Diagnostic utility of cyclin D1 in the diagnosis of small round blue cell tumors in children and adolescents: Beware of cyclin D1 expression in clear cell sarcoma of the kidney and CIC-DUX4 fusion–positive sarcomas. Comment on Magro et al. (2016)

• Published in: Human pathology 2017-09, Vol.67, p.225-226
• Main Authors: Creytens, David, MD, PhD, Ferdinande, Liesbeth, MD, PhD, Van Dorpe, Jo, MD, PhD
• Format: Article
• Language: English
• Subjects: Histopathology; Morphology; Neuroblastoma; Pathology; Pediatrics; Teenagers; Tumors
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2017.02.032

[...]cyclin D1 immunoreactivity was absent in rhabdomyosarcoma and lymphoblastic lymphoma. [...]this marker was also negative, or only focally positive, in the blastemal component of Wilms tumor and in the neoplastic cells of desmoplastic small round cell tumor, suggesting that cyclin D1 is a useful marker in separating EWS/pPNET and neuroblastoma from other (pediatric) small round blue cell tumors. Round cell sarcomas harboring CIC-DUX4 fusion, an important subgroup of pediatric primitive round cell sarcomas, are highly aggressive soft tissue tumors of children and young adults, mostly arising in deep or superficial soft tissue or very rarely in viscera and bone, which can mimic EWS both clinically and histologically [3-5]. [...]we have tested cyclin D1 (Clone SP4-R; Ventana, Tucson AZ) in 4 CIC-DUX4 fusion-positive round cell sarcomas. Similar to EWS, a strong and diffuse cyclin D1 expression was observed in all 4 cases (Fig. 2). [...]cyclin D1 expression is not helpful in differentiating this subgroup of fusion-positive round cell sarcomas from classical EWS.