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Giant retroperitoneal desmoid tumour
Neville D Perera1, V Hasanthi Vithana
1Department of Surgery, National Hospital of Sri Lanka, Colombo. nevi603@sltnet.lk
The Ceylon Medical Journal
|February 24, 2007
Summary
A rare giant retroperitoneal desmoid tumor occurred in a teenage girl. Despite aggressive treatment, the aggressive tumor invaded vital structures, leading to fatal gastrointestinal bleeding.
Area of Science:
- Oncology
- Surgical Pathology
- Pediatric Oncology
Background:
- Desmoid tumors, also known as aggressive fibromatosis, are rare soft-tissue neoplasms.
- Retroperitoneal desmoid tumors are particularly uncommon and can grow to significant sizes.
- Psoas fascia is a rare origin site for these aggressive fibromatosis cases.
Observation:
- A case report details a 16-year-old female with a massive (30 x 15 cm) retroperitoneal desmoid tumor originating from the psoas fascia.
- The tumor exhibited aggressive local invasion despite initial debulking surgery.
Findings:
- Standard treatment modalities including surgery, adjuvant radiotherapy, anti-estrogen therapy, and non-steroidal anti-inflammatory drugs were administered.
- Despite multimodal therapy, the tumor demonstrated relentless growth and invasion.
- The patient succumbed to massive gastrointestinal bleeding secondary to tumor invasion of major blood vessels and bowel three years post-diagnosis.
Implications:
- This case highlights the aggressive nature and potential lethality of large retroperitoneal desmoid tumors, even in young patients.
- It underscores the challenges in managing these rare neoplasms and the need for novel therapeutic strategies.
- Further research into the molecular mechanisms driving aggressive fibromatosis is warranted to improve patient outcomes.