Successful combined treatment for giant mesenteric desmoid tumor: case report and review of the literature

J Pediatr Surg. 2012 Jun;47(6):e25-30. doi: 10.1016/j.jpedsurg.2012.01.081.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Combined Modality Therapy
  • Diagnostic Imaging
  • Female
  • Fibromatosis, Aggressive / diagnosis
  • Fibromatosis, Aggressive / drug therapy
  • Fibromatosis, Aggressive / etiology
  • Fibromatosis, Aggressive / surgery*
  • Humans
  • Kidney Neoplasms / drug therapy
  • Kidney Neoplasms / radiotherapy
  • Kidney Neoplasms / surgery
  • Mesentery / pathology
  • Mesentery / surgery*
  • Methotrexate / administration & dosage
  • Neoplasm Recurrence, Local / drug therapy
  • Neoplasm Recurrence, Local / surgery
  • Neoplasms, Second Primary / diagnosis
  • Neoplasms, Second Primary / drug therapy
  • Neoplasms, Second Primary / etiology
  • Neoplasms, Second Primary / surgery
  • Nephrectomy
  • Peritoneal Neoplasms / diagnosis
  • Peritoneal Neoplasms / drug therapy
  • Peritoneal Neoplasms / etiology
  • Peritoneal Neoplasms / surgery*
  • Remission Induction
  • Vinblastine / administration & dosage
  • Wilms Tumor / drug therapy
  • Wilms Tumor / radiotherapy
  • Wilms Tumor / surgery

Substances

  • Vinblastine
  • Methotrexate