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[Juvenile fibromatosis with isolated intra-abdominal form. Apropos of 2 cases]
O Bosc1, R B Galifer, G Barneon
1Service de Chirurgie Infantile, CHU St-Charles, Montpellier.
Insights
Infantile fibromatosis in the abdomen is rare. Two cases highlight diagnostic challenges and the tendency for these benign fibroblastic tumors to infiltrate and recur after surgery.
Area of Science:
- Pediatric Surgery
- Surgical Pathology
- Oncology
Background:
- Infantile fibromatosis encompasses a spectrum of fibroblastic tumors.
- Intra-abdominal fibromatosis is exceptionally rare in infants and children.
- Early diagnosis and management are crucial for optimal outcomes.
Observation:
- Two unusual cases of intra-abdominal fibromatosis are presented.
- Case 1: Hepatic fibromatosis in an 11-year-old girl.
- Case 2: Desmoid tumor of the jejunal mesentery in a 10-month-old infant.
Findings:
- Pre-operative diagnosis of intra-abdominal fibromatosis is challenging.
- Histologic examination reveals benign fibroblastic proliferation.
- These tumors exhibit infiltrative growth patterns.
Implications:
- Surgical excision is the primary treatment modality.
- High recurrence rates necessitate vigilant post-operative monitoring.
- Further research into the pathogenesis and treatment of infantile fibromatosis is warranted.
Abstract:
Among infantile fibromatosis, isolated intra-abdominal forms are the most unusual. The authors report two cases, a hepatic fibromatosis in an eleven year old girl, and a desmoïd tumor located in the jejunal mesentery in a ten month old infant. Pre-operative diagnosis is very difficult and histologic findings show a benign fibroblastic proliferation that tends to infiltrate surrounding tissues and often recur after surgical excision.