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

• Main Authors: Ken Takao, Ayaka Tani, Tetsuya Suwa, Yayoi Kuwabara-Ohmura, Kenta Nonomura, Yanyan Liu, Takehiro Kato, Masami Mizuno, Takuo Hirota, Mayumi Enya, Katsumi Iizuka, Yukio Horikawa, Chiemi Saigo, Yusuke Kito, Tatsuhiko Miyazaki, Naoyuki Ohe, Toru Iwama, Daisuke Yabe
• Format: Article
• Language: English
• Published: BMC 2021-01-01
• Series: BMC Endocrine Disorders
• Subjects: PCNSL; Hypothalamus; Hypopituitarism; Case report
• Online Access: https://doi.org/10.1186/s12902-020-00675-5

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.