Abstract

Extrarenal rhabdoid tumours appearing in the soft tissues are very rare and might be confused with other high-grade sarcomas harbouring rhabdoid features. We present a case and highlight the differential diagnosis, the ancillary techniques necessary to define its true rhabdoid nature, and the existence of hereditary syndromes.

Highlights

  • Herbert Plasschaert1*, Frederik Deman2, Isabelle Vanden Bempt3, Veerle Labarque4, Michael Aertsen5, Hilde Brems3 and Raf Sciot2

  • Extrarenal rhabdoid tumours appearing in the soft tissues are very rare and might be confused with other high-grade sarcomas harbouring rhabdoid features

  • There was no loss of SMARCB1/INI1 in the PDGFB region (22q11) and no ETV6-NTRK3 rearrangement was found

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Summary

Introduction

Herbert Plasschaert1*, Frederik Deman2, Isabelle Vanden Bempt3, Veerle Labarque4, Michael Aertsen5, Hilde Brems3 and Raf Sciot2. Extrarenal rhabdoid tumours appearing in the soft tissues are very rare and might be confused with other high-grade sarcomas harbouring rhabdoid features. Imaging based on US and MRI revealed a large soft tissue tumour invading the pleura of the left lung (Figure 1). The initial histological differential diagnosis concerned Ewing sarcoma, congenital fibrosarcoma, pleuropulmonary blastoma, synovial sarcoma and rhabdoid tumour of soft tissue.

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