Aggressive intra-abdominal fibromatosis in children and response to chemotherapy.
Intra-abdominal fibromatosis (IAF) is a rare benign neoplasm arising from the abdominal fibrous tissue, mostly in the mesentery. IAF is characterized by a tendency to infiltrate the surrounding vessels and vital structures and recurrence after usually incomplete surgical removal. Accordingly, IAF is associated with considerable morbidity and mortality. The authors report on a boy who presented with a large IAF at the age of 5 years. Within 6 months after initial presentation, he underwent 4 subsequent abdominal explorations for diagnosis, tumor reduction, and intestinal obstructions. IAF was confirmed by the presence of vimentin and absence of other biological cell markers. Due to accelerated tumor growth and deteriorated general condition, as a last resort, a chemotherapy trial with vincristin and methotrexate was carried out. This regimen proved to be effective in reducing the tumor burden and improving the patient's general condition. Outcome of IAF depends on early diagnosis and complete tumor resection, and, if indicated, timely employment of neo/adjuvant chemotherapy. Radiotherapy must be considered in life-threatening conditions as the last resort in a growing child [2-4].
Alebouyeh
Mardawig
M
Shaheed Beheshti University of Medical Sciences, Department of Pediatric Hematology/Oncology, Shohada Medical Center, Tajrish-Tehran, Iran. alebouyehm@yahoo.com
Moussavi
Farideh
F
Tabari
Ahmad Kahlegh-Nejad
AK
Vossough
Parvaneh
P
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002675:Child, Preschool; D018221:Fibromatosis, Abdominal / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D018222:Desmoid Tumors / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D011859:Radiography; D047368:Tumor Burden / Q000187:drug effects; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D008727:Methotrexate