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Open Access Case Report

Case report: a unique pediatric case of a primary CD8 expressing ALK-1 positive anaplastic large cell lymphoma of skeletal muscle

Timo Gaiser15*, Eva Geissinger6, Torsten Schattenberg2, Hanns-Peter Scharf2, Matthias Dürken3, Dietmar Dinter4, Andreas Rosenwald6 and Alexander Marx1

Author affiliations

1 Institute of Pathology, Medical Faculty Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

2 Orthopedic Clinic, Medical Faculty Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

3 Department of Pediatrics, Medical Faculty Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

4 Radiology and Nuclear Medicine, Medical Faculty Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany

5 Section of Cancer Genomics, Center for Cancer Research, National Cancer Institute, National Institutes of Health, National Insitutes of Health, 50 South Drive, Bldg. 50, Bethesda, MD 20892, USA

6 Institute of Pathology, University of Würzburg, Josef-Schneider-Straße 2/Bau E2, 97080 Würzburg, Germany

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Citation and License

Diagnostic Pathology 2012, 7:38  doi:10.1186/1746-1596-7-38

Published: 12 April 2012

Abstract

Primary involvement of skeletal muscle is a very rare event in ALK-1 positive anaplastic large cell lymphoma (ALCL). We describe a case of a 10-year old boy presenting with a three week history of pain and a palpable firm swelling at the dorsal aspect of the left thigh. Histological examination of the lesion revealed a tumoral and diffuse polymorphic infiltration of the muscle by large lymphoid cells. Tumor cells displayed eccentric, lobulated "horse shoe" or "kidney-shape" nuclei. The cells showed immunohistochemical positivity for CD30, ALK-1, CD2, CD3, CD7, CD8, and Perforin. Fluorescence in situ hybridization analysis revealed a characteristic rearrangement of the ALK-1 gene in 2p23 leading to the diagnosis of ALK-1 positive ALCL. Chemotherapy according to the ALCL-99-NHL-BFM protocol was initiated and resulted in a complete remission after two cycles. This case illustrates the unusual presentation of a pediatric ALCL in soft tissue with a good response to chemotherapy.

Keywords:
ALK-1; Anaplastic large cell lymphoma; CD30; Pediatric lymphoma