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Desmoid tumors in childhood
P Scougall1, L T Staheli, D E Chew
1Royal North Shore Hospital, St. Leonards, Sydney, Australia.
Orthopaedic Review
|July 1, 1987
Summary
Extra-abdominal desmoid tumors in children, often found in the pelvis or forearm, are typically treated with surgical excision. Most patients achieve a tumor-free status post-surgery, indicating a good prognosis for these benign growths.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Tumor Biology
Background:
- Desmoid tumors, though benign, can present challenges due to their infiltrative nature.
- Extra-abdominal desmoid tumors are rare in pediatric populations, necessitating specific clinical review.
Observation:
- This review analyzed eight pediatric cases of extra-abdominal desmoid tumors.
- Common sites included the pelvis and forearm, with a slowly enlarging mass as the primary symptom.
- Diagnosis was consistently established via open biopsy.
Findings:
- Surgical excision was the primary treatment modality for all cases.
- Six out of eight patients achieved complete tumor remission after an average follow-up of 5.8 years.
- Tumor behavior in children mirrors that observed in adults.
Implications:
- Wide local excision is the recommended treatment for extra-abdominal desmoid tumors.
- Partial excision may be considered to preserve function when vital structures are involved.
- Observation is suitable for quiescent recurrent tumors; re-excision or radiotherapy are alternatives if further intervention is needed.