are relevant to decision-making and counselling when surgery is considered for patients with RPS.

A 49-year-old Japanese man had a chief complaint of left hypochondrium pain, and a CT scan revealed a mass in the left retroperitoneal space. CT and MRI scans revealed a hemorrhagic component and surrounding fibrous tissues. FDG PET/CT images showed increased uptake in the peripheral rim of the mass, indicating a malignant tumor. The SUV max was 7.6. We surgically resected the mass. The pathological examination confirmed the diagnosis of chronic expanding hematoma. It is difficult to differentiate CEH from malignant tumors on imaging; this should be recognized as a diagnostic pitfall.

Background Soft-tissue sarcomas are rare malignancies that consist of many different histologic subtypes and arise in various locations in the body. In patients with lung metastases from retroperitoneal sarcomas, the long-term outcomes and prognostic factors are unknown. This study is a retrospective review of patients undergoing pulmonary metastasectomy for retroperitoneal sarcoma metastases at one institution, with the purpose of determining prognostic factors and clinical outcomes. Methods This is a single-center, retrospective cohort study of patients undergoing pulmonary metastasectomy for lung metastases from various sarcomas at Okayama University Hospital from January 2006 to December 2018. The Kaplan-Meier method and log-rank test were used for the analyses, and cut-off values of continuous variables were determined by a receiver operating characteristic curve analysis. Results Twenty-four patients underwent the first pulmonary metastasectomy for lung metastases from retroperitoneal sarcoma in our hospital. Leiomyosarcoma was the most common histologic subtype of retroperitoneal sarcoma (79.2%, n = 19). Median overall survival was 49.9 months, and the 3-year and 5-year survival rates after the first pulmonary metastasectomy were 62.5% and 26.4% respectively. In univariate analysis, age ≥56 years, disease-free interval < 15 months, and size of metastasis (≥ 27 mm) were associated with poor survival. Conclusion Pulmonary metastasectomy can be considered as an effective management strategy in retroperitoneal sarcoma patients with lung metastases in appropriately selected cases, just as it is for other sarcomas.

Background and objectives Caval leiomyosarcomas (cLMS) are rare soft tissue sarcomas historically associated with high recurrence rates and poor prognosis. While radical resection remains the mainstay of therapy for cLMS, new systemic therapies have presented opportunities for multimodality treatment. We examined the clinical outcomes of patients with cLMS treated with modern, multimodality approaches, and compared their outcomes to those of patients with noncaval retroperitoneal LMS (ncLMS). Methods A retrospective, single-institution review identified all patients diagnosed with primary retroperitoneal LMS from 2012 to 2018. Radiographic and pathologic review distinguished patients with cLMS and ncLMS. Standard clinicopathologic variables and response to chemotherapy (when applicable) were analyzed. Primary endpoints were overall (OS) and progression-free survival (PFS). Results Eleven patients with cLMS were identified. Median tumor size was 7.5 cm (IQR, 5.0-14.3 cm); all patients had Stage II/III disease. Seven patients received neoadjuvant chemotherapy. Nine cLMS patients underwent R0/R1 resection; two did not complete resection. Six patients received adjuvant systemic therapy. Twenty patients with ncLMS were treated during the same period. No statistical intergroup differences were noted in tumor size, pathologic grade, stage, or resection margin status. Patients with ncLMS were less likely to receive neoadjuvant (10% vs. 64%) and adjuvant chemotherapy (30% vs. 55%). Two-year OS (81% vs. 78%; p = NS) and PFS (55% vs. 46%; p = NS) were comparable between cLMS and ncLMS patients. Conclusions Multimodality treatment with systemic therapy and aggressive surgical resection may achieve equivalent survival outcomes for patients with cLMS versus similar ncLMS. We recommend that all patients with cLMS be evaluated for multidisciplinary treatment. Genomic and proteomic expression profiling may identify novel or targetable mutations.

BACKGROUND: Prior studies evaluating the impact of adjuvant or neoadjuvant radiotherapy on clinical outcomes in retroperitoneal liposarcoma have been underpowered. METHODS: We queried the National Cancer Database for patients undergoing resection of retroperitoneal liposarcoma from 2004 to 2016. Cox proportional hazards modeling stratified by tumor size was used to identify factors associated with overall survival. RESULTS: 4018 patients met inclusion criteria. 251 had small (10 cm) tumors. Positive surgical margins were correlated with risk of death across all tumor size categories (10 cm HR 1.30, CI [1.12, 1.51]). Adjuvant radiotherapy was associated with improved survival for patients with large tumors only (HR 0.75, CI [0.64, 0.89]). CONCLUSIONS: In retroperitoneal liposarcoma, adjuvant radiation is associated with improved survival only for patients with tumors larger than 10 cm. Radiation should be used sparingly in patients with smaller tumors. SUMMARY: The use of radiotherapy in the management of retroperitoneal sarcoma remains controversial. We isolated retroperitoneal liposarcomas only and identified a survival benefit from radiotherapy treatment only in tumors larger than 10 cm and only in the adjuvant setting.

Objective To assess the response of chemotherapy on the primary tumor, compare it with the response in retroperitoneal disease, and study factors associated with pathological complete response. Methods We conducted a retrospective audit of all high inguinal orchidectomies (HIOs) performed after chemotherapy between 2012 and 2019 at a tertiary cancer center in India. Patient characteristics and histopathological response were extracted from electronic medical records, and predictors of testicular disease response were assessed. Results Of the 260 retroperitoneal lymph node dissections (RPLNDs) performed in the study period, 37 HIOs (14.23%) were carried out after chemotherapy. The median age of presentation was 28 years (16-41). Histopathology was divided into a viable tumor, mature teratoma, and necrosis/scarring. Residual disease was seen in 17 RPLND (46.0%) and 18 HIO (48.6%) specimens respectively. Of these 18, three patients had a residual viable tumor in the testis, and the remaining had a mature teratoma. Clinico-radiological assessment showed an average reduction of 61% in testicular disease size following chemotherapy. On orchidectomy histopathological assessment, the median tumor size was 9, 4, and 1.5 cm in specimens with a viable tumor, mature teratoma, and necrosis/scarring, respectively. Conclusions A low threshold for upfront chemotherapy in patients with a high disease burden may be considered as tumors within the testis respond to chemotherapy in more than half of the patients. Discordance rates of residual cancer in RPLND and HIO specimens exist but post-chemotherapy tumor size in testis correlates with the presence of a residual viable tumor.

Doxorubicin is one of the most active drugs for sarcoma. Pegylated liposomal doxorubicin (PLD) is a unique formulation of doxorubicin, which carries a more favorable toxicity profile in comparison with free doxorubicin. The main toxicity of PLD is hand-foot syndrome. Unlike free doxorubicin, PLD is unlikely to cause alopecia, nausea, myelosuppression, or cardiotoxicity. Additionally, no premedications are required. We describe the case of a 50-year-old man with advanced retroperitoneal liposarcoma who developed irreversible PLD-associated progressive renal failure requiring chronic hemodialysis due to a thrombotic microangiopathy. No cardiotoxicity was noted 84 months after he initiated PLD. This case describes a lesser known toxicity of PLD and may be a toxicity of long-term treatment with other liposomal drugs.

We aimed to explore the clinicopathologic and histologic characteristics, as well as the (differential) diagnosis of retroperitoneal malignant solitary fibrous tumors (RMSFTs) in this study. Nine cases of RMSFTs were recruited and identified by an experienced pathologist from the Pathology Department of Beijing Shijitan Hospital. Clinical information was extracted from medical records and obtained by phone calls. A systematic review of published literature on RMSFTs was conducted using PubMed. A pre-specified search strategy was adopted using the key words "solitary fibrous tumor" and "retroperitoneum." Case reports and literature published in the China Academic Journals (CNKI) and WAN FANG databases were also included. In total, 58 patients (33 males and 25 females) were included; their age ranged from 17 to 83 years, with a median age of 52 years. The tumor size ranged from 4 to 36 cm, and most patients had abdominal masses and pain. Of these patients, 56 underwent surgical resection, and two patients died and underwent an autopsy. All patients were followed up for up to 288 months (with a median follow-up of 36 months). RMSFTs are extremely rare. Their diagnosis mainly relies on the histological morphology and the expression profiles of a panel of pathologic molecules measured by immunohistochemistry. Diagnosis of RMSFTs is usually based on the expression of biomarkers such as vimentin, CD34, Bcl-2, CD99, and STAT6. Differential diagnosis includes spindle-shaped cell tumors, such as schwannoma, gastrointestinal stromal tumors, dermatofibrosarcoma protuberans, synovial sarcoma, malignant peripheral nerve sheath tumors, and fibrosarcoma. RMSFTs are prone to recur and even metastasize. Complete resection remains a major treatment, and close follow-up is highly recommended.

Melanotic schwannoma is an uncommon nerve sheath neoplasm. Only 10% of melanotic schwannoma are malignant whereas metastases from melanotic schwannoma are much less common. Hereby we present FDG PET/CT findings of recurrent left retroperitoneal melanotic schwannoma with widespread metastases in a 50-year-old man.

Treatment options for unresectable local recurrence or metastatic well-differentiated/dedifferentiated liposarcoma (WDLS/DDLS) remain limited. Different liposarcoma subtypes have varying clinical features and sensitivities to treatment regimens. The multitarget tyrosine kinase inhibitors (TKIs), such as pazopanib and regorafenib, have been approved for use in nonadipocytic soft tissue sarcomas (STS). Anlotinib, another TKI, has been approved in China for treating metastatic STS that has progressed after the use of anthracycline-based regimens. In this study, we aimed to evaluate the role of anlotinib in the treatment of local recurrence or metastatic WDLS/DDLS. From August 2018 to June 2020, 17 patients with unresectable local recurrence or metastatic WDLS/DDLS treated with anlotinib in our center were included. The follow-up cutoff time was set as 20 October 2020. Baseline and observation indicators were collected and analyzed. Estimated median progression-free survival (PFS) was 27.9 weeks, the PFS rate at 24 weeks was 58.8%, overall survival (OS) was 56.6 weeks, the disease control rate was 64.7% and no complete response or partial response was detected. Grade 3/4 adverse events occurred in four cases and could be managed. Anlotinib is a potential treatment option for unresectable local recurrence or metastatic WDLS/DDLS.

Background Given the rarity of retroperitoneal soft tissue sarcoma, few studies have assessed if radical excision of retroperitoneal soft tissue sarcoma with adjacent organs improves survival outcomes. This propensity score–matched study aimed to evaluate the impact of radical excision versus resection of tumor alone. Methods The National Cancer Database 2004 to 2015 was used to assess short- and long-term outcomes of resection of tumor alone versus radical excision (tumor plus ≥1 adjacent organs) via 1:1 propensity-matched analyses. Subgroup analyses included low-grade, high-grade, liposarcoma, leiomyosarcoma, adjacent organ involvement alone, localized tumors alone, and high-volume hospitals (≥10 resections/y). Multivariable logistic regression models identified factors associated with radical excision. Results Comparison of propensity-matched groups (N = 1,139/group) revealed no significant differences in 30-day mortality, 90-day mortality, or overall survival (for all, P > .580). For all subgroup analyses comparing resection of tumor alone with radical excision, including localized tumors without organ invasion (N = 208/group), there were no identified differences in short- or long-term survival. Although it yielded lower R2 resection rates (P = .007), radical excision was associated with greater mean length of stay (P Conclusion Radical excision was not associated with improved retroperitoneal soft tissue sarcoma survival irrespective of grade, histology, hospital volume, or adjacent organ involvement. Resection of ostensibly involved adjacent viscera may increase morbidity without survival benefit. These results inform ongoing discussion regarding histology-tailored, situation-specific extent of retroperitoneal soft tissue sarcoma resections.

Aim To study the applicability of 2 common nomograms in retroperitoneal sarcoma patients treated in an Asian tertiary hospital, and to study relapse patterns. Methods Patients from 1st January 2000 to 31st December 2017 were retrospectively analysed. R2 resection from an external institution were ineligible. Harrel's C concordance index and calibration plots were used to assess nomograms' accuracy. Results 109 patients were eligible, with a median age of 56, median tumour size of 16 cm and median follow up of 44.1 months. Most common subtypes were DDLPS (46.8%), LMS (19.3%) and WDLPS (13.8%). Overall, 81.1% had passed away from sarcoma. Recurrence patterns differed with subtypes; 6.7% of WDLPS and 66.7% of LMS had distant relapses. WDLPS patients had a median sarcoma specific survival of 139 months (122-NA), while the rest had a median SSS of 72.3 months (59.7-NA). The 5-years and 7-year OS were 63.0% (95% CI: 53.6%–74.0%) and 51.6% (41.1%–64.8%) respectively. The 5-years and 7-year DFS were 22.4% (15.5%–32.3%) and 17.5% (11%–27.8%) respectively. The 4-years, 8-years and 12-year SSS were 71.3% (62.7%–81.0%), 51.8% (40.8%–65.8%) and 32.2% (19.7%–52.6%) respectively. The concordance indices for 7-year DFS and OS (Sarculator) were 0.63 and 0.73. The concordance indices for 4-, 8- and 12-year SSS (MSKCC) were 0.64, 0.72 and 0.72. Conclusion Both nomograms were effective in our cohort. Both nomograms can be further improved by specifying the type of relapse and including a longer-term endpoint for the Sarculator.

BackgroundMetastatic germ cell cancer of the testis is characterized by favorable prognosis since effective treatment methods are available even in cases of extensive disease. Retroperitoneal masses frequently encroach major blood vessels requiring a vascular intervention usually performed in association with the post-chemotherapy retroperitoneal lymph node dissection (RPLND). Reported clinical case describes a successful pre-treatment endovascular surgery for abdominal aortic rupture allowing for full-dose systemic chemotherapy administration, and subsequent radical surgical intervention at primary tumor site as well as metastatic retroperitoneal lymph node dissection including the reconstruction of inferior caval vein.Case presentationPatient presented with left-sided testicular tumor and voluminous retroperitoneal mass with vascular involvement. Soon after the patient had been admitted for the first cycle of cisplatin-based chemotherapy, computed tomographic angiography (CTA) revealed a dorsal aortic wall rupture with active extravasation and irregular pseudoaneurysmatic dilatation of the aorta below the leak area. Retroperitoneal intratumoral hemorrhage associated with the bilateral iliac venous thrombosis required an endovascular repair procedure of infrarenal abdominal aorta.ConclusionsFollowing the successful endovascular aortic repair 3 cycles of BEP (bleomycin, etoposide, cisplatin) regimen were administered with subsequent delayed left radical orchiectomy and RPLND associated with vena cava inferior (VCI) resection. Reconstruction of VCI was originally not deemed necessary as collateral blood flow appeared sufficient, however, intraoperative complications resulted in the need for unilateral VCI reconstruction, using the interposed bypass between right common iliac vein and infrarenal segment of VCI. Histopathologic examination of the attained specimen detected no vital cancer structures. The patient remains disease-free 18 months after the RPLND.

BackgroundParagangliomas (PGLs) are extremely rare neuroendocrine tumours arising from extra-adrenal chromaffin cells. PGLs are clinically rare, difficult to diagnose and usually require surgical intervention. PGLs mostly present catecholamine-related symptoms. We report a case of Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal PGL. There has been only one similar case reported in literature.Case presentationWe present a unique case of a 52-year-old female with acute abdomen induced by haemorrhagic retroperitoneal PGL. The patient had a 5-h history of sudden onset of serve right lower quadrant abdominal pain radiating to the right flank and right lumbar region. Patient had classic symptoms of acute abdomen. Abdominal ultrasound revealed a large abdominal mass with a clear boundary. A Computed Tomography Angiography (CTA) of superior mesenteric artery was also performed to in the emergency department. The CTA demonstrated a large retroperitoneal mass measured 9.0 × 7.3 cm with higher density inside. A provisional diagnosis of retroperitoneal tumour with haemorrhage was made. The patient received intravenous fluids, broad-spectrum antibiotics and somatostatin. On the 3rdday of admission, her abdominal pain was slightly relieved, but haemoglobin decreased from 10.9 to 9.4 g/dL in 12 h suggesting that there might be active bleeding in the abdominal cavity. Thus, we performed a midline laparotomy for the patient. Haemorrhage was successfully stopped during operation. The retroperitoneal tumour with haemorrhage was completely removed. The abdominal pain was significantly relieved after surgery. The patient initially presented with acute abdomen instead of catecholamine-related symptoms. The diagnosis of retroperitoneal PGL with haemorrhage was finally confirmed by postoperative pathological and immunohistochemical results. The postoperative course was uneventful. At the 1-year follow-up visit, no tumour recurrence was observed by Single Photon Emission Computed Tomography. A literature review was performed to further understand and analyse the aforementioned disease.ConclusionAcute abdomen as the initial manifestation of haemorrhagic retroperitoneal paraganglioma is extremely rare. Abdominal Computed Tomography is essential to locate the lesion and differentiate between other causes of acute abdomen. PGLs are hypervascular tumours. We should be aware that ruptured retroperitoneal PGL with massive bleeding could be life threatening and require emergency laparotomy.

Objective To determine feasibility and safety of robotic excision of local ipsilateral recurrences after nephrectomy for renal cell carcinoma (RCC). Surgical resection is an option for treatment of low burden locally recurrent RCC, potentially delaying the use of systemic therapy. This has historically been performed by open technique, which can impart significant morbidity. We present our experience with robotic excision. Methods We reviewed our institutional experience of patients with surgically excised RCC who underwent robotic excision of ipsilateral retroperitoneal recurrence in 2015-2018. Demographics and clinicopathological variables, including operative and postoperative outcomes, were examined. Results Twelve robotic excisions of ipsilateral local recurrences were performed in our hospital in 2015-2018. Mean age was 65.48 years (± standard deviation, SD: 9.51), 10 patients were male, and mean BMI 34.75 kg/m2 (± 6.71). Nine patients recurred after radical nephrectomy, and 3 after partial nephrectomy. Mean size of recurrence was 2.97 cm (±1.69). Mean anesthesia time, EBL, and LOS were 213 minutes (± 38.92), 152 mL (± 130.75), and 43 hours (± 12.64), respectively. All surgical margins were negative. No surgical complications were reported. Median follow-up was 19.0 months [interquartile range, IQR 12.7-30.0]. Five patients out of 12 recurred following robotic excision, these were treated with either systemic therapy, radiation, or palliative surgeries. Mean time for subsequent recurrence was 26.5 months. Conclusion In this small case series, robotic excision of ipsilateral RCC retroperitoneal recurrence appears safe, technically feasible, and oncologically sound in expert hands and carefully selected patients.

Background Cabozantinib Versus Sunitinib as Initial Targeted Therapy for Patients With Metastatic Renal Cell Carcinoma of Poor or Intermediate Risk: The Alliance A031203 CABOSUN Trial (CABOSUN) was a randomized, open‐label, phase 2 trial evaluating first‐line cabozantinib versus sunitinib in patients with advanced renal cell carcinoma (aRCC). This post hoc analysis evaluated quality‐adjusted survival using Quality‐adjusted Time Without Symptoms of disease or Toxicity of treatment (Q‐TWiST). Methods Survival plots for cabozantinib and sunitinib (650‐day follow‐up) were partitioned into 3 health states: time spent before disease progression without toxicity (TWiST; toxicity based on National Cancer Institute Common Terminology Criteria for Adverse Events [version 4.0] grade 3/4 adverse events), time spent before disease progression with toxicity (TOX; durations of adverse events based on published literature), and time after disease recurrence (relapse) or progression to death (REL). Q‐TWiST was the sum of the mean time spent in each state, with each state weighted to reflect patient preferences (from 0 [worst] to 1 [best]) using utility scores. TWiST was always weighted as 1. Overall survival and time to disease progression were based on all randomized patients (157 patients); TOX was based on all randomized and treated patients (150 patients). Results Across all utility combinations tested, Q‐TWiST was found to be longer with cabozantinib versus sunitinib (range of differences, +24 days to +137 days). Q‐TWiST differences that were found to be statistically significant (+92 days [95% confidence interval, 5‐178 days] to +137 days [95% confidence interval, 60‐214 days]) were of a clinically meaningful effect size (≥80 days), and were based on utility values that included those considered relevant for patients with aRCC (REL utility weight of 0.355, TOX utility weight of 0‐1, and TWiST utility weight of 1). Conclusions In patients with aRCC, first‐line cabozantinib was found to provide longer quality‐adjusted survival compared with sunitinib. These findings may help to inform clinical decision making. Lay Summary Cabozantinib and sunitinib are drugs that are used to treat patients with advanced kidney cancer. Clinical trials have shown that cabozantinib offers benefits over sunitinib, giving patients more time before their cancer progresses.It is important that this additional time before disease progression does not come at the expense of patients' quality of life, which can be affected by treatment side effects and/or ongoing cancer symptoms. Both quantity and quality of life are central to optimal treatment.In the current analysis of patients with advanced kidney cancer who were initiating treatment for the first time, cabozantinib provided more quality time before cancer progression compared with sunitinib., The current post hoc analysis of the Cabozantinib Versus Sunitinib as Initial Targeted Therapy for Patients With Metastatic Renal Cell Carcinoma of Poor or Intermediate Risk: The Alliance A031203 CABOSUN Trial (CABOSUN) finds that cabozantinib offers statistically and clinically significant improvements in quality‐adjusted survival compared with sunitinib in treatment‐naive patients with advanced renal cell carcinoma (aRCC). This finding may help to inform clinical decision making in the first‐line treatment of patients with aRCC.

Introduction Retroperitoneal sarcoma (RPS) is a rare malignancy, and curative resection is considered the main therapy. Use of chemotherapy and/or radiation in addition to surgery (multimodality therapy) is controversial. Objective To determine treatment factors that influence overall survival in RPS. Methods This retrospective Institutional Review Board-approved study identified patients with RPS treated at a single institution between 2000 and 2017. Patient, tumor, and treatment modalities were collected. Prism (v.8.2.1) was used to calculate Kaplan-Meier survival curves. Results There were 695 patients with sarcoma between 2000 and 2017, and 61 adults had RPS. The mean age was 59 (range 31-86) years, with 57.4% females (n = 35). Patients were 68.9% Caucasian (n = 42), 21.3% Hispanic (n = 13), 8.2% black (n = 5), and 1.6% Asian (n = 1). There were 4 patients who had neoadjuvant therapy (chemotherapy, n = 3; radiation, n = 2) and 17 who had adjuvant therapy (chemotherapy, n = 6; radiation, n = 14). There was no significant difference in survival between the groups who received multimodality therapy compared to surgery alone. There was a significant improvement in the median overall survival for patients who underwent one or multiple surgeries ( P < .05). Conclusions These institutional data suggest that treatment factors associated with overall survival included multiple resections. Use of multimodality therapy was low and did not influence overall survival in patients with RPS compared to surgery alone.

BackgroundVitreoretinal lymphomas are difficult to diagnose due to their insidious onset and inaccessible focal points. Natural killer/T-cell derived malignancies are rare as intraocular lymphomas and usually have a rapid progression and a poor prognosis. Therefore, it is essential to make a definite diagnosis, especially differentially with B-cell-derived lymphomas, which account for most cases of vitreoretinal lymphomas.Case presentationThis case report describes a 55-year-old female reporting a 10-month history of painless decline in her vision of the right eye. Optical coherence tomography of the patient revealed hyperreflective nodules and irregular humps in the retinal pigment epithelium layer. The right vitreous was aspirated for diagnostic assessment, revealing an interleukin-10 level of 39.4 pg/mL and an interleukin-10/interleukin-6 ratio of 1.05. The right vitreous humor was positive for Epstein–Barr virus DNA. Upon a systemic examination, a high metabolic nodule was found in the retroperitoneal area and proven to be positive for Epstein–Barr virus-encoded mRNA, CD2, CD3ε, TIA-1, and Ki-67. Considering the homology of the two lesions, the patient was diagnosed with metastatic vitreoretinal lymphoma secondary to retroperitoneal extranodal natural killer/T-cell derived lymphoma. The patient received systemic chemotherapy and regular intravitreal injections of methotrexate. Her visual acuity of the right eye had improved from 20/125 to 20/32 at the latest follow-up. No new lesions were found.ConclusionsA definitive diagnosis of vitreoretinal lymphoma is challenging. On some occasions in which pathological evidence is missing, the available examination results and clinical observations must be comprehensively considered. This study herein summarized pertinent pieces of literature and reports and reviewed available practicable methods to make a definitive diagnosis of intraocular extranodal natural killer/T-cell lymphoma, which was particularly distinct from the common diffuse large B-cell lymphomas.

Palbociclib has been evaluated in early phase trials for well-differentiated liposarcoma (WDLPS) and dedifferentiated liposarcoma (DDLPS) patients, with reported median progression-free survival (PFS) of 18 weeks. Here, we report on real-world use and surgical outcomes associated with palbociclib treatment. We retrospectively reviewed 61 consecutive patients with retroperitoneal WDLPS (n = 14) or DDLPS (n = 47) treated with palbociclib monotherapy between 1 March 2016 and 28 February 2021 at The University of Texas MD Anderson Cancer Center. At palbociclib initiation, median age was 64 (interquartile range [IQR] 56-72). In WDLPS and DDLPS cohorts, the median number of prior systemic treatments was 0 (IQR 0-0) and 2 (IQR 0-4), respectively. Median number of prior surgeries was 2 (WDLPS IQR 1-2.75) and 2 (DDLPS IQR 1-3). Median PFS was 9.2 (WDLPS IQR 3.9-21.9) and 2.6 months (DDLPS IQR 2.0-6.1), with median time on treatment of 7.4 months (WDLPS IQR 3.5-14.2) and 2.7 months (DDLPS IQR 2.0-5.7). Twelve patients ultimately underwent surgical resection. Resections were macroscopically complete (R0/R1) in half (n = 6/12), among whom only one patient experienced relapse after resection (median follow-up 7.5 months). All patients who underwent macroscopically incomplete resections progressed after surgery with median time to progression of 3.3 months (IQR 2.3-4.4). Surgery after palbociclib treatment was not associated with improved overall survival. Efficacy of palbociclib monotherapy for patients with advanced WDLPS and DDLPS is disappointing. While palbociclib may have been used to delay surgery, there was no clear benefit from treatment and few patients achieved prolonged tumor control.

A 54-year-old Japanese woman developed simultaneous abdominal distension and bilateral leg edema. Her medical history and results of periodic medical check-up were unremarkable. Blood tests revealed severe hypoproteinemia and acute kidney injury, and urinalysis revealed 4+ proteinuria and 2+ hematuria. Abdominal computed tomography revealed a large intra-abdominal mass with fat tissue density. She underwent emergency tumor excision, splenectomy, and distal pancreatectomy. However, hypoproteinemia and acute kidney injury worsened. Therefore, she was transferred to the nephrology division for confirmation of diagnosis and for treatment of acute kidney injury and nephrotic syndrome. We conducted percutaneous kidney biopsy and diagnosed minimal change disease (MCD). Intravenous prednisolone was started, and heavy proteinuria and systemic edema were gradually alleviated. She achieved complete remission 2 months later, and oral prednisolone was tapered. Histopathological diagnosis of abdominal tumor was dedifferentiated liposarcoma of retroperitoneal origin. Immunohistochemical staining revealed strong expression of vascular endothelial growth factor in the tumor cells in the dedifferentiated component. Currently, her clinical course is stable without recurrence of liposarcoma and nephrotic syndrome. MCD develops in patients with Hodgkin’s lymphoma, solid organ cancers, hematological malignancies, and thymoma, whereas concurrent MCD and liposarcoma are rare. Remission of nephrotic syndrome and normalized kidney function induced by steroid therapy are important for better management of patients with malignancy.

Background: Hepatic metastasis of soft tissue sarcoma is rare compared to lung metastasis, and the literature is scarce. We examined the risk of hepatic metastasis according to the site of occurrence and histological type. Methods: From a Hospital-based Cancer Registry, 658 patients registered between 2007 and 2017 with soft tissue sarcomas were evaluated. The exclusion criteria were gastrointestinal stromal tumors, tumors of unknown origin, and follow-up periods of less than 1 month. SPSS 25 was used for statistical analysis. Results: The risk of hepatic metastasis was significantly higher in the retroperitoneum (HR, 5.981; 95% CI, 2.793-12.808) and leiomyosarcoma (HR, 4.303; 95% CI, 1.782-10.390). Multivariate analysis showed that the risk of hepatic metastasis as first distant metastasis was high in leiomyosarcoma (HR, 4.546; 95% CI, 2.275-9.086) and retroperitoneal onset (HR, 4.588; 95% CI, 2.280-9.231). The 2-year survival rate after hepatic metastasis was 21.7%. Conclusions: The onset of hepatic metastasis indicates a poor prognosis. However, hepatic metastasis from retroperitoneal sarcoma and leiomyosarcoma may be the first distant metastasis in some cases. For retroperitoneal sarcoma and leiomyosarcoma, additional screening for hepatic metastasis such as contrast CT should be considered during staging and follow-up after treatment., Article, Cancer medicine 17(17) : 6159-6165(2020)

Background Retroperitoneal sarcoma represents 15% of sarcomas. The mainstay of treatment is surgery where a majority of patients require multi-visceral resections that may significantly impact their quality of life (QOL) following surgery. Studies in other cancers have shown that QOL may not be significantly impacted after radical or extensive surgery. However, there are limited studies examining the QOL specifically in patients with retroperitoneal sarcoma. In this pilot study, we retrospectively evaluated the QOL of patients with retroperitoneal sarcoma. Methods 32 out of 90 patients who underwent surgical intervention for retroperitoneal sarcoma in National Cancer Centre Singapore from January 1999 to August 2018 who were alive and on follow-up were included in this study. EORTC-QLQ-C30 was administered to the patients. Results The median age of our patients was 59 years (range, 35–84), and median time from surgery to the implementation of questionnaire was 2.5 years (range, 0.05–9.6). Younger patients had significantly better differences in global health, physical and role functioning scores as compared to older individuals. Female patients reported higher global health, physical, emotional and social functioning scores than males. Patients who were more than 2 years post-surgery exhibited better QOL scores as compared to those who had more recent surgery. Our patients had comparable global health and functioning scores compared to a reference group of outpatient cancer patients at our institution. Conclusions Our pilot study investigating the QOL of patients with retroperitoneal sarcoma has shown that patients need to be followed up for at least 2 years following surgery to evaluate their QOL. In general, they achieved better functioning scores when compared with other cancer patients. These findings support the need for larger-scale prospective studies to further evaluate the QOL of these patients.

Objective This study sought to investigate the characteristics of primary and repeated recurrent retroperitoneal liposarcoma. Methods Patients treated with primary or recurrent retroperitoneal liposarcoma between 2005 and 2018 were retrospectively reviewed. Survival time analysis of recurrence-free survival and overall survival was conducted using Kaplan–Meier analysis and log-rank test. Results Fifty-two patients with primary retroperitoneal liposarcoma were analysed. Amongst them, 46 patients (88%) had undergone surgery. Histologic grades included well-differentiated (n = 21), dedifferentiated (n = 21), myxoid (n = 3) and pleomorphic (n = 1) subtypes. The patients undergoing R0 resection in the first surgery had significantly higher recurrence-free survival rates compared with the patients undergoing non-R0 resection (3-year recurrence-free survival: 80 versus 38%; 5-year recurrence-free survival: 49 versus 29%, P = 0.033). Although overall survival rates tended to be higher in the patients undergoing R0 resection compared with the non-R0 resection, it did not reach to a statistical significant difference (5-year overall survival: 93 versus 75%; 10-year overall survival: 93 versus 59%, P = 0.124). The recurrence rates were 65, 67, 73 and 100%, and the median recurrence-free survival times were 46, 20, 9 and 3 months after the first, second, third and fourth surgeries, respectively. The 5-year overall survival rates were 82, 69, 40 and 0% after the first, second, third and fourth surgeries, respectively. Conclusions With repeated recurrence and surgeries, the time to recurrence decreased and the recurrence rate increased. R0 resection in the first surgery was considered the most important for longer recurrence-free survival and radical cure.

BACKGROUND The novel composite metric textbook outcome (TO) has increasingly been used as a quality indicator but has not been reported among patients undergoing surgical resection for retroperitoneal sarcoma (RPS) using multi-institutional collaborative data. METHODS All patients who underwent resection for RPS between 2000 to 2016 from eight academic institutions were included. TO was defined as a patient with R0/R1 resection that discharged to home and was without transfusion, reoperation, grade ≥2 complications, hospital-stay >50th percentile, or 90-day readmission or mortality. Univariate and multivariable analyses were performed. RESULTS Among 627 patients, 56.1% were female and the median age was 59 years. A minority of patients achieved a TO (34.9%). Factors associated with achieving a TO were tumor size

Introduction Short-term outcomes after distal pancreatectomy (DP) for retroperitoneal (RP) tumors are unknown. We sought to identify rates of postoperative pancreatic fistula (POPF) and morbidity after en bloc DP with RP tumor resection. Methods A retrospective review of 43 patients who underwent DP with RP tumor resection (1/2011–12/2017) was performed. Results Seventeen patients had RP sarcoma, 12 renal cell carcinoma, 11 gastrointestinal stromal tumor, and 3 adrenocortical carcinoma. Grade III-IV complications occurred in 7 patients. Grade B POPF occurred in 14 patients, grade C POPF in none, and biochemical leak in 6. Of 22 patients who developed radiographically evident peri-pancreatic fluid collections, 7 required percutaneous drainage. The 90-day readmission rate was 33%. Conclusions DP with RP tumor resection is associated with high rates of clinically relevant POPF compared to historical results for DP for primary pancreatic tumors. Multi-center studies to identify targetable predictors and risk mitigation strategies for POPF in this rare high-risk population are needed.

Background Elevations in inflammatory biomarkers, including neutrophil-to-lymphocyte ratio (NLR) or platelet-to-lymphocyte ratio (PLR), are reportedly associated with decreased overall survival (OS) or recurrence-free survival (RFS) in patients with numerous cancers. A large multicenter sarcoma data set was used to determine if elevated NLR or PLR was associated with worse survival and can guide treatment selection. Materials and methods A total of 409 patients with a primary retroperitoneal sarcoma (n = 268) or truncal (n = 141) sarcoma from 2000 to 2015 were analyzed using the US Sarcoma Collaboration database. Binary NLR and PLR values were developed using receiver operating characteristic curves. Kaplan-Meier model and Cox proportional hazards model identified predictors of decreased OS and RFS. Point biserial analyses were used to correlate binary and continuous data. Results Neither elevated NLR nor PLR was predictive of decreased OS or RFS. These findings persisted despite exclusion of comorbid inflammatory conditions. Further, NLR and PLR were not correlated with tumor grade. In multivariate models, decreased RFS was associated with tumor factors (e.g., positive margins, tumor grade, tumor size, necrosis, positive nodes); decreased OS was associated with histologic subtype, male gender, and nodal involvement. Conclusions Although several small studies have suggested that elevated NLR and PLR are associated with decreased survival in patients with abdominal or truncal sarcoma, this large multicenter study demonstrates no association with decreased OS, decreased RFS, or tumor grade. Rather, survival outcomes are best predicted using previously established tumoral factors.

Retroperitoneal liposarcoma (RLPS) is one of the most common types of retroperitoneal sarcomas, and has a high recurrence rate. There is an urgent need to further explore its pathogenesis and develop more effective treatment strategies. The aim of the present study was to identify potential driver genes of RLPS through bioinformatics analysis and molecular biology to elucidate potential targets that are suitable for further analysis for the treatment of RLPS. Differentially expressed genes (DEGs) between liposarcoma and normal fatty (NF) tissues were identified based on microarray data through bioinformatics analysis, and thymidylate synthase (TYMS) was selected from the DEGs, based on high content screening (HCS). TYMS expression was evaluated in RLPS tumor tissues and cell lines. A total of 21 RLPS tissues and 10 NF frozen tissues were used for reverse transcription-quantitative PCR, and 47 RLPS formalin-fixed specimens were used for immunohistochemical analysis. The effect of TYMS downregulation on cell proliferation, apoptosis, cell cycle progression, and cell migration and invasion were evaluated using lentivirus-mediated short hairpin RNA. The underlying mechanisms of TYMS in RLPS were examined by protein microarray and verified by western blotting. A total of 855 DEGs were identified. TYMS knockdown had the most notable effect on the proliferative capacity of RLPS cells according to the HCS results. TYMS mRNA expression levels were higher in RLPS tissues compared with NF tissues (P

BACKGROUND. Resection of recurrent retroperitoneal well-differentiated liposarcoma (RP-WDLPS) is unlikely to result in cure. Thus, most clinicians delay surgery after recurrence until symptom intolerance or increasing rate of disease progression. The aim of this study was to determine whether longer surveillance intervals in this population would impact outcomes or delay treatment in those who recur. METHODS. A retrospective review of patients with primary RP-WDLPS who underwent resection between April 1996 and April 2017 and surveillance at MDACC (n = 91) was performed. RESULTS. Median age at diagnosis of primary RP-WDLPS was 61 years; median tumor size was 30 cm. Complete resection was achieved in 85 (93.4%) patients. Among patients who underwent complete resection, recurrence occurred in 53 (60.2%) with median time to recurrence of 27.0 months. Thirty-six (69.6%) of these patients underwent resection of recurrent disease at a median 40.2 months from primary tumor resection. Surveillance imaging at 4-month (vs 3-month) intervals would not have impacted recurrence management in 84 (95.5%) patients; imaging at 6-month (vs 3-month) intervals would not have impacted management of recurrence in 80 (90.9%). CONCLUSIONS. Recurrence was common, often occurring beyond the early postoperative period following primary RP-WDLPS resection. More frequent surveillance imaging (q3–4 vs q6 months) in the first 2 years following primary RP-WDLPS resection may not significantly impact timing of surgery or systemic therapy for recurrence. If longer surveillance intervals were shown to be safe with equivalent outcomes in prospective studies, the resulting change in practice could lead to decreased anxiety and cost for patients and healthcare systems.

Paragangliomas (PGLs) arise from chromaffin cells of the extra-adrenal sites along the sympathetic and/or the parasympathetic chain. Majority of the PGLs occur in the abdomen. When retroperitoneal PGLs produce symptoms of compression or invasion of adjacent organs, they could be misdiagnosed as a tumor of adjacent organs. Here, we report a rare case of ureteral obstruction caused by an extra-adrenal retroperitoneal PGL that mimicked a ureteral tumor. A 46-year-old female presented with a 2-year history of hypertension. Routine laboratory tests were unremarkable. The computed tomography (CT) scan showed a hypervascular mass located in the left mid-ureteral section. Ureteropyelography revealed left ureteral obstruction at the level of L4 to L5 vertebrae and hydroureteronephrosis. Cystoscopy confirmed left ureteral obstruction. Based on the clinical and imaging findings, a left ureteral tumor was suspected preoperatively. The tumor was completely resected, and the involved segment of the ureter was removed. No significant enlarged lymph nodes were seen in the pelvic cavity and retroperitoneum. A histopathological examination of the tumor confirmed retroperitoneal PGL partly infiltrating the ureter. The patient's blood pressure returned to the normal range postoperatively. Our case demonstrates the importance of considering extra-adrenal PGL in the differential diagnosis of retroperitoneal tumors.