Department of Diabetes and Endocrinology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.
Division of Pathology, Gifu University Hospital, Gifu, Japan.
BMC Endocr Disord. 2021 Jan 11;21(1):13. doi: 10.1186/s12902-020-00675-5.
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.
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.
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.
原发性中枢神经系统淋巴瘤是一种罕见的中枢神经系统结外淋巴瘤。原发性中枢神经系统淋巴瘤病变通常出现在脑室附近，而伴有下丘脑-垂体病变的原发性中枢神经系统淋巴瘤的报道较少。
我们治疗了一名 56 岁男性的原发性中枢神经系统淋巴瘤，其原发性病变位于下丘脑，在突然出现内分泌异常后通过磁共振成像发现。最初，他因低钠血症被收入我科。内分泌检查结合头部磁共振成像和内镜活检显示，由于生长迅速、弥漫性大 B 细胞淋巴瘤累及下丘脑，导致下丘脑垂体功能减退和三级肾上腺皮质功能减退。大剂量甲氨蝶呤联合全脑放疗使肿瘤缓解，部分激素反应正常化。
虽然原发性中枢神经系统淋巴瘤很少见，但需要注意的是，可能会导致垂体功能减退，且其缓解可使部分内分泌异常得到恢复。
