Primary leptomeningeal presentation of T-cell lymphoma: Report of a patient and review of the literature

Abstract

Although secondary leptomeningeal lymphoma is relatively common, primary leptomeningeal lymphoma is rare. We report a patient with the extremely unusual clinical and pathologic presentation of a primary T‐cell lymphoma of the leptomeninges. Diagnosis was established by morphologic and immunologic studies of cerebrospinal fluid (CSF) and brain biopsy. The leptomeningeal lymphoma responded well to intrathecal chemotherapy and irradiation but th epatient has severe residual motor paralysis. The clinical and pathological features of primary and secondary central nervous system (CNS) lymphoma are reviewed in reference to this most unusual patient.

Radiation therapy for neoplasms of the brain

“…62'157'161 However, at least one case of primary leptomeningeal T cell lymphoma has been reported. 97 In immunosuppressed or immunocomproraised patients, primary CNS lymphomas occur with greater frequency t4~ and at a younger age than in nonimmunologically suppressed individuals. 54 During the last few years there has been a substantial increase in CNS lymphoma, especially in patients with acquired immunodeficiency syndrome.…”

Primary leptomeningeal lymphoma

“…The estimated incidence of PLML is 7% of all PCNSLs, which itself comprises approximately 2% of all primary brain tumors and 0.8% of all lymphomas. 5 There are few case reports or small series, [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] with the largest published case series consisting of 9 patients. In that series, the median age at diagnosis was 57 years, all patients had abnormal CSF, and the median survival was only 8 months.…”

Avaliação neurológica de pacientes adultos com linfoma não-Hodgkin: estudo prospectivo

“…Os sintomas e sinais neurológicos foram considerados em pacientes com manifestações clínicas associadas à presença tumoral confirmada pela biópsia, necrópsia e/ou presença de células neoplásicas no LCR, incluindo-se ainda doentes que tiveram melhora da sintomatologia após introdução da terapêutica. Não foi abordada a detecção de anticorpos anti-HIV no soro, possivelmente porque as observações predominaram em período que antecedeu à participação significativa de casos de SIDA [14][15][16][17] .…”