Pavan Sugoor(1), Amit Gupta(1), Shraddha Patkar(1), Bharat Rekhi(2), Mahesh Goel(1)(3)
1.Department of Surgical Oncology, Tata Memorial Center, Mumbai, India2.Department of Pathology, Tata Memorial Center, Mumbai, India3.Gastrointestinal and HPB Surgery, Department of Surgical Oncology, Tata Memorial Center, Mumbai, India
Publication
Ind J Surg Onc
Summary
We report on a case of multicentric fibromatosis originating from the retroperitoneal space and submandibular triangle, in an 18-year-old lady. Computed tomography revealed a retroperitoneal abdominopelvic tumor extending from the left sub-diaphragmatic space to the pelvic inlet which had enveloped the solid viscera in the left upper quadrant with a displaced celiac axis. She also had a recurrent resectable fibromatosis in left submandibular gland. Histopathological evaluation revealed fibromatosis. Preoperatively, Vinblastin-, Methotrexate-, Tamoxifen-based systemic chemotherapy was offered for 12 weeks in an attempt to downsize the mass. An en bloc complete resection of tumor with multi-visceral resection was performed to achieve negative margins and since then, the patient has remained asymptomatic without any signs of tumor recurrence during the 12th month follow-up visit. Complete resection with negative margins is the treatment of choice and majority of the lesions are amenable for surgical resections. Adjuvant therapy using non-steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for un-resectable or recurrent cases.
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