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Background  Chest wall sarcomas are a rare group of soft tissue malignancies with variable presentations. Here we describe the definitive management of a large, rapidly progressing chest wall sarcoma arising from the pectoralis major muscle.    Case report  An obese 42-year-old African American male with multiple medical comorbidities presented with new onset right-sided chest pain and a palpable right chest mass. Initial CT chest demonstrated a 9x9x9cm necrotic mass arising from the pectoralis major. CT-guided core biopsy was positive for high-grade spindle cell neoplasm (positive for smooth muscle actin, desmin, S100, and CD31; negative for CD34, PAX8, and beta-catenin). Staging imaging 2 months later demonstrated growth of the mass to 21.4 × 17.8 × 13.7 cm. The patient underwent neoadjuvant chemoradiation with surveillance CT imaging demonstrating a stable tumor. Then he underwent wide local excision of the mass followed by delayed local myocutaneous flap reconstruction and skin grafting. Final pathology was R0 resection, 38x20x18 cm tumor with 70% gross necrosis. Microscopic examination confirmed high-grade sarcoma with smooth muscle differentiation. Final pathologic staging was Stage III G3 pT2bNxMx.    Conclusions  This patient presented with a rare, rapidly enlarging high-grade leiomyosarcoma of the chest wall without metastases or violation of the thorax. We describe the definitive management including a multidisciplinary team to manage a complex and rapidly progressive sarcoma of the chest wall.

R0 resection and reconstruction for a large, rapidly progressive chest wall sarcoma