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

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 t...

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Bibliographic Details
Main Authors: Gaiser, Timo (Author) , Schattenberg, Torsten (Author) , Scharf, Hanns-Peter (Author) , Dürken, Matthias (Author) , Dinter, Dietmar (Author) , Marx, Alexander (Author)
Format: Article (Journal)
Language:English
Published: 12 April 2012
In: Diagnostic pathology
Year: 2012, Volume: 7, Pages: 38
ISSN:1746-1596
DOI:10.1186/1746-1596-7-38
Online Access:Verlag, kostenfrei, Volltext: http://dx.doi.org/10.1186/1746-1596-7-38
Verlag, kostenfrei, Volltext: https://doi.org/10.1186/1746-1596-7-38
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Author Notes:Timo Gaiser, Eva Geissinger, Torsten Schattenberg, Hanns-Peter Scharf, Matthias Dürken, Dietmar Dinter, Andreas Rosenwald and Alexander Marx
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Summary: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.
Item Description:Gesehen am 11.06.2018
Physical Description:Online Resource
ISSN:1746-1596
DOI:10.1186/1746-1596-7-38

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