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Annals of Surgery Jul 2023The aim of the present study was to compare the effect of radiotherapy (RT) on abdominal recurrence-free survival (ARFS) in patients with primary retroperitoneal sarcoma...
OBJECTIVE
The aim of the present study was to compare the effect of radiotherapy (RT) on abdominal recurrence-free survival (ARFS) in patients with primary retroperitoneal sarcoma treated in the EORTC-STBSG-62092 (STRASS) phase 3 randomized controlled trial (STRASS cohort) and off-trial (STREXIT cohort) and to pool STRASS and STREXIT data to test the hypothesis that RT improves ARFS in patients with liposarcoma.
BACKGROUND
The STRASS trial did not show any difference in ARFS between patients treated with preoperative radiotherapy+surgery (RT+S) versus surgery alone (S).
METHODS
All consecutive adult patients not enrolled in STRASS and underwent curative-intent surgery for a primary retroperitoneal sarcoma with or without preoperative RT between 2012 and 2017 (STRASS recruiting period) among ten STRASS-recruiting centres formed the STREXIT cohort. The effect of RT in STREXIT was explored with a propensity score (PS)-matching analysis. Primary endpoint was ARFS defined as macroscopically incomplete resection or abdominal recurrence or death of any cause, whichever occurred first.
RESULTS
STRASS included 266 patients, STREXIT included 831 patients (727 after excluding patients who received preoperative chemotherapy, 202 after 1:1 PS-matching). The effect of RT on ARFS in STRASS and 1:1 PS-matched STREXIT cohorts, overall and in patients with liposarcoma, was similar. In the pooled cohort analysis, RT administration was associated with better ARFS in patients with liposarcoma [N=321, hazard ratio (HR), 0.61; 95% confidence interval (CI), 0.42-0.89]. In particular, patients with well-differentiated liposarcoma and G1-2 dedifferentiated liposarcoma (G1-2 DDLPS, n=266) treated with RT+S had better ARFS (HR, 0.63; 95% CI, 0.40-0.97) while patients with G3 DDLPS and leiomyosarcoma had not. At the current follow-up, there was no association between RT and overall survival or distant metastases-free survival.
CONCLUSIONS
In this study, preoperative RT was associated with better ARFS in patients with primary well-differentiated liposarcoma and G1-2 DDLPS.
Topics: Adult; Humans; Sarcoma; Liposarcoma; Retroperitoneal Neoplasms; Retroperitoneal Space; Proportional Hazards Models; Neoplasm Recurrence, Local
PubMed: 35833413
DOI: 10.1097/SLA.0000000000005492 -
Current Oncology (Toronto, Ont.) Apr 2023Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease... (Review)
Review
Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to achieve the complete en bloc resection of the tumor along with involved organs and structures to maximize the clearance of the disease. The extent of resection also needs to consider the risk of complications. Unfortunately, the overarching challenge in primary RPS treatment is that even with optimal surgery, tumor recurrence occurs frequently. The pattern of recurrence after surgery (e.g., local versus distant) is strongly associated with the specific histologic type of RPS. Radiation and systemic therapy may improve outcomes in RPS and there is emerging data studying the benefit of non-surgical treatments in primary disease. Topics in need of further investigation include criteria for unresectability and management of locally recurrent disease. Moving forward, global collaboration among RPS specialists will be key for continuing to advance our understanding of this disease and find more effective treatments.
Topics: Humans; Neoplasm Recurrence, Local; Sarcoma; Treatment Outcome; Retroperitoneal Neoplasms; Soft Tissue Neoplasms
PubMed: 37232807
DOI: 10.3390/curroncol30050349 -
European Journal of Surgical Oncology :... Jun 2023Recurrent retroperitoneal sarcomas are rare, with patterns of recurrence determined by the histologic subtype. A range of patient characteristics and treatment profiles... (Review)
Review
Recurrent retroperitoneal sarcomas are rare, with patterns of recurrence determined by the histologic subtype. A range of patient characteristics and treatment profiles combined with a myriad of presentations and clinical courses of recurrences make this diverse entity challenging to manage. Although surgical resection improves survival in select patients, the oncological outcomes are inferior to that of primary retroperitoneal sarcomas. Management options for unresectable disease include local ablative therapy, radiation and systemic therapy, with palliative surgery indicated occasionally. Attempts at disease control must be balanced with potential morbidity and impact on the patient's quality of life. This review aims to offer insights into the current understanding of recurrent retroperitoneal sarcomas and provide some guidance on management.
Topics: Humans; Quality of Life; Neoplasm Recurrence, Local; Retrospective Studies; Sarcoma; Retroperitoneal Neoplasms
PubMed: 35810040
DOI: 10.1016/j.ejso.2022.06.008 -
BMJ Case Reports May 2024Abnormal cystic lymphangioma is a rare, benign tumour of lymphatic origin that often presents with subtle clinical symptoms. A man in his 20s sought medical attention...
Abnormal cystic lymphangioma is a rare, benign tumour of lymphatic origin that often presents with subtle clinical symptoms. A man in his 20s sought medical attention for a progressively tender mass located in the right lower quadrant. Following ultrasound and CT imaging, surgical pathology confirmed the presence of abnormal cystic lymphangioma. This case is noteworthy because of the patient's young age and the retroperitoneal location. An exploratory laparotomy was performed, mitigating the risk of recurrence. In the future, if imaging identifies a cystic mass, consideration of abnormal cystic lymphangioma within the differential diagnosis is imperative. Despite its predominantly subtle symptoms and non-malignant nature, the mass effect by the abnormal cystic lymphangioma can compromise surrounding structures. Overall, clinicians confronted with a cystic mass should adopt a comprehensive diagnostic approach, encompassing abnormal cystic lymphangioma in the differential diagnosis due to its unknown and complex nature.
Topics: Humans; Male; Retroperitoneal Neoplasms; Lymphangioma, Cystic; Diagnosis, Differential; Tomography, X-Ray Computed; Adult; Ultrasonography; Laparotomy
PubMed: 38719266
DOI: 10.1136/bcr-2023-258099 -
Der Chirurg; Zeitschrift Fur Alle... Jan 2022Retroperitoneal soft tissue sarcomas are rare and heterogeneous tumors with high recurrence rates that require a multimodal treatment approach and a surgical resection... (Review)
Review
BACKGROUND
Retroperitoneal soft tissue sarcomas are rare and heterogeneous tumors with high recurrence rates that require a multimodal treatment approach and a surgical resection strategy adapted to tumor localization and histological subtype.
OBJECTIVE
Based on current scientific data this article intends to provide an overview on subtype-specific features, prognostic factors and operative techniques in the surgical management of retroperitoneal soft tissue sarcomas.
MATERIAL AND METHODS
A review of the literature addressing surgical management of retroperitoneal soft tissue sarcomas was performed. Current evidence and recommendations were summarized.
RESULTS AND CONCLUSION
Macroscopically complete tumor resection represents the sole curative treatment option for both primary and recurrent retroperitoneal soft tissue sarcomas. To minimize the probability of tumor-infiltrated resection margins, compartmental resection has become a standard treatment for retroperitoneal soft tissue sarcomas. This approach includes resection of all organs and structures adjacent to the tumor. Multivisceral resection is often associated with this approach and it is acceptable in terms of morbidity and mortality if performed at a center with experience in retroperitoneal sarcoma surgery. Histologic subtype, tumor grading, and quality of initial surgical treatment are major prognostic factors for oncologic overall survival.
Topics: Humans; Margins of Excision; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms
PubMed: 34596706
DOI: 10.1007/s00104-021-01506-6 -
Current Oncology (Toronto, Ont.) Feb 2023Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g.,... (Review)
Review
Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g., well-differentiated liposarcoma is more likely to recur locoregionally, whereas leiomyosarcoma is more likely to develop distant metastases). Radiotherapy may provide effective locoregional control in limited circumstances and the data on the impact of chemotherapy are scant. Surgery for locally recurrent disease is associated with the greatest survival benefit; however, data are retrospective and from a highly selected subgroup of patients. Limited retrospective data have also suggested a survival association with the resection of limited distant metastases. Given the complexity of these patients, multidisciplinary evaluation at a high-volume sarcoma center is critical.
Topics: Humans; Retrospective Studies; Neoplasm Recurrence, Local; Sarcoma; Liposarcoma; Leiomyosarcoma; Retroperitoneal Neoplasms; Soft Tissue Neoplasms
PubMed: 36975422
DOI: 10.3390/curroncol30030209 -
International Journal of Urology :... Dec 2020Retroperitoneal tumors are extremely rare tumors occurring in the retroperitoneum. Retroperitoneal tumors are divided into benign tumors and malignant tumors, including... (Review)
Review
Retroperitoneal tumors are extremely rare tumors occurring in the retroperitoneum. Retroperitoneal tumors are divided into benign tumors and malignant tumors, including retroperitoneal sarcoma. Approximately 70-80% of primary retroperitoneal soft-tissue tumors are malignant; however, these only account for 0.1-0.2% of all malignancies. Retroperitoneal sarcoma is an orphan malignant disease with a low incidence. The information on benign retroperitoneal tumors is limited. The American Joint Committee on Cancer/TNM classification updated to the 8th edition in 2017. In 2010, three new drugs for soft tissue sarcoma were approved based on the results of phase III trials, but the histological subtypes of the patients enrolled in the trials of each drug differed. Recently, in addition to surgery for retroperitoneal sarcoma, the effectiveness of perioperative radiation therapy has become interesting. For malignant retroperitoneal tumors and retroperitoneal sarcoma, survival improvement and locoregional recurrence prevention can be undertaken by carrying out surgery to secure negative margins with wide and combined resection of some adjacent organs, and cooperation with a trained medical team comprising of radiologists, pathologists and medical oncologists in centralized hospitals. Some clinical trials aimed at further improving treatment results by adding preoperative chemotherapy and radiation therapy based on histological confirmation using a correct needle biopsy are in progress. In recent years, molecular profiling has been used to select eligible patients for chemotherapy. In the future, precision medicine with next-generation sequencing technology will be expected among the diverse and potential future treatments for retroperitoneal sarcoma. In this review, we summarized the current state of retroperitoneal tumors and retroperitoneal sarcoma.
Topics: Humans; Margins of Excision; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Treatment Outcome
PubMed: 32914475
DOI: 10.1111/iju.14361 -
Journal of Surgical Oncology Jan 2018Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all retroperitoneal sarcomas. Surgical resection remains the standard of care.... (Review)
Review
Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all retroperitoneal sarcomas. Surgical resection remains the standard of care. Unfortunately, many RPLPS patients will develop a local recurrence and subsequently die in the absence of distant metastasis. This review outlines the factors that predict local recurrence and influence the management of first and subsequent multiply recurrent RPLPS.
Topics: Disease Management; Humans; Liposarcoma; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms
PubMed: 29266232
DOI: 10.1002/jso.24929 -
Journal of the National Comprehensive... Jul 2022Retroperitoneal sarcoma comprises a small subset of all soft tissue sarcoma and includes various histopathologic subtypes, each with unique patterns of behavior and... (Review)
Review
Retroperitoneal sarcoma comprises a small subset of all soft tissue sarcoma and includes various histopathologic subtypes, each with unique patterns of behavior and differential risks for local recurrence and hematogenous metastatic spread. The primary treatment modality is surgery, although even with complete macroscopic resection, recurrence is common. The rationale for the addition of radiotherapy to resection is to improve local control; however, the use of radiation therapy for retroperitoneal sarcoma is controversial, and existing data are suboptimal to guide management. Treatment decisions should be determined with multidisciplinary input and shared decision-making. When used in selected patients, radiation therapy should be delivered preoperatively; postoperative treatment is not recommended.
Topics: Combined Modality Therapy; Humans; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 35830885
DOI: 10.6004/jnccn.2022.7035 -
Journal of Surgical Oncology Jan 2018The focus of this review is on the management of the less common sarcomas occurring in the retroperitoneal space, including solitary fibrous tumor (SFT), malignant... (Review)
Review
The focus of this review is on the management of the less common sarcomas occurring in the retroperitoneal space, including solitary fibrous tumor (SFT), malignant peripheral nerve sheath tumor (MPNST), perivascular epithelioid cell tumor (PEComa), and undifferentiated pleomorphic sarcoma (UPS) of the psoas muscle. As for other retroperitoneal sarcomas, surgical resection is the mainstay of curative therapy, and multidisciplinary preoperative assessment, including percutaneous needle biopsy for histologic confirmation, is the basis for personalized management, as the surgical management, and the integration of systemic therapy and radiation therapy is unique to each histologic subtype.
Topics: Disease Management; Humans; Retroperitoneal Neoplasms; Sarcoma
PubMed: 29127695
DOI: 10.1002/jso.24893