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- Title
Superficial CD34‐positive fibroblastic tumor and PRDM10‐rearranged soft tissue tumor are overlapping entities: a comprehensive study of 20 cases.
- Authors
Perret, Raul; Michal, Michael; Carr, Richard A; Velasco, Valérie; Švajdler, Marian; Karanian, Marie; Meurgey, Alexandra; Paindavoine, Sandrine; Soubeyran, Isabelle; Coindre, Jean‐Michel; Boidot, Romain; Charon‐Barra, Céline; Geneste, Damien; Weingertner, Noelle; Pissaloux, Daniel; Tirode, Franck; Baud, Jessica; Le Loarer, François
- Abstract
Aims: Superficial CD34‐positive fibroblastic tumor (SCD34FT) and PRDM10‐rearranged soft tissue tumor (PRDM10‐STT) are rare mesenchymal tumors. These lesions have clinicopathological similarities, but their relationship remains controversial. This study aimed to characterise a series of cases of SCD34FT and PRDM10‐STT. Methods and results: Ten lesions each of SCD34FT and PRDM10‐STT were studied using immunohistochemistry, array‐comparative genomic hybridisation (aCGH), RNA sequencing and exome sequencing. Tumors mainly occurred in young adults, were generally small (< 5 cm) and arose predominantly in the superficial soft tissues of the lower extremities. Follow‐up data were available in 15 cases (SCD34FT, n = 7, median 16 months; PRDM10‐STT, n = 8, median 14 months), local recurrences occurred in four cases (SCD34FT, two of 10; PRDM10‐STT, two of 10), while no distant spread was documented. Morphologically, tumors were relatively well‐circumscribed and composed of sheets and fascicles of spindle and pleomorphic cells showing low mitotic activity (< 1/mm²) without necrosis. Other findings included: granular cell change, lipoblast‐like cells, ectatic blood vessels with fibrinous material, myxoid stromal changes, metaplastic bone and increased mitotic activity (> 1/mm²). All tumors diffusely expressed CD34, while pan‐keratin and desmin were commonly seen focally. SynCAM3 was diffusely expressed in 12 cases (SCD34FT, n = 5; PRDM10‐STT, n = 7), independently of fusion status. aCGH profiles were 'flat' (PRDM10‐STT, n = 4; SCD34FT, n = 2) and exome sequencing showed no recurrent pathogenic mutations (PRDM10‐STT, n = 2; SCD34FT, n = 4). Overall, the only morphological features seen exclusively in PRDM10‐STT were myxoid stromal changes (three of 10) and metaplastic bone (two of 10). Conclusion: We expand the current knowledge on PRDM10‐STT and SCD34FT and provide additional evidence for considering them as overlapping entities.
- Subjects
SOFT tissue tumors; RNA sequencing; BLOOD vessels
- Publication
Histopathology, 2021, Vol 79, Issue 5, p810
- ISSN
0309-0167
- Publication type
Article
- DOI
10.1111/his.14429