뇌하수체기능저하증과 중추성 요붕증을 동반한 원발성 중추신경계 림프종 1예

• Published in: Endocrinology and metabolism (Seoul) pp. 260 - 265
• Main Authors: 안미선, 김순선, 김태호, 한승진, 김대중, 김효철, 김세혁, 한재호, 김호성, 정윤석
• Format: Journal Article
• Language: Korean
• Published: 대한내분비학회 01.08.2008
• Subjects: 내과학
• ISSN: 2093-596X; 2093-5978

Primary central nervous system (CNS) lymphoma is an uncommon neoplasm. However, the incidence of primary CNS lymphoma has increased more than 10-fold over the past three decades, and continues to accelerate. Currently, primary CNS lymphoma represents 4 to 7 percent of all newly diagnosed primary CNS tumors. Primary CNS lymphoma may arise from different parts of the brain, with deep hemispheric periventricular white matter being the most common site of origin. The presenting symptoms in primary CNS lymphoma vary depending on the location of the mass. Involvement of the hypothalamic-pituitary axis may cause hypopituitarism, diabetes insipidus, headache, diplopia, and blurred vision. We experienced a case of a 58-year-old woman who developed central diabetes insipidus and panhypopituitarism secondary to primary CNS lymphoma. Hypothalamic and thalamic involvement were suspected based on brain MRI, and primary CNS lymphoma was confirmed by a CT-guided stereotactic biopsy. Through performing a water deprivation test and a combined pituitary stimulation test, we diagnosed complete type central diabetes insipidus and panhypopituitarism. Symptomatic relief was obtained with desmopressin, levothyroxine, hydrocortisone, and high-dose methotrexate-based chemotherapy. The thalamic and hypothalamic masses were significantly decreased in size after chemotherapy. We report the details of this case along with a review of the literature concerning primary CNS lymphom Primary central nervous system (CNS) lymphoma is an uncommon neoplasm. However, the incidence of primary CNS lymphoma has increased more than 10-fold over the past three decades, and continues to accelerate. Currently, primary CNS lymphoma represents 4 to 7 percent of all newly diagnosed primary CNS tumors. Primary CNS lymphoma may arise from different parts of the brain, with deep hemispheric periventricular white matter being the most common site of origin. The presenting symptoms in primary CNS lymphoma vary depending on the location of the mass. Involvement of the hypothalamic-pituitary axis may cause hypopituitarism, diabetes insipidus, headache, diplopia, and blurred vision. We experienced a case of a 58-year-old woman who developed central diabetes insipidus and panhypopituitarism secondary to primary CNS lymphoma. Hypothalamic and thalamic involvement were suspected based on brain MRI, and primary CNS lymphoma was confirmed by a CT-guided stereotactic biopsy. Through performing a water deprivation test and a combined pituitary stimulation test, we diagnosed complete type central diabetes insipidus and panhypopituitarism. Symptomatic relief was obtained with desmopressin, levothyroxine, hydrocortisone, and high-dose methotrexate-based chemotherapy. The thalamic and hypothalamic masses were significantly decreased in size after chemotherapy. We report the details of this case along with a review of the literature concerning primary CNS lymphom KCI Citation Count: 0