Abstract

Background & ObjectivesPrimary chest wall sarcomas are rare and therapeutically challenging tumors. Herein we report the outcomes of a surgery-based multimodality therapy for these pathologies over an 11-year period. In addition, we present a case that illustrates the surgical challenges that extensive chest wall resection may pose.MethodsUsing the Society of Thoracic Surgeons general thoracic surgery database, we have prospectively collected data in our institute on all patients undergoing chest wall resection and reconstruction for primary chest wall sarcomas between June 2008–October 2019.ResultsWe performed 28 surgical procedures on 25 patients aged 5 to 91 years (median age 33). Eleven tumors were bone- and cartilage-derived and 14 tumors originated from soft tissue elements. Seven patients (7/25, 28%) received neo-adjuvant therapy and 14 patients (14/25, 56%) received adjuvant therapy. The median number of ribs that were resected was 2.5 (range 0 to 6). In 18/28 (64%) of surgeries, additional skeletal or visceral organs were removed, including: diaphragm [1], scapula [2], sternum [2], lung [2], vertebra [1], clavicle [1] and colon [1]. Chest wall reconstruction was deemed necessary in 16/28 (57%) of cases, polytetrafluoroethylene (PTFE) Gore-Tex patches was used in 13/28 (46%) of cases and biological flaps where used in 4/28 (14%) of cases. R0, R1 and R2 resection margins were achieved in 19/28 (68%), 9/28 (32%) and 0/28 (0%) of cases, respectively. The median follow up time was 33 months (range 2 to 138). During the study period, disease recurred in 8/25 (32%) of patients. Of these, 3 were re-operated on and are free of disease. At date of last follow up, 5/25 (20%) of patients have died due to their disease and in contrast, 20/25 (80%) were alive with no evidence of disease.ConclusionsSurgery-based multimodality therapy is an effective treatment approach for primary chest wall sarcomas. Resection of additional skeletal or visceral organs and reconstruction with synthetic and/or biological flaps is often required in order to obtain R0 resection margins. Ultimately, long-term survival in this clinical scenario is an achievable goal.

Highlights

  • Primary chest wall tumors comprise 1 to 2% of all thoracic neoplasms

  • Data collection Using our departmental prospectively maintained Society of Thoracic Surgeons (STS) database, we identified 25 patients that were operated by a single surgical team (UI, OW) for primary chest wall sarcomas between June 2008 to October 2019

  • Eleven tumors (44%) were bone- and cartilage-derived (6 Ewing sarcoma, 4 chondrosarcoma and 1 atypical ossifying fibromyxoid tumor) and 14 (56%) tumors originated from soft tissue elements (5 desmoid, 2 synovial sarcoma, 2 pleomorphic sarcoma, 1 leiomyosarcoma, 1 fibromixoid sarcoma, 1 angiosarcoma, 1 liposarcoma and 1 epithelioid hengioendothelioma)

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Summary

Introduction

Primary chest wall tumors comprise 1 to 2% of all thoracic neoplasms. They are classified according to their tissue of origin (bone and cartilage vs soft tissue) and according to their malignant potential (benign vs malignant). The clinical presentation of primary chest wall sarcomas is diverse. Aggressive chest wall sarcomas, often induce pain, shortness of breath, and in some cases, systemic symptoms [1, 3]. Given the rare occurrence of primary chest wall sarcomas only a limited number of publications have been published on the long-term outcomes of surgery in this clinical scenario. We present a case that illustrates the surgical challenges that extensive chest wall resection may pose

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