Successful combined treatment for giant mesenteric desmoid tumor: case report and review of the literature.
Mesenteric aggressive fibromatosis, also known as abdominal desmoid tumor (DT), is a rare monoclonal neoplasm arising from muscoloaponeurotic structures, caused by a generalized defect in growth regulation of the connective tissue. Childhood abdominal DT is very rare (1), and the mesenteric localization is one of the rarest forms (approximately 5% of total cases). Despite its benign microscopic appearance and nonmetastasizing behavior, abdominal DT shows a high risk of recurrence (30%-80%) (2) and local aggressive growth. We report a case of a 7-year-old girl with a giant mesenteric fibromatosis, with multiple recurrence after surgical resections, successfully treated with low-dose of vinblastine (3-6 mg/m(2) per week) and methotrexate (20-30 mg/m(2) per week) for 24 months (every 7 days for 11 months and every 2 weeks for the last 13 months). After a follow-up of 47 months from the end of treatment, the child is in good health and in complete remission. Prolonged therapy with low-dose methotrexate and vinblastine appears to control abdominal DT and is associated with stable disease in patients with tumor unresponsive to surgery.
Pediatric Surgical Unit, Infermi Hospital, Rimini, Italy.
Silvana
S
Federici
Maurizio
M
Mordenti
Vincenzo
V
Domenichelli
Gabriella
G
Pelusi
Simona
S
Straziuso
Roberta
R
Pericoli
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D003952:Diagnostic Imaging; D005260:Female; D018222:Desmoid Tumors / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D008643:Mesentery / Q000473:pathology / Q000601:surgery*; D008727:Methotrexate / Q000008:administration & dosage; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy / Q000601:surgery; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000601:surgery; D009392:Nephrectomy; D010534:Peritoneal Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D012074:Remission Induction; D014747:Vinblastine / Q000008:administration & dosage; D009396:Wilms Tumor / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery
D014747:Vinblastine; D008727:Methotrexate