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- Title
BENEFITS OF METHOTREXATE/VINORELBINE CHEMOTHERAPY FOR DESMOID FIBROMATOSIS: A CASE REPORT.
- Authors
Vizitiu, Alin-Ştefan; Olteanu, Diana
- Abstract
Introduction: Mesenteric Fibromatosis (MF) is a rare proliferative fibroblastic lesion of the small intestinal mesentery, characterized by difficulties in diagnostic and therapeutic approaches. It constitutes 8% of all desmoid tumors and represents 0.03% of all neoplasms. It has a genetic component, being part of Gardner's syndrome. However, it is histologically benign but it may invade locally and recur after excision. The presenting features of MF are asymptomatic abdominal mass, abdominal discomfort, bowel or ureteral obstruction, intestinal perforation, fistulas. Case Report: In June 2021, an 18-year-old woman, with a family history of Familial Adenomatous Polyposis (FAP), presents with an intestinal obstruction caused by a proliferative mass located on the mesentery, for which terminal ileum, cecum, and ascendant colon are resected. Histopathological examinations confirm the diagnosis of MF. A postoperative MRI reveals remaining tumors in the area of the spleen and the left psoas major. Consequently, a colonoscopy is undertaken, displaying a total stenosis of the transverse colon, resulting in the impossibility of surgically restoring the digestive continuity. As a first-line treatment, chemotherapy with VAC (vincristine, actinomycin-D, and cyclophosphamide) is initiated. After 6 cycles, an MRI outlines one more time the impossibility of operating the tumors. Therefore, the second-line treatment, with Methotrexate (MTX) and Vinblastine is chosen. After 2 cycles, the treatment is switched to MTX/Vinorelbine(VNL) per os, considered to have a better efficacy. In July 2021, another MRI showcases a new mesentery tumoral mass (level of L1-L2 vertebrae) undistinguished from the proper small intestine, while the previous one diminished in size. The decision is taken to remove both masses, but tumoral formations persist on the left abdominal wall. These are interpreted as stationary evolution of the disease and the treatment with MTX/VNL is preserved. Discussions : For young patients with unresectable tumors, radical surgery and radiotherapy are inapplicable, due to long-term complications. Hence, chemotherapy with vinorelbine combined with low-dose methotrexate was an optional choice for local control. Conclusions: The tendency for recurrence makes the treatment of these rare fibrous tumors challenging, multidisciplinary approach being needed with long-term follow-up. Furthermore, VNL was shown to be better tolerated than vinblastine, with lower toxicity rates, especially neurotoxicity.
- Subjects
ROMANIA; VINORELBINE; METHOTREXATE; CONFERENCES &; conventions; CONNECTIVE tissue tumors; DRUG efficacy
- Publication
Acta Marisiensis. Seria Medica, 2024, Vol 70, p336
- ISSN
2668-7755
- Publication type
Article