Umer Waseem, Mohammed Ahmed Salah, Khan Adeel Ahmad, Saddique Muhammad Umar, Zahid Muhammad
Internal Medicine Department Hamad Medical Corporation Doha Qatar.
Department of Endocrinology Hamad Medical Corporation Doha Qatar.
Clin Case Rep. 2023 Mar 14;11(3):e6967. doi: 10.1002/ccr3.6967. eCollection 2023 Mar.
Insulinomas are rare functioning neuroendocrine (NEN) tumors. Up to 10% of insulinomas are associated with multiple endocrine neoplasia 1 (MEN1). Most of the tumors present with symptomatic hypoglycemia. Several non-invasive and invasive techniques are used to localize the lesion. We present a case of insulinoma presenting with seizure episodes with negative results on non-invasive imaging diagnosed and localized with endoscopic ultrasound. A 36-year-old male was brought by ambulance to the emergency department with an episode of generalized tonic-clonic seizures. He had been previously healthy and did not have family history of neuro-endocrine tumors. At the time of the attack, the patient's blood glucose checked via point-of-care testing was 28.8 (70-99 mg/dL). He was given IV dextrose. Physical examination after the patient regained consciousness was completely unremarkable. Hypoglycemia workup revealed a normal morning cortisol level of 281 (138-689 nmol/L). Insulin level was 62.4 mcunit/ml (2.36-24.9), and c-peptide was 8.13 (1.1-4.4 ng/mL) consistent with hyperinsulinemia. Magnetic resonance cholangiopancreatography (MRCP), fluorine-18-l-dihydroxyphenylalanine whole-body positron emission tomography scan (NM 18F-DOPA whole-body PET scan), and gallium Ga 68 dodecanetetraacetic acid (Ga-68 DOTATATE) scan were normal and did not reveal any pancreatic lesion consistent with insulinoma. Due to high suspicion of insulinoma and negative non-invasive imaging, an endoscopic ultrasound (EUS) was performed, which showed a hypoechoic homogenous mass lesion sized 13 × 9 mm in the proximal body/neck of the pancreas. A fine needle biopsy (FNA) via EUS was performed. Histopathology showed a well-differentiated neuroendocrine tumor, consistent with Grade 1 insulinoma (T1N0M0). The patient underwent a distal pancreatectomy and splenectomy. In cases of high clinical and biochemical suspicion of insulinoma but negative non-invasive imaging, invasive modalities should be used to localize the culprit lesion.
胰岛素瘤是罕见的功能性神经内分泌(NEN)肿瘤。高达10%的胰岛素瘤与多发性内分泌腺瘤病1型(MEN1)相关。大多数肿瘤表现为症状性低血糖。有多种非侵入性和侵入性技术用于定位病变。我们报告一例胰岛素瘤患者,其表现为癫痫发作,非侵入性成像结果为阴性,最终通过内镜超声诊断并定位。一名36岁男性由救护车送至急诊科,发生一次全身性强直阵挛发作。他既往健康,无神经内分泌肿瘤家族史。发作时,通过即时检测测得患者血糖为28.8(70 - 99mg/dL)。给予其静脉注射葡萄糖。患者意识恢复后的体格检查完全无异常。低血糖检查显示早晨皮质醇水平正常,为281(138 - 689nmol/L)。胰岛素水平为62.4微单位/毫升(2.36 - 24.9),C肽为8.13(1.1 - 4.4ng/mL),符合高胰岛素血症。磁共振胰胆管造影(MRCP)、氟 - 18 - l - 二羟基苯丙氨酸全身正电子发射断层扫描(NM 18F - DOPA全身PET扫描)和镓Ga 68十二烷四乙酸(Ga - 68 DOTATATE)扫描均正常,未发现任何与胰岛素瘤相符的胰腺病变。由于高度怀疑胰岛素瘤且非侵入性成像为阴性,遂进行内镜超声(EUS)检查,结果显示胰腺体部/颈部近端有一个大小为13×9mm的低回声均匀肿块病变。通过EUS进行了细针穿刺活检(FNA)。组织病理学显示为高分化神经内分泌肿瘤,符合1级胰岛素瘤(T1N0M0)。患者接受了远端胰腺切除术和脾切除术。对于临床和生化高度怀疑胰岛素瘤但非侵入性成像为阴性的病例,应采用侵入性方法来定位罪魁祸首病变。