Department of Endocrinology and Metabolism, Kocaeli University College of Medicine, Kocaeli, 41000, Turkey.
Department of Pathology, Kocaeli University College of Medicine, Kocaeli, Turkey.
J Med Case Rep. 2021 May 25;15(1):293. doi: 10.1186/s13256-021-02866-7.
Primary central nervous system lymphoma (PCNSL) is a rare but well-known extra-nodal lymphoma, which usually presents with non-Hodgkin B-cell lymphomas. PCNSL is generally located around the ventricle and is often detected as multiple lesions. It is rarely seen in the area of the hypothalamus.
We report the case of a 48-year-old Caucasian woman with progressive short-term memory deterioration, headache, mental confusion, diabetes insipidus (DI) and hypopituitarism. Early findings were suggestive of a pituitary apoplexy. The results of tests performed during the initial admission at the tertiary health center revealed hypernatremia, hypopituitarism and DI. Intravenous hydrocortisone treatment was initiated for the secondary adrenal insufficiency, and 75 mcg/day of levothyroxine was started for the secondary hypothyroidism on the fourth day following hydrocortisone treatment. A daily dose of 120 mg desmopressin melt tablet was started twice a day for polyuria/polydipsia after the patient's volume status was balanced. A brain magnetic resonance imaging scan revealed a mass lesion in the hypothalamic area, which was surrounded by marked edema. Anti-edema treatment was initially started considering the suggestion by our neurosurgery team. The patient's clinical and laboratory findings improved after the initiation of the anti-edema therapy. Afterwards, a biopsy was performed, which diagnosed a malignant diffuse large B-cell lymphoma. Subsequently, intravenous high-dose methotrexate-based therapy was started; however, after the second cycle of chemotherapy, the patient died due to sepsis.
In this report, we present a case of hypopituitarism that developed due to the mass effect of hypothalamic lymphoma with clinical findings of pituitary apoplexy. Intracranial masses may cause obvious endocrinological findings related to hypopituitarism, while vague findings may also be observed due to partial failure. Therefore, it is important to perform a comprehensive endocrinological examination at the time of diagnosis in patients with intracranial masses.
原发性中枢神经系统淋巴瘤(PCNSL)是一种罕见但广为人知的结外淋巴瘤,通常表现为非霍奇金 B 细胞淋巴瘤。PCNSL 通常位于脑室周围,通常被检测为多个病变。很少在下丘脑区域发现。
我们报告了一例 48 岁白人女性,表现为进行性短期记忆恶化、头痛、精神错乱、尿崩症(DI)和垂体功能减退。早期发现提示垂体卒中。在三级保健中心初次入院期间进行的检查结果显示高钠血症、垂体功能减退和 DI。为继发性肾上腺功能不全开始静脉注射氢化可的松治疗,在氢化可的松治疗后第 4 天开始每天 75mcg 左甲状腺素治疗继发性甲状腺功能减退。在患者的容量状态平衡后,开始每天两次服用 120mg 去氨加压素片治疗多尿/多饮。脑磁共振成像扫描显示下丘脑区域有一个肿块病变,周围有明显的水肿。考虑到我们神经外科团队的建议,最初开始了抗水肿治疗。抗水肿治疗开始后,患者的临床和实验室检查结果有所改善。随后进行了活检,诊断为恶性弥漫性大 B 细胞淋巴瘤。随后,开始了静脉注射大剂量甲氨蝶呤为基础的治疗;然而,在第二个化疗周期后,患者因脓毒症死亡。
在本报告中,我们介绍了一例因下丘脑淋巴瘤的肿块效应导致的垂体功能减退病例,其临床表现为垂体卒中。颅内肿块可能导致明显的与垂体功能减退相关的内分泌学发现,而部分功能衰竭也可能观察到模糊的发现。因此,在诊断颅内肿块患者时,进行全面的内分泌检查非常重要。