Sclerosing rhabdomyosarcoma in childhood: case report and review of the literature.
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.
Vadgama
Bhumita
B
Department of Histopathology, Camelia Botnar Laboratories, Great Ormond Street Hospital for Children, London WC1N 3JH, UK.
Sebire
Neil James
NJ
Malone
Marian
M
Ramsay
Alan Drummond
AD
D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017570:MyoD Protein / Q000235:genetics / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012208:Rhabdomyosarcoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012447:Sacrum / Q000473:pathology*; D012598:Sclerosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein