Archives of Pathology & Laboratory Medicine
Anaplastic Large T-Cell Lymphoma, ALK, Central Nervous System Lymphoma
Anaplastic large cell lymphoma (ALCL) is a rare T-cell lymphoma usually ALK+ and presenting in immunocompetent young adults and children with a female preponderance. Central nervous system (CNS) involvement of ALCL T-cell lymphoma is extremely rare; approximately 30 cases are reported in the literature, mostly in male patients of Korean and Japanese ancestry and involving the parietal and frontal lobes. We report a unique case of ALK+ ALCL in an Asian woman and predominantly involving the occipital lobe. The patient, an immuno-competent 18-year-old woman with a recent history of viral meningitis, presented with left-sided vision changes. Magnetic resonance imaging showed a mass in the medial left occipital gyrus, with no evidence of disease outside the CNS. The patient underwent stereotactic biopsy of the mass. Histopathologic sections showed neural tissue with perivascular cuffing and diffuse parenchymal and leptomeningeal infiltration of discohesive, pleomorphic predominantly large cells with abundant cytoplasm, round to irregular nuclei, dispersed chromatin, and prominent nucleoli. Immunohistochemistry showed that the large cells were positive for CD3, CD7, CD8, ALK1, CD25, EMA, granzyme B, and CD30, which confirmed the diagnosis of ALK+ primary CNS ALCL. Interphase FISH hybridization studies were positive for ALK-associated translocation t(2;5). Because of the rarity of this lymphoma, the pathogenesis, prognostic factors, and treatment strategies have not been well studied, except for ALK positivity, which is associated with a good prognosis. We believe this case will be a unique addition to the cases previously reported in the literature on this extremely rare lymphoma.
Jariwala, A., Almira-Suarez, M., & Lee, E. (2017). ALK-Positive Primary Central Nervous System Anaplastic Large T-Cell Lymphoma: A Unique Case Presentation. Archives of Pathology & Laboratory Medicine, 141 (9). Retrieved from https://hsrc.himmelfarb.gwu.edu/smhs_path_facpubs/177