Primary central nervous system anaplastic large-cell lymphoma mimicking lymphomatosis cerebri
Primary central nervous system lymphoma (PCNSL) is usually diffuse large B-cell lymphoma. Anaplastic large-cell lymphoma (ALCL) rarely occurs in the central nervous system. PCNSL always presents as single or multiple nodular contrast-enhancing mass lesions within T2-hyperintense areas on magnetic resonance imaging (MRI). Infrequently, diffuse infiltrating change with little contrast enhancement called lymphomatosis cerebri can be seen in PCNSL. In this report, we describe a 75-year-old immunocompetent man who had progressive dementia. On MRI, diffuse white matter lesions with little contrast enhancement were observed to gradually progress, which was clinically consistent with his worsening condition. A biopsy specimen revealed non-destructive, diffusely infiltrating, anaplastic large CD30-positive lymphoma, indicating a diagnosis of ALCL. After the biopsy, he was treated by whole brain irradiation (total 46 Gy) and focal boost irradiation (total 14 Gy). However, his performance status worsened and there was no symptom improvement. The patient died 8 months after symptom onset. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described herein.
KeywordsLymphomatosis cerebri PCNSL ALCL ALK-1
- 8.George DH, Scheithauer BW, Aker FV, Kurtin PJ, Burger PC, Cameselle-Teijeiro J, McLendon RE, Parisi JE, Paulus W, Roggendorf W, Sotelo C (2003) Primary anaplastic large-cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. Am J Surg Pathol 27(4):487–493PubMedCrossRefGoogle Scholar
- 26.Tajima Y, Miyazaki Y, Higashi T, Kishimoto R, Sudoh K, Matsumoto A, Kikuchi S, Tashiro K (2003) Primary CD30/Ki-1 positive anaplastic large-cell lymphoma of the central nervous system occurring in a patient with a seventeen-year history of essential thrombocythemia. Leuk Lymphoma 44(7):1243–1245PubMedCrossRefGoogle Scholar