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[Familial abdominal fibromatosis]
S Constantinoiu1, A R Mocanu, D Pop
1Laboratorul de Anatomie Patologică, Spitalul Clinic, Griviţa.
Chirurgia (Bucharest, Romania : 1990)
|January 1, 1995
Summary
This study follows two brothers with abdominal fibromatosis, detailing their complex surgeries for large tumors. Both patients experienced good postoperative recovery, with chromosomal analysis revealing anomalies.
Area of Science:
- Oncology
- Genetics
Background:
- Abdominal fibromatosis is a rare condition characterized by benign fibrous tumors.
- Familial occurrence of fibromatosis suggests a potential genetic predisposition.
Observation:
- Two brothers presented with distinct abdominal fibromatosis manifestations.
- One brother required extensive surgery for a large retroperitoneal and intraperitoneal tumor, including jejunal resection.
- The other brother underwent surgery for a nuchal fibrosarcoma and subsequently for a gastric fibroid tumor necessitating a gastro-colectomy.
Findings:
- Both patients demonstrated good postoperative outcomes.
- Karyotype analysis revealed anomalies in the short arms of chromosomes 21 and 22, though considered normal.
Implications:
- This case series highlights the varied clinical presentations of abdominal fibromatosis within a family.
- The observed chromosomal anomalies warrant further investigation into their role in the pathogenesis of familial fibromatosis.