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

An Erratum to this article was published on 11 May 2017

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.

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Additional information

Correspondence to: Alessandro Gronchi, MD

Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy, e-mail: alessandro.gronchi@istitutotumori.mi.it

Correspondence to: Carol J. Swallow, MD, PhD

Department of Surgical Oncology, Mount Sinai Hospital and Princess Margaret Cancer Center, and Department of Surgery University of Toronto, Toronto, Canada, e-mail: cswallow@mtsinai.on.ca

An erratum to this article is available at http://dx.doi.org/10.1245/s10434-017-5889-0.

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Trans-Atlantic RPS Working Group. Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol 23, 3531–3540 (2016). https://doi.org/10.1245/s10434-016-5336-7

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Keywords

  • Sarcoma
  • Synovial Sarcoma
  • Malignant Peripheral Nerve Sheath Tumor
  • Clear Cell Sarcoma
  • Netherlands Cancer Institute