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

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.

Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

“…First, the pure mass effect of the growing tumor with compression of the surrounding structures causes symptoms such as ureteric obstruction with hydronephrosis, intestinal obstruction, and vascular or neural compression. Second, direct infiltration of the surrounding tissues can lead to ischemia, perforation, fistula formation in hollow organs, and gastrointestinal or intratumoral bleeding [3,[8][9][10][11][12].…”

“…In contrast to our case, for patients with asymptomatic, nonprogressive desmoid tumors the front-line approach is a dynamic monitoring with a type of wait-and-see policy. This strategy is applicable because of the possibility of self-limitation of the tumors, even their spontaneous regression [5,8,9].…”

“…When a symptomatic, resectable desmoid tumor is diagnosed, especially in cases with an intraabdominal location, the treatment is mainly surgical, and the purpose is complete tumor removal with negative microscopic margins [5,8,9]. Therefore, the intraoperative frozen sections are useful for evaluating the resection margins [16].…”

Familial Adenomatous Polyposis and Desmoid Tumor Treated with Multivisceral Transplantation and Kidney Autotransplantation: Case Report and Literature Review

“…DT arise from fascial or deep musculoaponeurotic structures. They can be classified as extra-abdominal, abdominal wall, and intra-abdominal types [10]. The etiology of DT is still unclear.…”

“…DT are usually presented as a painless or minimally painful mass with a history of slow growth [10]. Intra-abdominal DT are large neoplasms that remain asymptomatic until their size causes visceral compression.…”