トルコ鞍内病変による視力障害にて発病した全身性B細胞悪性リンパ腫の1例

Systemic Non-Hodgkin Lymphoma Initially Presented with Visual Disturbance due to Intrasellar Lymphoma : A Case Report

I．はじめに

　中枢神経症状で初発した悪性リンパ腫のほとんどすべては中枢神経系原発悪性リンパ腫であり，中枢神経症状で初発する全身性non-Hodgkinリンパ腫はきわめてまれである^4-6,8）．われわれは，トルコ鞍内腫瘤による視力障害にて発病した全身性non-Hodgkinリンパ腫（B細胞型）と思われる1例を経験したので，診断・治療上の問題点に考察を加えて報告する．

A case with systemic non-Hodgkin lymphoma involving the sella turcica and kidney is reported. A 69- year-old man presented with a progressive two month history of visual disturbance and headache. Neurol- ogical examination revealed bilateral visual disturbance and right optic atrophy. MRI showed a contrast- enhancing mass in the sella turcica. The tumor extended to the right optic nerve. Without extensive studies for systemic disease, the patient immediately underwent transsphenoidal surgery. The slightly firm, fibrous and vascular-rich tumor was subtotally removed. Thehistopathological examination revealed a malignantlymphoma, diffuse-large-cell type with B-cell phenotype. The postoperative course was uneventful and thepatient's symptoms subsided gradually. The patient received radiation therapy and the tumor disappeared.Postoperative CT examinations of the abdomen and pelvis revealed a large mass at the upper portion ofthe left kidney. Ga-scan also suggested the mass to be consistent with the abdominal CT. However, the pa-tient suddenly died of acute heart failure with unknown cause just before starting chemotherapy for syste-mic lymphoma.

Patients presenting primary central nervous system lymphoma (PCNSL) may have systemic non-Hodg-kin lymphoma. To exclude systemic non-Hodgkin lymphoma, systemic investigation is essential for the ini-tial management of patients presenting PCNSL.