Superficial CD34‐positive fibroblastic tumor and PRDM10‐rearranged soft tissue tumor are overlapping entities: a comprehensive study of 20 cases

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 SCD34...

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Veröffentlicht in:Histopathology Jg. 79; H. 5; S. 810 - 825
Hauptverfasser: 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
Format: Journal Article
Sprache:Englisch
Veröffentlicht: England Wiley Subscription Services, Inc 01.11.2021
Wiley
Schlagworte:
Adolescent
Adult
Antigens, CD34 - metabolism
Biomarkers, Tumor
Blood vessels
Bone tumors
Cancer
CD34 antigen
comparative genomic hybridisation
Desmin
DNA-Binding Proteins - genetics
DNA-Binding Proteins - metabolism
Female
Fibroblasts - pathology
Gene Rearrangement
Humans
Hybridization
Immunohistochemistry
Keratin
Life Sciences
Male
Mesenchyme
Middle Aged
PRDM10
RNA sequencing
sarcoma
Soft Tissue Neoplasms - genetics
Soft Tissue Neoplasms - metabolism
Soft Tissue Neoplasms - pathology
Soft tissues
superficial CD34‐positive fibroblastic tumor
Transcription Factors - genetics
Transcription Factors - metabolism
Tumors
Young Adult
Young adults
RNA sequencing
comparative genomic hybridisation
sarcoma
superficial CD34-positive fibroblastic tumor
PRDM10
ISSN:0309-0167, 1365-2559, 1365-2559
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Zusammenfassung: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.
Bibliographie:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:0309-0167
1365-2559
1365-2559
DOI:10.1111/his.14429