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Case Reports
. 2015 Mar;42(3):182-187.
doi: 10.1111/cup.12446. Epub 2015 Feb 5.

ALK-positive (2p23 rearranged) anaplastic large cell lymphoma with localization to the skin in a pediatric patient

Melissa Pulitzer  1 , Olakunle Ogunrinade  2 , Oscar Lin  1 , Peter Steinherz  3
Affiliations
Case Reports

ALK-positive (2p23 rearranged) anaplastic large cell lymphoma with localization to the skin in a pediatric patient

Melissa Pulitzer et al. J Cutan Pathol. 2015 Mar.

Abstract

Anaplastic large cell lymphoma (ALCL) either as primary cutaneous or nodal disease is rare in children and difficult to distinguish, which is important both prognostically and for treatment purposes. We present a case of anaplastic lymphoma kinase (ALK)+ skin-limited ALCL that highlights this challenge and draws attention to pitfalls in assessing ALK status. The patient was an 11-year old girl with a twice recurrent nodule on her right shoulder. Each biopsy revealed a deep infiltrate of atypical lymphocytes that expressed CD3, CD4, CD43, CD45RO and CD30. The initial biopsy was epithelial membrane antigen (EMA)+ with vague cytoplasmic ALK-1 positivity by immunohistochemistry, while the second biopsy was EMA+ and nuclear ALK-1+. Fluorescence in situ hybridization analysis for an ALK (2p23) rearrangement of the first specimen was negative, while an ALK gene rearrangement was present in the second specimen. Therefore, this case was treated as nodal ALCL, despite negative bone marrow and radiographic imaging studies. The patient was treated with combination chemotherapy and remains disease-free. Demonstration of nuclear ALK-positivity, ALK (2p23) gene rearrangement is suggestive of systemic ALCL. Without evidence of systemic disease, this case highlights challenges of skin-limited ALCL, whose clinical behavior as either cutaneous ALCL systemic ALCL may not be immediately apparent.

Keywords: EMA; anaplastic large cell lymphoma; anaplastic lymphoma kinase; nodal ALCL; skin-limited ALCL.

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Figures

Fig. 1
Fig. 1
A) Skin biopsy specimen shows a pan-dermal infiltrate of monomorphic atypical lymphocytes from the first nodule, ×40, hematoxylin and eosin. B) Atypical lymphocytes from the first nodule are depicted, ×100, hematoxylin and eosin. C) There is a perivascular and intravascular infiltrate of atypical lymphocytes in the second nodule, ×40, hematoxylin and eosin. D) Skin biopsy specimen demonstrates a superficial and deep periadnexal infiltrate of leomorphic lymphocytes in the second nodule, ×40, hematoxylin and eosin.
Fig. 2
Fig. 2
Immunohistochemical stains from the second nodule are depicted: CD30 (A), anaplastic lymphoma kinase (ALK) (B), ×40.
Fig. 3
Fig. 3
Fluorescence in situ hybridization (FISH) using a dual-color 2p23 LSI anaplastic lymphoma kinase (ALK) locus-specific probe on a paraffin-embedded section from the skin biopsy of the second nodule demonstrates separation of the red and green signals, thus indicating rearrangement in the 2p23 anaplastic lymphoma kinase gene locus.
See this image and copyright information in PMC

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