Diagnosis and treatment of primary central nervous system lymphoma with the primary lesion in the hypothalamus: a case report

Abstract

Background Primary central nervous system lymphoma is a rare extra-nodal lymphoma of the central nervous system. Primary central nervous system lymphoma lesions usually appear in the vicinity of the ventricle, and there are few reports of primary central nervous system lymphoma with hypothalamic-pituitary lesions. Case presentation We treated a 56-year-old male with primary central nervous system lymphoma with the primary lesion in the hypothalamus, which was found by magnetic resonance imaging after sudden onset of endocrinological abnormalities. Initially, he was hospitalized to our department for hyponatremia. Endocrinological examination in conjunction with head magnetic resonance imaging and endoscopic biopsy revealed hypothalamic hypopituitarism and tertiary hypoadrenocorticism caused by a rapidly growing, diffuse large B-cell lymphoma in the hypothalamus. Remission of the tumor was achieved by high-dose methotrexate with whole brain radiotherapy, and some of the hormone responses were normalized. Conclusions While primary central nervous system lymphoma is rare, it is important to note that hypopituitarism can result and that the endocrinological abnormalities can be partially restored by its remission.

The sellar region as presenting theater for hematologic malignancies—A 17-year single-center experience

“…In our study, hypopituitarism was present in two out of five patients (both with lymphoma); the patient with AML had isolated diabetes insipidus, while hyperprolactinemia due to compression of the pituitary stalk was present in three patients. Hypothalamo-pituitary dysfunction may recover (fully or partially) after treatment of lymphoma or may persist, necessitating lifelong hormone replacement therapy [10,15,16,20,21,26,27]. In our study, hypothalamopituitary dysfunction completely normalized in the patient with IVL, the only one with long lasting remission.…”