Yonsei Med J.  2013 May;54(3):791-796. 10.3349/ymj.2013.54.3.791.

Primary Central Nervous System ALK Positive Anaplastic Large Cell Lymphoma with Predominantly Leptomeningeal Involvement in an Adult

• Park JS
• Park H
• Park S
• Kim SJ
• Seol HJ
• Ko YH

• KMID: 1727900
• DOI: http://doi.org/10.3349/ymj.2013.54.3.791

Abstract

A 31-year-old Korean male presented with altered consciousness and severe headache. Brain MRI delineated focal leptomeningeal enhancement without any intracerebral lesions. Diagnosis was made based on a brain biopsy showing anaplastic large cell lymphoma (ALCL), immunohistochemical stains revealing positivity for anaplastic lymphoma kinase (ALK) and an absence of involvement in any other organs; specifically, the primary central nervous system ALK+ALCL. Complete remission was achieved following 5 cycles of systemic chemotherapy with a high dose of Methotrexate and a simultaneous 7 cycles of intrathecal triple chemotherapy. Diagnosis of primary leptomeningeal ALK+ALCL is challenging given its rarity and non-specific symptoms along with non-pathognomonic radiologic findings. We present the first case of primary leptomeningeal ALK-positive ALCL where the clinical course, pathologic characteristics and treatment modality are described as well as a review of literature.

Keyword

ALK-positive; primary; CNS; anaplastic large-cell lymphoma; leptomeningeal

MeSH Terms

Adult
Antineoplastic Agents/therapeutic use
Biopsy
Brain/metabolism/pathology
Diagnosis, Differential
Humans
Immunohistochemistry
Lymphoma, Large-Cell, Anaplastic/*diagnosis/drug therapy/pathology
Male
Meningeal Neoplasms/*diagnosis/drug therapy/pathology
Receptor Protein-Tyrosine Kinases/*metabolism
Antineoplastic Agents
Receptor Protein-Tyrosine Kinases

Figure

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  Fig. 1 Magnetic resonance imaging of his brain showed hyperintense signal in the right temporal sulci from the Flair image (A) and leptomeningeal enhancement in the right temporal and insular gyri from Gadolinium enhanced image (B).

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  Fig. 2 The histologic section showed brain parenchyma infiltrated by numerous small-to-medium sized neoplastic cells with perivascular cuffing of neoplastic cells (A, HE ×100) and the neoplastic cells had irregular nuclei with a moderate amount of cytoplasm (B, HE ×400). Large atypical cells with horseshoe shaped nuclei, which are hallmark cells of anaplastic large cell lymphoma, were also present (B, inset, HE ×1000)

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  Fig. 3 Immunohistochemical stains revealed immunopositivity for CD30, ALK, Granzyme B, CD45RO and EMA. ALK, anaplastic lymphoma kinase.

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  Fig. 4 Fluorescence in situ hybridization for translocations involving ALK at 2p23: a unique sequence break apart probe targeting ALK gene locus was used. Translocation involving ALK at 2p23 was detected. ALK, anaplastic lymphoma kinase.

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