Ramirez-Loera C, Sandoval-López D, Mejía-Pérez S

Language: English
References: 17
Page: 64-67
PDF size: 247.67 Kb.

ABSTRACT

Introduction: Neurofibromatosis type 1 (NF-1) comprises a spectrum of malignant lesions. Central nervous system (CNS) tumors, such as malignant gliomas, appear as predominant clinical manifestation. Malignant lymphomas constitute a rare entity in adults with NF-1, but there can be atypical presentations mimicking other brain tumors.
Case presentation: We report the very unusual case of a 53-year-old Mexican female with history of NF-1, who developed a primary central nervous system lymphoma (PCNSL) as first neoplastic lesion. She initially presented sub-acute and progressive left-sided numbness and weakness, leading to hemiplegia. mri showed an heterogenous left central lobe lesion with surrounding edema. After lesion-centered craniectomy for surgical resection, pathology revealed lymphoid lineage cells. Immunohistochemical staining showed positivity of CD20, PAX-5, Ki-67, validating pcnsl diagnosis. 6-months follow-up mri showed no residual mass. Two months later patient recovered fully.
Conclusion: PCNSL is a malignant serious neurologic condition with symptoms that mimic other brain tumors. Patients with NF-1 can initially present these lesions. Diagnosis should be considered within the NF-1 spectrum, and subsequently, a thorough study of this entity should be made, since pathophysiological mechanisms behind it are still unclear. Studies of prevalence and further guidelines are mandatory to critically appraise its medical and surgical management.

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