Sclerosing rhabdomyosarcoma in childhood: case report and review of the literature

Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
Bhumita VadgamaAlan Drummond Ramsay

Abstract

Rhabdomyosarcoma is the most common soft tissue malignancy in children but is rare in adults. The latest World Health Organization classification of soft tissue tumors recognizes embryonal, alveolar, and pleomorphic rhabdomyosarcomas. More recently, a sclerosing variant of rhabdomyosarcoma has been recognized and reported in seven adult patients. We describe a pediatric case of sclerosing rhabdomyosarcoma presenting as a sacral mass in a 3-year-old girl. Morphologically, the tumor showed a prominent sclerosing hyaline matrix and demonstrated pseudovascular and microalveolar architectural foci. Focal positivity was seen with desmin, smooth muscle actin, and myogenin. MyoD1 showed uniform diffuse nuclear staining. Fusion transcripts were not demonstrated by reverse transcriptase-polymerase chain reaction analysis. The histology, immunohistochemistry, and molecular genetics matched those reported in the seven adult cases of sclerosing rhabdomyosarcoma. This is the first case report, to our knowledge, of this rare tumor arising in the pediatric age group, and we compare the features with those reported in adult sclerosing rhabdomyosarcoma.

References

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Citations

Feb 6, 2013·Diagnostic Pathology·Xu-Yong LinEn-Hua Wang
Sep 16, 2009·Annals of Diagnostic Pathology·Janez Lamovec, Metka Volavsek
Apr 16, 2014·APMIS : Acta Pathologica, Microbiologica, Et Immunologica Scandinavica·Bharat Rekhi, Tanvi Singhvi
Mar 4, 2017·Pathology·Dina El DemellawyAhmed Nasr
Sep 9, 2006·International Journal of Surgical Pathology·Eduardo ZambranoMiguel Reyes-Múgica
Feb 19, 2009·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Dorothée Bouron-Dal SoglioJean-Christophe Fournet
Apr 2, 2005·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Melissa C ChilesUNKNOWN Soft Tissue Sarcoma Committee of Children's Oncology Group
Apr 15, 2014·ISRN Oncology·Amandeep KumarBhawani S Sharma
Nov 9, 2006·Archives of Pathology & Laboratory Medicine·David M Parham, Dale A Ellison
Oct 2, 2007·Pathology, Research and Practice·Radzislaw KordekDorota Jesionek-Kupnicka
May 21, 2009·Human Pathology·Dorothée Bouron-Dal SoglioJean-Christophe Fournet

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