Wang Yong, Zhang Hui
Department of Radiology, the First Hospital of Hebei Medical University, Shijiazhuang 050000, China.
Department of Radiology, Hebei General Hospital, Shijiazhuang 050000, China.
J Int Med Res. 2021 Jan;49(1):300060520961682. doi: 10.1177/0300060520961682.
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant genetic disease. MEN1 with multiple endocrine adenomatosis complicated by multiple endocrine tumors is often misdiagnosed or missed. Herein, we describe the first reported case of refractory hypoglycemia and liver and lung metastases in a patient with MEN1.Case presentation: A 40-year-old man presented with a 3-month history of intermittent palpitations, fatigue, and sweating. The patient had a history of prolactinoma resection and refractory hypoglycemia 2 years earlier. Analyses of blood samples showed a decrease in random and fasting blood glucose and an increase in prolactin (PRL). Computed tomography (CT) and magnetic resonance imaging scans revealed two substantial masses in the pancreas and large masses in the liver and lung. Positron emission tomography-CT images showed hypermetabolic masses in the pancreatic body and tail. The liver and lung lesions were also hypermetabolic. The pancreatic lesion was surgically removed, and pathology confirmed that the mass was MEN1. The liver and lung masses were confirmed as metastatic tumors.
If clinicians better understand MEN1, they can obtain a detailed patient and family history during the initial visit, allowing earlier diagnosis and intervention and improved prognosis.
1型多发性内分泌腺瘤病（MEN1）是一种罕见的常染色体显性遗传病。MEN1合并多发性内分泌腺病并伴有多种内分泌肿瘤时，常被误诊或漏诊。在此，我们报告首例MEN1患者出现难治性低血糖及肝肺转移的病例。
一名40岁男性，有3个月间歇性心悸、乏力和出汗病史。该患者2年前有催乳素瘤切除及难治性低血糖病史。血液样本分析显示随机血糖和空腹血糖降低，催乳素（PRL）升高。计算机断层扫描（CT）和磁共振成像扫描显示胰腺有两个实质性肿块，肝脏和肺部有较大肿块。正电子发射断层扫描-CT图像显示胰体和胰尾有高代谢肿块。肝脏和肺部病变也呈高代谢。胰腺病变经手术切除，病理证实肿块为MEN1。肝脏和肺部肿块被确认为转移瘤。
如果临床医生对MEN1有更好的了解，他们可以在初次就诊时获取详细的患者和家族病史，从而实现早期诊断和干预，改善预后。
