Complete Radiological Response to Sorafenib in Aggressive Intra-Abdominal Desmoid Tumor: A Case Report

Abstract

Desmoid tumors are malignant non-inflammatory fibroblastic tumors with a tendency for local invasion and recurrence post resection. They are considered a locally aggressive proliferative disease within the family of soft-tissue sarcomas but, metastasis is uncommon. The purpose of this report is to analyze a rare clinical case of an aggressive mesenteric desmoid tumor successfully treated with sorafenib. Our case is a 32-year-old male patient with no comorbidities who presented in 2022 with right iliac fossa pain and underwent surgery for suspected appendicitis. The exploratory laparotomy revealed a voluminous central mesenteric mass measuring approximately 10 cm, in contact with the ascending colon and cecum, exhibiting bleeding upon manipulation. The appendix appeared macroscopically normal, with no evidence of inflammation or abscess formation. Multiple biopsies of the tumor were obtained for analysis. The histopathological examination concluded to a desmoid tumor, and the decision was to put the patient under Sorafenib 400 mg per day. The assessment after three months found an excellent clinical and radiological response, with a total disappearance of the symptoms. A complete radiological response was achieved after 18 months of treatment. This case study allowed us to support the efficacy of Sorafenib, as a hopeful therapeutic option in the treatment of symptomatic and locally advanced surgically unresectable desmoid tumors.