Journal of Korean Endocrine Society, 23(4):260-265, 2008
Journal of Korean Endocrine Society; 대한내분비학회지
저자들은 시상 및 시상하부를 침범한 원발성 중추신경계 림프종에 의한 완전 중추성 요붕증 및 뇌하수체전엽기능저하증을 보이는 증례를 경험하였기에 문헌고찰과 함께 이를 보고하는 바이다.
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.
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