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WHO Classification of soft tissue and bone tumors
- Publisher
- SAGE
- Copyright
- © The Author(s) 2023
- ISSN
- 1066-8969
- eISSN
- 1940-2465
- DOI
- 10.1177/10668969221150369
- Publisher site
- See Article on Publisher Site
Abstract
Periosteal fasciitis is a subtype of nodular fasciitis originating from the periosteum. The diagnosis can be challenging and requires radiologic–pathologic correlation. Advances in molecular analysis confirmed that nodular fasciitis and its related lesions harbor a USP6 gene rearrangement with one of the several potential partners. Herein, we report a case of periosteal fasciitis with metaplastic bone formation detected incidentally during a radiologic survey for breast carcinoma. Radiologic examination revealed a 2.4 cm, heterogeneous, avidly enhancing lesion of the right femoral distal metaphysis concerning for low-grade periosteal chondrosarcoma. Histological examination of a core needle biopsy revealed a tumor composed of bland spindle cells with myofibroblastic and osteoblastic phenotypes admixed with immature bone and cartilaginous elements. Molecular analysis revealed a novel STAG1::USP6 fusion that helped arrive at the right diagnosis and further expands the molecular profile of USP6-associated neoplasms.
Journal
International Journal of Surgical Pathology – SAGE
Published: Oct 1, 2023
Keywords: periosteal fasciitis; myositis ossificans; STAG1::USP6; fusion gene; periosteal chondrosarcoma; parosteal osteosarcoma