A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes
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. Mag...
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| Language: | English |
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Elsevier
2025-05-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1930043325000834 |
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| author | Boon Tat Yeap, MD, MMed Ikhwan Nasir, MD, MMed Thai Hau Koo, MD Aaron Gerarde Paul, MD, MMed Mohd Hazeman Zakaria, MD, MMed |
| author_facet | Boon Tat Yeap, MD, MMed Ikhwan Nasir, MD, MMed Thai Hau Koo, MD Aaron Gerarde Paul, MD, MMed Mohd Hazeman Zakaria, MD, MMed |
| author_sort | Boon Tat Yeap, MD, MMed |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-294c8cd1f961426a92172dbd3c486570 |
| institution | DOAJ |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-294c8cd1f961426a92172dbd3c4865702025-08-20T03:06:00ZengElsevierRadiology Case Reports1930-04332025-05-012052384238710.1016/j.radcr.2025.01.083A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and OutcomesBoon Tat Yeap, MD, MMed0Ikhwan Nasir, MD, MMed1Thai Hau Koo, MD2Aaron Gerarde Paul, MD, MMed3Mohd Hazeman Zakaria, MD, MMed4Department of Anaesthesiology and Intensive Care, Hospital Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia; Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu, Sabah, MalaysiaDepartment of Anaesthesiology and Intensive Care, Hospital Universiti Malaysia Sabah, Kota Kinabalu, Sabah, MalaysiaDepartment of Internal Medicine, School of Medical Sciences, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, MalaysiaDepartment of Orthopaedics, Hospital Queen Elizabeth, Kota Kinabalu, Sabah, MalaysiaDepartment of Radiology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Selangor, Malaysia; Corresponding author.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.http://www.sciencedirect.com/science/article/pii/S1930043325000834Desmoid fibromatosisPaediatricsRadiologyOrthopedicsOncology |
| spellingShingle | Boon Tat Yeap, MD, MMed Ikhwan Nasir, MD, MMed Thai Hau Koo, MD Aaron Gerarde Paul, MD, MMed Mohd Hazeman Zakaria, MD, MMed A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes Radiology Case Reports Desmoid fibromatosis Paediatrics Radiology Orthopedics Oncology |
| title | A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes |
| title_full | A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes |
| title_fullStr | A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes |
| title_full_unstemmed | A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes |
| title_short | A Rare Case of Desmoid Fibromatosis in a Pediatric Patient: Surgical Management and Outcomes |
| title_sort | rare case of desmoid fibromatosis in a pediatric patient surgical management and outcomes |
| topic | Desmoid fibromatosis Paediatrics Radiology Orthopedics Oncology |
| url | http://www.sciencedirect.com/science/article/pii/S1930043325000834 |
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