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Abstract
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
Figures and Tables
Fig. 1
Homogeneously well enhancing mass lesion was noted in bilateral medial thalamus and hypothalamus on T1-weighted image. Extension into bilateral optic chiasm and proximal optic nerve was suspected. Minimal surrounding brain parenchymal edema was noted. Pituitary stalk was thickened.
Fig. 2
Microscopic section from a tumor specimen. A. The tumor cells were pleomorphic with large nuclei and a coarse chromatin pattern. Tumor cells were clustered around a cerebral blood vessel in a pattern typical for primary central nervous system lymphoma. (hematoxylin and eosin stain, ×400). B. Tumor cells expressed the CD20 and showed dark membrane staining. (CD20 immunohistochemical stain, ×400)
Fig. 3
After chemotherapy, brain MRI T1-weighted image showed decreased tumor size on both thalamus and hypothalamus, and diminished brain parenchymal edema.
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