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Unlike for extremity sarcomas, the efficacy of radiotherapy for retroperitoneal sarcoma is not established. The aim of this study was to evaluate the impact of preoperative radiotherapy plus surgery versus surgery alone on abdominal recurrence-free survival. EORTC-62092 is an open-label, randomised, phase 3 study done in 31 research institutions, hospitals, and cancer centres in 13 countries in Europe and North America. Adults (aged ≥18 years) with histologically documented, localised, primary retroperitoneal sarcoma that was operable and suitable for radiotherapy, who had not been previously treated and had a WHO performance status and American Society of Anesthesiologists score of 2 or lower, were centrally randomly assigned (1:1), using an interactive web response system and a minimisation algorithm, to receive either surgery alone or preoperative radiotherapy followed by surgery. Randomisation was stratified by hospital and performance status. Radiotherapy was delivered as 50·4 Gy (in 28 daily fractions of 1·8 Gy) in either 3D conformal radiotherapy or intensity modulated radiotherapy, and the objective of surgery was a macroscopically complete resection of the tumour mass with en-bloc organ resection as necessary. The primary endpoint was abdominal recurrence-free survival, as assessed by the investigator, and was analysed in the intention-to-treat population. Safety was analysed in all patients who started their allocated treatment. This trial is registered with ClinicalTrials.gov, NCT01344018. Between Jan 18, 2012 and April 10, 2017, 266 patients were enrolled, of whom 133 were randomly assigned to each group. The median follow-up was 43·1 months (IQR 28·8–59·2). 128 (96%) patients from the surgery alone group had surgery, and 119 (89%) patients in the radiotherapy and surgery group had both radiotherapy and surgery. Median abdominal recurrence-free survival was 4·5 years (95% CI 3·9 to not estimable) in the radiotherapy plus surgery group and 5·0 years (3·4 to not estimable) in the surgery only group (hazard ratio 1·01, 95% CI 0·71–1·44; log rank p=0·95). The most common grade 3–4 adverse events were lymphopenia (98 [77%] of 127 patients in the radiotherapy plus surgery group vs one [1%] of 128 patients in the surgery alone group), anaemia (15 [12%] vs ten [8%]), and hypoalbuminaemia (15 [12%] vs five [4%]). Serious adverse events were reported in 30 (24%) of 127 patients in the radiotherapy plus surgery group, and in 13 (10%) of 128 patients in the surgery alone group. One (1%) of 127 patients in the radiotherapy plus surgery group died due to treatment-related serious adverse events (gastropleural fistula), and no patients in the surgery alone group died due to treatment-related serious adverse events. Preoperative radiotherapy should not be considered as standard of care treatment for retroperitoneal sarcoma. European Organisation for Research and Treatment of Cancer, and European Clinical Trials in Rare Sarcomas.

Introduction Extrarenal Wilms tumor is an extremely rare condition, typically documented only in isolated case reports. Unlike classical intrarenal Wilms tumors, extrarenal Wilms tumors arise outside the kidney, often presenting a diagnostic challenge owing to its unusual location and overlapping features with other retroperitoneal tumors. Its rarity necessitates further documentation to improve recognition and management. This report presents a case of extrarenal Wilms tumor located in the retroperitoneal space of a 6-year-old girl. Case presentation A 6-year-old white Iranian girl presented with abdominal pain and swelling in the upper left abdomen. Physical examination revealed a firm, nontender, immobile mass. Ultrasound imaging identified a well-defined mass with significant necrotic and cystic areas. Abdominopelvic computed tomography scan showed a large mass on the left side of the abdomen, exerting pressure on the adjacent pancreas, spleen, and left kidney. The patient underwent laparotomy and received 19 weeks of chemotherapy, including actinomycin-D and vincristine. Post-treatment, she fully recovered and underwent monthly sonography follow-ups for 6 months after completing chemotherapy and has shown no signs of recurrence to date. Conclusions Extrarenal Wilms tumor should be considered in the differential diagnosis of abdominal pain, especially in young children, owing to its rarity and the potential for misdiagnosis as other retroperitoneal tumors. A definitive diagnosis is made through surgical intervention followed by histopathological examination.

Background Neuroblastoma is a rare, aggressive childhood malignancy originating from neural crest progenitor cells of the sympathetic nervous system. While primarily a pediatric tumor, adult-onset neuroblastoma poses significant diagnostic and therapeutic challenges owing to its rarity, absence of standardized treatment protocols, and poor response to therapy. This case highlights a unique presentation of refractory retroperitoneal neuroblastoma in an adult female patient, managed with limited resources in a resource-constrained setting. Case presentation A 41-year-old female from a Dalit community in eastern Nepal presented with a 2-month history of abdominal pain, early satiety, and bloating. Imaging revealed a large retroperitoneal mass encasing critical vascular structures. Histopathology and biopsy confirmed neuroblastoma, but cytogenetic analysis could not be performed owing to resource limitations. The tumor was classified as stage L2 per the International Neuroblastoma Risk Group Staging System. Surgical resection was deemed infeasible owing to tumor encasement of major vessels. The patient was treated with eight cycles of alternating vincristine, cisplatin, etoposide, and cyclophosphamide; and vincristine, carboplatin, etoposide, and cyclophosphamide chemotherapy regimens, resulting in minimal tumor shrinkage. Subsequent palliative chemotherapy with temozolomide and irinotecan also failed to induce significant tumor regression. Advanced therapeutic options such as chimeric antigen receptor T cell therapy were unavailable owing to resource constraints, leaving the patient with limited treatment options. Conclusion This case underscores the challenges in diagnosing and managing adult-onset neuroblastoma, particularly in resource-limited settings where advanced diagnostic and therapeutic options are unavailable. The absence of standardized treatment protocols for adult neuroblastoma highlights the need for focused research on context-adapted treatment algorithms, affordable molecular diagnostics, and cost-effective therapeutic approaches, particularly in resource-limited settings. This report emphasizes the importance of collaborative efforts to develop globally accessible treatment strategies for rare and aggressive malignancies such as refractory neuroblastoma, particularly in underserved regions.

Based on the demonstrated clinical activity of immune-checkpoint blockade (ICB) in advanced dedifferentiated liposarcoma (DDLPS) and undifferentiated pleomorphic sarcoma (UPS), we conducted a randomized, non-comparative phase 2 trial ( NCT03307616 ) of neoadjuvant nivolumab or nivolumab/ipilimumab in patients with resectable retroperitoneal DDLPS (n = 17) and extremity/truncal UPS (+ concurrent nivolumab/radiation therapy; n = 10). The primary end point of pathologic response (percent hyalinization) was a median of 8.8% in DDLPS and 89% in UPS. Secondary end points were the changes in immune infiltrate, radiographic response, 12- and 24-month relapse-free survival and overall survival. Lower densities of regulatory T cells before treatment were associated with a major pathologic response (hyalinization > 30%). Tumor infiltration by B cells was increased following neoadjuvant treatment and was associated with overall survival in DDLPS. B cell infiltration was associated with higher densities of regulatory T cells before treatment, which was lost upon ICB treatment. Our data demonstrate that neoadjuvant ICB is associated with complex immune changes within the tumor microenvironment in DDLPS and UPS and that neoadjuvant ICB with concurrent radiotherapy has significant efficacy in UPS.

Introduction EORTC-62092 (STRASS) was a phase 3, randomized study that compared surgery alone versus surgery plus neoadjuvant radiotherapy (RT) for retroperitoneal sarcomas. RT was not associated with improved abdominal recurrence-free survival, the primary outcome measure, although on subanalysis, there may have been benefit for well-differentiated (WD) liposarcoma. This study investigated the real-world use and outcomes of RT (neoadjuvant and adjuvant) for the management of retroperitoneal liposarcoma. Methods We queried the National Cancer Database (NCDB) (2004–2017) for patients with nonmetastatic, primary retroperitoneal liposarcoma treated with resection with or without RT ( n = 3911). Patients were stratified by treatment type and histology [WD ( n = 2252), dedifferentiated (DD) ( n = 1659)]. Propensity score (PS) matching was used before comparison of treatment groups. Overall survival (OS) was the primary outcome measure. Results Median follow-up time was 4.1 years, and median OS was 10.7 years. There was no association between RT and OS for either WDLPS or DDLPS cohorts. We performed a subgroup analysis of neoadjuvant RT only, similar to STRASS. For WDLPS after PS matching ( n = 208), neoadjuvant RT was not associated with OS (hazard ratio [HR] 1.01, p = 0.0523) but was associated with longer postoperative hospital stay ( p = 0.012). For DDLPS after PS matching ( n = 290), neoadjuvant RT was not associated with OS (HR 1.02, p = 0.889). For both WD-LPS and DD-LPS, utilization of neoadjuvant RT was associated with treatment at high-volume (≥ 10 cases/year) and academic/network facilities. Conclusions For primary retroperitoneal liposarcoma treated with surgical resection, radiotherapy was not associated with an overall survival benefit in this propensity-matched, adjusted analysis of the NCDB.

Background Primary retroperitoneal serous adenocarcinoma (PRSA) is an extremely uncommon malignancy exclusively reported in females. Due to the rarity of the disease, it is difficult to establish a standardized treatment. Case presentation We describe a unique case of PRSA in a 71-year-old male who presented with right-sided lower back pain and numbness. Magnetic resonance imaging identified a mass invading the adjacent psoas muscle and twelfth rib. Tissue biopsy confirmed poorly differentiated PRSA. Patient was initially treated with neoadjuvant carboplatin and paclitaxel chemotherapy regimen. This resulted in complete radiological resolution of the tumor. However, 12 weeks later, rapid recurrence was noted on follow-up CT scan. The patient was then treated with external radiotherapy with concurrent nivolumab, an anti-PD-1 antibody. The patient displayed a positive response to treatment with reduction in primary tumor and metastases and had a sustained disease control. Conclusion Treatment with radiotherapy in combination with anti-PD-1 antibody could be an effective modality of management for PRSA.

The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumours and malignant neoplasms that can be either primary or metastatic lesions. Retroperitoneal tumours can cause a diagnostic dilemma and present several therapeutic challenges because of their rarity, relative late presentation and anatomical location, often in close relationship with several vital structures in the retroperitoneal space. A comprehensive literature search was conducted using PubMed. Relevant international articles published in the last ten years were assessed. The keywords for search purposes included: retroperitoneum, benign, sarcoma, neoplasm, diagnosis and surgery, radiotherapy, chemotherapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article. Tumours usually present late and cause symptoms or become palpable once they have reached a significant size. Retroperitoneal tumours are best evaluated with good quality cross-sectional imaging and preoperative histology by core needle biopsy is required when imaging is non-diagnostic. Sarcomas comprise a third of retroperitoneal tumours. Other retroperitoneal neoplasms include lymphomas and epithelial tumours or might represent metastatic disease from known or unknown primary sites. The most common benign pathologies encountered in the retroperitoneum include benign neurogenic tumours, paragangliomas, fibromatosis, renal angiomyolipomas and benign retroperitoneal lipomas. Complete surgical resection is the only potential curative treatment modality for retroperitoneal sarcomas and is best performed in high-volume centres by a multidisciplinary sarcoma team. The ability completely to resect a retroperitoneal sarcoma and tumour grade remain the most important predictors of local recurrence and disease-specific survival.

Introduction Retroperitoneal soft tissue sarcomas (RPS) are rare tumors. Surgery is the mainstay of curative therapy, but local recurrence is common. No recommendations concerning the best management of recurring disease have been developed so far. Although every effort should be made to optimize the initial approach, recommendations to treat recurring RPS will be helpful to maximize disease control at recurrence. Methods An RPS transatlantic working group was established in 2013. The goals of the group were to share institutional experiences, build large multi-institutional case series, and develop consensus documents on the approach to this difficult disease. The outcome of this document applies to recurrent RPS that is nonvisceral in origin. Included are sarcomas of major veins, undifferentiated pleomorphic sarcoma of psoas, ureteric leiomyosarcoma (LMS). Excluded are desmoids-type fibromatosis, angiomyolipoma, gastrointestinal stromal tumors, sarcomas arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing sarcoma, alveolar/embryonal rhabdomyosarcoma, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma. Results Recurrent RPS management was evaluated from diagnosis to follow-up. It is a rare and complex malignancy that is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, but some patients may experience prolonged disease control also at recurrence, when the approach is optimized and follows the recommendations contained herein. Conclusions International collaboration is critical for adding to the present knowledge. A transatlantic prospective registry has been established.