Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. Apr 24, 2019; 10(4): 183-191
Published online Apr 24, 2019. doi: 10.5306/wjco.v10.i4.183
Recurrent aggressive mesenteric desmoid tumor successfully treated with sorafenib: A case report and literature review
Aikaterini Mastoraki, Dimitrios Schizas, Chrysovalantis Vergadis, Leon Naar, Alexios Strimpakos, Michail G Vailas, Natasha Hasemaki, George Agrogiannis, Theodore Liakakos, Nikolaos Arkadopoulos
Aikaterini Mastoraki, Leon Naar, Alexios Strimpakos, Nikolaos Arkadopoulos, 4th Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Athens 11527, Greece
Dimitrios Schizas, Michail G Vailas, Natasha Hasemaki, Theodore Liakakos, 1st Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece
Chrysovalantis Vergadis, Department of Radiology, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece
George Agrogiannis, 1st Department of Pathology, National and Kapodistrian University of Athens, Athens 11527, Greece
Author contributions: Mastoraki A, Naar L and Arkadopoulos N designed the report; Mastoraki A, Schizas D, Hasemaki N and Vergadis C collected the patient’s clinical data; Strimpakos A, Vailas MG and Agrogiannis G provided relevant figures; Mastoraki A, Schizas D, Arkadopoulos N and Liakakos T analyzed the data and wrote the paper.
Informed consent statement: Provided; nevertheless, patient’s initials or characteristics are not exposed.
Conflict-of-interest statement: The authors declare that no conflict of interest exists.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Aikaterini Mastoraki, MD, PhD, Doctor, 4th Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, 1, Rimini Street, Chaidari, Athens 11527, Greece. dr_kamast@yahoo.gr
Telephone: +30-6932-577710 Fax: +30-210-5326412
Received: January 8, 2019
Peer-review started: January 8, 2019
First decision: January 26, 2019
Revised: February 22, 2019
Accepted: March 26, 2019
Article in press: March 27, 2019
Published online: April 24, 2019
Abstract
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.

Keywords: Desmoid tumor, Aggressive fibromatosis, Case report, Pathogenesis, Therapeutic approach, Sorafenib treatment

Core tip: Desmoid tumors (DT) are locally advanced but histologically benign monoclonal neoplasms that can occur from any musculoaponeurotic structure. DT 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 promising therapeutic option against DTs, while several pharmacological agents have been successfully used, including dacarbazine-doxorubicin, anti-estrogens, non-steroidal anti-inflammatory drugs, imatinib mesylate and sunitinib.