PMID: 9543674Apr 17, 1998Paper

Solid alveolar rhabdomyosarcoma of the thorax in a child

Histopathology
H SarteletE Brambilla

Abstract

This case illustrates the difficulties and pitfalls of diagnosis of alveolar rhabdomyosarcoma in its solid variant and in an unusual primary location, the mediastinum. A 9-year-old boy presented with a primary thoracic tumour associated with metastasis in the left sacroiliac joint. Bronchial and mediastinal biopsies showed a malignant neoplasm with a solid sheet-like pattern of small round cells with a high nuclear to cytoplasmic ratio associated with little or no fibrosis usually evocative of a peripheral neuroectodermal tumour (PNET) at this age. Immunohistochemical positive staining with vimentin (80% of tumour cells), desmin (20%) and titin (30%) antibodies was suggestive of a rhabdomyosarcoma. In addition, all neural cell adhesion molecule (NCAM) markers tested were positive as well as MIC2, a marker for the Ewing family of sarcomas. There was no rhabdomyoid differentiation at ultrastructural examination. Molecular analysis with RT-PCR amplification of RNA isolated from the tumour demonstrated the presence of a PAX3/FKHR fusion transcript, product of a t(2;13) reciprocal translocation, a genetic marker specific for alveolar rhabdomyosarcoma. The diagnostic methodology of a small round cell tumour of the child must now incl...Continue Reading

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Citations

Jun 15, 2011·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Jinglan LiuJean-Pierre de Chadarévian
Nov 18, 2008·Journal of Pediatric Hematology/oncology·Prasanth GanesanSameer Bakhshi
Sep 12, 2015·Virchows Archiv : an International Journal of Pathology·Michael A den BakkerPhilipp Ströbel
Dec 13, 2005·Cancer Genetics and Cytogenetics·Nuno CerveiraManuel R Teixeira
Sep 13, 2016·Expert Review of Respiratory Medicine·Giuseppe PelosiAngelica Sonzogni
May 23, 2000·Human Pathology·S LantuéjoulE Brambilla
Dec 21, 2007·Virchows Archiv : an International Journal of Pathology·Tadaki MatsumuraTadashi Hasegawa

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