Τίτλος:
Recurrent aggressive mesenteric desmoid tumor successfully treated with
sorafenib: A case report and literature review
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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 CINNB1, 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.
Συγγραφείς:
Mastoraki, Aikaterini
Schizas, Dimitrios
Vergadis,
Chrysovalantis
Naar, Leon
Strimpakos, Alexios
Vailas,
Michail G.
Hasemaki, Natasha
Agrogiannis, George
Liakakos,
Theodore
Arkadopoulos, Nikolaos
Περιοδικό:
World Journal of Clinical Oncology
Εκδότης:
BAISHIDENG PUBLISHING GROUP INC
Λέξεις-κλειδιά:
Desmoid tumor; Aggressive fibromatosis; Case report; Pathogenesis;
Therapeutic approach; Sorafenib treatment
DOI:
10.5306/wjco.v10.i4.183
