Aggressive fibromatosis in giant retroperitoneal tumor

Abstract

Retroperitoneal fibromatosis is an aggressive fibromatosis of slow and progressive installation, and currently there are discrepancies related to its optimal treatment. We present the case of a female patient of 30 years old with a history of bronchial asthma, which was studied in 2011 for abdominal pain, gastric fullness, and increased abdominal perimeter. A large tumor mass was diagnosed that involved abdominal organs and large vessels. In the case of retroperitoneal fibromatosis, surgery was performed twice, without resecting the tumor. With the confirmation by biopsy of an aggressive fibromatosis, it´s proposed by Oncology Service (January 2012), the use of chemotherapy with anthracyclines in neo-adjuvant setting, obtaining an objective response at the end of six cycles, achieving the resection of 80% of the tumor. Subsequently, he remained with hormone therapy (tamoxifen-20mg/day), and at the end of 4 months, another surgical intervention was performed, obtaining a complete resection of the residual tumor disease. It is maintained to date, in follow-up consultation by Oncology, with a satisfactory evolution, asymptomatic, and without clinical or imaging evidence of fibromatosis.