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...
Gespeichert in:
| Hauptverfasser: | , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
12 April 2012
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| In: |
Diagnostic pathology
Year: 2012, Jahrgang: 7, Pages: 38 |
| ISSN: | 1746-1596 |
| DOI: | 10.1186/1746-1596-7-38 |
| Online-Zugang: | 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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| Verfasserangaben: | Timo Gaiser, Eva Geissinger, Torsten Schattenberg, Hanns-Peter Scharf, Matthias Dürken, Dietmar Dinter, Andreas Rosenwald and Alexander Marx |
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| 245 | 1 | 0 | |a Case report |b a unique pediatric case of a primary CD8 expressing ALK-1 positive anaplastic large cell lymphoma of skeletal muscle |c Timo Gaiser, Eva Geissinger, Torsten Schattenberg, Hanns-Peter Scharf, Matthias Dürken, Dietmar Dinter, Andreas Rosenwald and Alexander Marx |
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| 520 | |a 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. | ||
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