ABSTRACT
Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. We sought to identify the role of hyperthermic intraperitoneal chemotherapy in patients with intra-abdominal desmoplastic small round cell tumor. Our review of the available literature revealed no clear survival benefit in performing hyperthermic intraperitoneal chemotherapy after cytoreductive surgery.
Subject(s)
Desmoplastic Small Round Cell Tumor , Peritoneal Neoplasms , Sarcoma , Male , Humans , Hyperthermic Intraperitoneal Chemotherapy , Desmoplastic Small Round Cell Tumor/drug therapy , Desmoplastic Small Round Cell Tumor/pathology , Peritoneal Neoplasms/drug therapy , Combined Modality TherapyABSTRACT
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.
Subject(s)
Retroperitoneal Neoplasms , Sarcoma , Humans , Quality of Life , Neoplasm Recurrence, Local/pathology , Retrospective Studies , Sarcoma/surgery , Retroperitoneal Neoplasms/surgeryABSTRACT
Retroperitoneal liposarcomas are a rare entity and are comprised mostly of the well-differentiated and dedifferentiated subtypes. Eight-year survival ranges from 30% to 80% depending on histologic subtype and grade. Surgery is the cornerstone of treatment and compartment resection is the current standard. Mesenteric liposarcomas are extremely rare and comprise more high-grade lesions, with poorer prognosis of 50% 5-year overall survival. They are managed with a similar aggressive surgical approach. This review presents the current management of retroperitoneal and mesenteric liposarcomas.
