Recurrent aggressive mesenteric desmoid tumor successfully treated with sorafenib: A case report and literature review.

Autor:	Mastoraki A; 4 Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 11527, Greece. dr_kamast@yahoo.gr., Schizas D; 1 Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece., Vergadis C; Department of Radiology, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece., Naar L; 4 Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 11527, Greece., Strimpakos A; 4 Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 11527, Greece., Vailas MG; 1 Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece., Hasemaki N; 1 Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece., Agrogiannis G; 1 Department of Pathology, National and Kapodistrian University of Athens, Athens 11527, Greece., Liakakos T; 1 Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece., Arkadopoulos N; 4 Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 11527, Greece.
Jazyk:	angličtina
Zdroj:	World journal of clinical oncology [World J Clin Oncol] 2019 Apr 24; Vol. 10 (4), pp. 183-191.
DOI:	10.5306/wjco.v10.i4.183
Abstrakt:	Background: Desmoid tumors (DT) are locally advanced but histologically benign monoclonal neoplasms that can occur from any musculoaponeurotic structure. The aim of this report is to analyze a rare clinical case of an aggressive intra-abdominal DT successfully treated with sorafenib. Case Summary: A 36-year-old man presented with increasing colicky abdominal pain and a self-palpable mass in his left abdomen. Fourteen years earlier he was diagnosed with a large intra-abdominal tumor, which adhered to the left colonic flexure, part of the major gastric curvature and the spleen. Subsequent exploratory laparotomy revealed a voluminous mass in the epigastrium, arising from the posterior surface of the stomach and invading the superior mesenteric vessels, transverse mesocolon and the small bowel mesentery. As the tumor was unresectable, a jejunojejunal bypass was performed. Traditional therapeutic interventions proved insufficient, and the patient was started on sorafenib with a subsequent full-disease response. Conclusion: DT's pathogenesis has been associated with mutations in the adenomatous polyposis coli ( APC ) gene or beta-catenin gene CTNNB1 , sex steroids or previous surgical trauma. Local treatment modalities, such as surgery or radiotherapy, are implemented in aggressively progressing or symptomatic patients. Sorafenib is a hopeful therapeutic option against DTs, while several pharmacological agents have been successfully used. Competing Interests: Conflict-of-interest statement: The authors declare that no conflict of interest exists.
Databáze:	MEDLINE
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