Superficial CD34-positive fibroblastic tumor and PRDM10-rearranged soft tissue tumor are overlapping entities: a comprehensive study of 20 cases
Perret, Raul Michal, Michael Velasco, Valérie Švajdler, Marian Karanian, Marie Meurgey, Alexandra Paindavoine, Sandrine Soubeyran, Isabelle Coindre, Jean-Michel
Perret, Raul
Michal, Michael
Velasco, Valérie
Švajdler, Marian
Karanian, Marie
Meurgey, Alexandra
Paindavoine, Sandrine
Soubeyran, Isabelle
Coindre, Jean-Michel
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Affiliation
Institut Bergonié, Bordeaux, France; Charles University, Plzen, Czech Republic; Bioptical Laboratory Ltd, Plzen, Czech Republic; South Warwickshire University NHS Foundation Trust; Centre Leon Berard, Lyon, France; Université Lyon, France; University of Bordeaux, Talence, France; Centre Georges-François Leclerc, Dijon, France; Institut Bergonié, Bordeaux, France; Strasbourg Regional University Hospital (Hautepierre Hospital), France; INSERM U1218, Action Unit, Bordeaux, France
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2021-11
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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.
Citation
Perret R, Michal M, Carr RA, Velasco V, Švajdler M, Karanian M, Meurgey A, Paindavoine S, Soubeyran I, Coindre JM, Boidot R, Charon-Barra C, Geneste D, Weingertner N, Pissaloux D, Tirode F, Baud J, Le Loarer F. Superficial CD34-positive fibroblastic tumor and PRDM10-rearranged soft tissue tumor are overlapping entities: a comprehensive study of 20 cases. Histopathology. 2021 Nov;79(5):810-825. doi: 10.1111/his.14429. Epub 2021 Sep 5.
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