Journal article
Cellular Fibroma of Tendon Sheath With Novel TNC-USP6 Gene Fusion Clinically Mimicking Arthritis in a 7-Year-Old Boy
Pediatric and developmental pathology, Vol.25(2), pp.192-196
03/2022
DOI: 10.1177/10935266211043869
PMID: 34520696
Abstract
Cellular fibroma of tendon sheath (CFTS) is a rare, benign myofibroblastic neoplasm of tenosynovial soft tissues closely resembling nodular fasciitis (NF), but is histomorphologically distinct from classic fibroma of tendon sheath (FTS). We report a case of a pediatric patient with thumb swelling clinically concerning for arthritis with a biopsy demonstrating myofibroblastic proliferation with features consistent with NF/CFTS, and molecular studies confirming the presence of a USP6 gene fusion (TNC-USP6). This case highlights a unique clinical presentation of CFTS in a pediatric patient mimicking an inflammatory or reactive/non-neoplastic musculoskeletal disorder and the increasingly crucial role of molecular testing to differentiate a reactive myofibroblastic process from a neoplasm. Moreover, this report identifies TNC as a new fusion partner to USP6 fusion partner adding to our growing understanding of the USP6-rearranged family of tumors.
Keywords: TNC; USP6; bone and soft tissue pathology; cellular fibroma of tendon sheath; juvenile idiopathic arthritis; nodular fasciitis.
Details
- Title: Subtitle
- Cellular Fibroma of Tendon Sheath With Novel TNC-USP6 Gene Fusion Clinically Mimicking Arthritis in a 7-Year-Old Boy
- Creators
- Joshua M EisenbergJoseph A Buckwalter V - University of Iowa, Orthopedics and RehabilitationAnthony N Snow - University of Iowa, PathologyJonathan Davick - University of Iowa, Pathology
- Resource Type
- Journal article
- Publication Details
- Pediatric and developmental pathology, Vol.25(2), pp.192-196
- DOI
- 10.1177/10935266211043869
- PMID
- 34520696
- NLM abbreviation
- Pediatr Dev Pathol
- eISSN
- 1615-5742
- Language
- English
- Electronic publication date
- 09/14/2021
- Date published
- 03/2022
- Academic Unit
- Pathology; Orthopedics and Rehabilitation
- Record Identifier
- 9984186509902771
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