Bandaru Sindhura, Jaju Amruta, Manthri Sukesh, Mamillapalli Chaitanya, Jakoby Michael G
Endocrinology, Terrebonne General Medical Center, Springfield, USA.
Endocrinology, Southern Illinois University School of Medicine, Springfield, USA.
Cureus. 2021 Dec 1;13(12):e20086. doi: 10.7759/cureus.20086. eCollection 2021 Dec.
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder characterized by tumors of parathyroid, anterior pituitary, and pancreatic islet cells. Pituitary adenomas in MEN1 can be aggressive and invade surrounding structures including the skull base. However, acute bacterial meningitis in patients with newly diagnosed macroprolactinomas is an exceptional finding. We present the case of a young man with suppurative meningitis complicating an invasive macroprolactinoma as the initial manifestation of MEN1. A 33-year-old male was admitted to the hospital with fever, headache, and nuchal rigidity and subsequently diagnosed with bacterial meningitis. Computed tomography (CT) and subsequent magnetic resonance imaging (MRI) of the sella turcica revealed a 5 x 3.5 cm pituitary mass invading both cavernous sinuses and the left sphenoid sinus. Laboratory evaluation was notable for significantly elevated serum prolactin level (2,484 ng/mL, 2.6-13.2) and evidence of hypopituitarism. Primary hyperparathyroidism was indicated by hypercalcemia (13.5 mg/dL, 8.5-10.5), low serum phosphorus (2.0 mg/dL, 2.5-4.9), and elevated intact parathyroid hormone (PTH) level (290 pg/mL, 15-60). No visual field deficits were identified. The patient was managed with hydrocortisone, levothyroxine, and cabergoline. However, cerebral spinal fluid (CSF) rhinorrhea compelled subtotal transsphenoidal resection of the tumor and repair of the CSF leak. Three-and-a-half gland parathyroid resection was performed after recovery from pituitary surgery and successfully treated hypercalcemia. Abdominal MRI revealed a 1.2 cm cystic mass in the neck of the pancreas, and pancreatic polypeptide was approximately fourfold elevated. A clinical diagnosis of MEN1 was made based on the occurrence of macroprolactinoma, multiple parathyroid adenomas, and pancreatic findings. This case appears to be the first in which bacterial meningitis was the initial presentation of an invasive macroprolactinoma in a patient with MEN1.
多发性内分泌腺瘤1型（MEN1）是一种罕见的常染色体显性疾病，其特征为甲状旁腺、垂体前叶和胰岛细胞肿瘤。MEN1患者的垂体腺瘤可能具有侵袭性，并侵犯包括颅底在内的周围结构。然而，新诊断的大泌乳素瘤患者发生急性细菌性脑膜炎是一种罕见情况。我们报告一例年轻男性，其化脓性脑膜炎并发侵袭性大泌乳素瘤，为MEN1的首发表现。一名33岁男性因发热、头痛和颈项强直入院，随后被诊断为细菌性脑膜炎。蝶鞍的计算机断层扫描（CT）及后续磁共振成像（MRI）显示一个5×3.5 cm的垂体肿块，侵犯双侧海绵窦和左侧蝶窦。实验室检查显著升高的血清泌乳素水平（2484 ng/mL，正常范围2.6 - 13.2）及垂体功能减退证据引人注目。高钙血症（13.5 mg/dL，正常范围8.5 - 10.5）、低血清磷（2.0 mg/dL，正常范围2.5 - 4.9）和升高的完整甲状旁腺激素（PTH）水平（290 pg/mL，正常范围15 - 60）提示原发性甲状旁腺功能亢进。未发现视野缺损。患者接受氢化可的松、左甲状腺素和卡麦角林治疗。然而，脑脊液鼻漏促使进行肿瘤次全经蝶窦切除术及脑脊液漏修补术。垂体手术后恢复后进行了甲状旁腺次全切除术，成功治疗了高钙血症。腹部MRI显示胰腺颈部有一个1.2 cm的囊性肿块，胰多肽水平约升高四倍。基于大泌乳素瘤、多发甲状旁腺腺瘤和胰腺检查结果，临床诊断为MEN1。该病例似乎是首例细菌性脑膜炎为MEN1患者侵袭性大泌乳素瘤首发表现的病例。
