Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group

Abstract

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.

Guidelines for the Treatment of Recurrent Retroperitoneal Sarcoma: Are we Trying to Fit a Square Peg into a Round Hole?

“…This issue of Annals of Surgical Oncology includes an article that provides guidelines for the treatment of recurrent retroperitoneal sarcomas. 4 The current trend in clinical management is the creation of treatment guidelines for specific disease processes. The underlying hypothesis that guidelines derived from a subset of patients with the more common histologic subtypes can be generalized to all individuals within a disease group needs to be carefully scrutinized.…”

Clinicopathological characteristics and experience in the treatment of giant retroperitoneal liposarcoma: A case report and review of the literature

“…Even huge, those bulks can be removed as well by a multidisciplinary team in a specialized center, which is consistent with the consensus management of RPS in the adult. 30 It has been demonstrated 31 that they can be successfully resected obtaining negative microscopic margins with combined resection of involved organs such as kidney, ureter and large bowel. Long-term prognosis without complete resection is poor with average 5 and 10-year survival rates of 16.7% and 8.0% respectively.…”

Individualisierte multimodale Therapie retroperitonealer Weichteilsarkome

“…Retroperitoneale Sarkome stellen keine definierte Erkrankung dar, sie sind vielmehr durch ihre anatomische Lage im Retroperitoneum und der Entwicklung aus Zellen mesenchymaler Liniendifferenzierung charakterisiert. Aus den über 100 histo-und molekularpathologisch unterschiedenen Sarkomentitäten treten nur vergleichsweise wenige im Retroperitoneum auf [1,2].…”