Intra-abdominal desmoid tumors: a case report
Annali Italiani Di Chirurgia
|October 23, 2014
Summary
Desmoid tumors, rare fibroblastic growths, can be associated with familial adenomatous polyposis (FAP). This case highlights the challenges in managing intra-abdominal desmoid tumors, even with treatment, emphasizing the need for expert multidisciplinary care.
Area of Science:
- Oncology
- Gastroenterology
- Surgical Pathology
Background:
- Desmoid tumors are rare fibroblastic neoplasms affecting musculoaponeurotic tissues.
- Approximately 5% of desmoid tumors are associated with familial adenomatous polyposis (FAP).
- Intra-abdominal desmoid tumors often present late due to asymptomatic growth until visceral compression occurs.
Observation:
- A 54-year-old male with a history of FAP and prophylactic colectomy presented with fever and abdominal pain.
- An intra-abdominal mass with desmoid characteristics was detected and grew despite tamoxifen therapy.
- Initial medical management failed to alleviate symptoms, necessitating surgical intervention.
Findings:
- Desmoid tumors, while histologically benign, can exhibit aggressive local behavior and cause significant complications.
- Treatment of intra-abdominal desmoid tumors is challenging, with surgery reserved for specific indications like obstruction or perforation.
- Effective management requires a multidisciplinary team experienced in sarcoma and fibromatosis.
Implications:
- Early detection and multidisciplinary management are crucial for improving outcomes in patients with desmoid tumors, particularly those with FAP.
- Further research into optimal therapeutic strategies for intra-abdominal desmoid tumors is warranted.
- Understanding the behavior of desmoid tumors in the context of FAP is essential for patient care and risk stratification.
