TY - JOUR
T1 - Primary natural killer/T-cell lymphoma presenting as leptomeningeal disease
AU - Liao, Bing
AU - Kamiya-Matsuoka, Carlos
AU - Gong, Yun
AU - Chen, Merry
AU - Wolf, Brian A.
AU - Fowler, Nathan H.
PY - 2014/8/15
Y1 - 2014/8/15
N2 - Introduction Primary central nervous system natural killer/T-cell lymphoma (primary-CNS-NK/TCL) is a rare non-Hodgkin's lymphoma. To our knowledge, only five patients have been described previously, all of whom were male, with brain parenchymal involvement and previous Epstein-Barr virus infection, it has never been reported to present as leptomeningeal disease as our case. Our objective is to report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease and to share our diagnostic/therapeutic approach to this rare disease. Methods We report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease. The patient was diagnosed and treated at The University of Texas MD Anderson Cancer Center. Results The patient presented with multiple cranial neuropathies and gait ataxia. Brain and spinal cord magnetic resonance imaging demonstrated leptomeningeal enhancement of the cerebellar folia/vermis, spinal cord dura, and both temporal lobes as well as adjacent brain parenchymal disease. Cerebrospinal fluid (CSF) revealed atypical lymphoma cells of NK/T-cell lineage by flow cytometric immunophenotyping. Molecular analysis using real-time quantitative polymerase chain reaction did not detect Epstein-Barr virus DNA in the lymphoma cells. Bone marrow biopsy revealed no morphologic, flow cytometric, or immunohistochemical evidence of B-, T- or NK-cell lymphoma. Slit-lamp examination demonstrated no evidence of intraocular lymphoma. Whole-body PET scan showed no evidence of malignancy other than CNS disease. The patient was given systemic chemotherapy with high-dose methotrexate, vincristine, and procarbazine, along with intrathecal therapy with free cytarabine. The patient showed clinicoradiographic improvement and CSF cytology became negative. Conclusion This case highlights an atypical presentation of primary-CNS-NK/TCL with a potentially successful treatment regimen.
AB - Introduction Primary central nervous system natural killer/T-cell lymphoma (primary-CNS-NK/TCL) is a rare non-Hodgkin's lymphoma. To our knowledge, only five patients have been described previously, all of whom were male, with brain parenchymal involvement and previous Epstein-Barr virus infection, it has never been reported to present as leptomeningeal disease as our case. Our objective is to report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease and to share our diagnostic/therapeutic approach to this rare disease. Methods We report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease. The patient was diagnosed and treated at The University of Texas MD Anderson Cancer Center. Results The patient presented with multiple cranial neuropathies and gait ataxia. Brain and spinal cord magnetic resonance imaging demonstrated leptomeningeal enhancement of the cerebellar folia/vermis, spinal cord dura, and both temporal lobes as well as adjacent brain parenchymal disease. Cerebrospinal fluid (CSF) revealed atypical lymphoma cells of NK/T-cell lineage by flow cytometric immunophenotyping. Molecular analysis using real-time quantitative polymerase chain reaction did not detect Epstein-Barr virus DNA in the lymphoma cells. Bone marrow biopsy revealed no morphologic, flow cytometric, or immunohistochemical evidence of B-, T- or NK-cell lymphoma. Slit-lamp examination demonstrated no evidence of intraocular lymphoma. Whole-body PET scan showed no evidence of malignancy other than CNS disease. The patient was given systemic chemotherapy with high-dose methotrexate, vincristine, and procarbazine, along with intrathecal therapy with free cytarabine. The patient showed clinicoradiographic improvement and CSF cytology became negative. Conclusion This case highlights an atypical presentation of primary-CNS-NK/TCL with a potentially successful treatment regimen.
KW - CNS lymphoma
KW - Epstein-Barr virus
KW - Leptomeningeal disease
KW - Natural killer/T-cell lymphoma
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U2 - 10.1016/j.jns.2014.05.015
DO - 10.1016/j.jns.2014.05.015
M3 - Article
C2 - 24880537
AN - SCOPUS:84904267055
SN - 0022-510X
VL - 343
SP - 46
EP - 50
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 1-2
ER -