Onset of primary brain T-lymphoma simulating a progressive leukoencephalopathy

Abstract

We describe the clinical, CT and MRI evolution of a patient with a primary T-lymphoma of the brain showing features similar to those of leukoencephalopathy. We report the findings that led to the cerebral biopsy performed and we discuss the striking features of this case in the light of previous reports in the literature.

Lymphomatosis Cerebri Presenting as Rapidly Progressive Dementia

“…11 Diffusely infiltrating PCNSL is very rare in immunocompetent patients and has infrequently been reported. [12][13][14][15][16][17] It is even rarer for T-cell lymphoma to present as diffuse cerebral infiltration. 18 Although we believe this to be a primary CNS lymphoma, the poorly characterized lowerextremity lesions may suggest the presence of a rare metastatic primary cutaneous T-cell lymphoma like mycosis fungoides (MF).…”

Primary brain T-cell lymphoma of the lymphoblastic type presenting as altered mental status

“…As in the present case, immunophenotyping demonstrating CD3 and TdT positivity confirms the diagnosis of T-cell lymphoblastic lymphoma. TPCNSL of the lymphoblastic type is an extremely rare tumor with only five reported cases in the literature (Table 1) [4, 11, 19–21]. Of the four which provided clinical characteristics, two were supratentorial, one was leptomeningeal, and one was located in the cerebellum.…”

“…The overall median age of patients with TPCNSL is 60 [22]. With the exception of one case report describing a 2-year-old female [4], the remaining cases and ours involve patients in the fifth and sixth decades [11, 19, 21]. There is a male predominance of TPCNSL [22].…”

“…Primary CNS lymphomas are most commonly diffuse large-cell lymphomas, with lymphoblastic types occurring in only 2.9% [15]. To our knowledge, of the reports which describe histologic analysis, only five report the lymphoblastic type of TPCNSL (Table 1) [4, 11, 19–21]. In this report, we present a 56-year-old man with altered mental status and diffuse non-enhancing magnetic resonance imaging (MRI) abnormalities in the brainstem, cerebellum, and cerebral hemisphere with pathology findings consistent with primary brain T-cell lymphoblastic lymphoma.…”

Primary T-cell lymphoma of the cerebellum

“…To date, there have been only about twenty reported cases of primary CNS T-cell lymphomas (Table 1) [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] most of them unassociated with AIDS [4]. In comparison with B-cell PLCNS posterior fossa and leptomeningeal tumours are more frequent [4].…”