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The Desmoid Tumor Research Foundation

March 2025

A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes

Author Information

Boon Tat Yeap 1 2, Ikhwan Nasir 1, Thai Hau Koo 3, Aaron Gerarde Paul 4, Mohd Hazeman Zakaria 5

1Department of Anaesthesiology and Intensive Care, Hospital Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia. 2Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia. 3Department of Internal Medicine, School of Medical Sciences, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia. 4Department of Orthopaedics, Hospital Queen Elizabeth, Kota Kinabalu, Sabah, Malaysia. 5Department of Radiology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor, Malaysia.

Publication

Radiol Case Rep

Summary

Desmoid fibromatosis (DF) is a rare, locally aggressive tumor arising from the abdominal fascia or musculoaponeurosis, commonly affecting individuals between 15 and 60 years of age. We present a case of a 13-year-old boy with a 9-month history of progressive swelling in the left posterior thigh. Magnetic resonance imaging (MRI) revealed a soft tissue tumor encasing the sciatic nerve. A biopsy confirmed the diagnosis of DF, showing spindle cells arranged in fascicles. The patient underwent wide local excision of the tumor with preservation of the sciatic nerve. Postoperative recovery was smooth, and 1-year follow-up MRI showed no recurrence. Surgical excision remains the primary treatment, especially in symptomatic patients, although recurrence is common even with negative margins. This case underscores the importance of regular follow-up for DF and a multidisciplinary approach to optimize management and surveillance.

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