Desmoplastic fibroma in a child: a 9-year follow-up case report

Bibliographic Details
Title: Desmoplastic fibroma in a child: a 9-year follow-up case report
Authors: Yaokai Lu, Wei Lan, Qiangchu Wu, Yi Fu, Shengyuan Lan, Xixiong Wang, Xuwei Huang, Lu Ye
Source: BMC Musculoskeletal Disorders, Vol 25, Iss 1, Pp 1-9 (2024)
Publisher Information: BMC, 2024.
Publication Year: 2024
Collection: LCC:Diseases of the musculoskeletal system
Subject Terms: Desmoplastic fibroma, Tumor resection, Free vascularized fibular proximal epiphyseal transfer, Arthrodesis, Diseases of the musculoskeletal system, RC925-935
More Details: Abstract Background Desmoplastic fibroma is an extremely rare primary bone tumor. Its characteristic features include bone destruction accompanied by the formation of soft tissue masses. This condition predominantly affects individuals under the age of 30. Since its histology is similar to desmoid-type fibromatosis, an accurate diagnosis before operation is difficult. Desmoplastic fibroma is resistant to chemotherapy, and the efficacy of radiotherapy is uncertain. Surgical excision is preferred for treatment, but it entails high recurrence. Further, skeletal reconstruction post-surgery is challenging, especially in pediatric cases. Case presentation Nine years ago, a 14-year-old male patient presented with a 4-year history of progressive pain in his left wrist. Initially diagnosed as fibrous dysplasia by needle biopsy, the patient underwent tumor resection followed by free vascularized fibular proximal epiphyseal transfer for wrist reconstruction. However, a histological examination confirmed a diagnosis of desmoplastic fibroma. The patient achieved bone union and experienced a recurrence in the ipsilateral ulna 5 years later, accompanied by a wrist deformity. He underwent a second tumor resection and wrist arthrodesis in a single stage. The most recent annual follow-up was in September 2023; the patient had no recurrence and was satisfied with the surgery. Conclusions Desmoplastic fibroma is difficult to diagnose and treat, and reconstruction surgery after tumor resection is challenging. Close follow-up by experienced surgeons may be beneficial for prognosis.
Document Type: article
File Description: electronic resource
Language: English
ISSN: 1471-2474
Relation: https://doaj.org/toc/1471-2474
DOI: 10.1186/s12891-024-07454-6
Access URL: https://doaj.org/article/31396db355a5447b8f5482229f83c470
Accession Number: edsdoj.31396db355a5447b8f5482229f83c470
Database: Directory of Open Access Journals
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More Details
ISSN:14712474
DOI:10.1186/s12891-024-07454-6
Published in:BMC Musculoskeletal Disorders
Language:English