Bilici Meliha Esra, Ustundağ Yücel
Department of Pediatric Endocrinology, Bulent Ecevit University Faculty of Medicine, Zonguldak, Türkiye.
Department of Gastroenterology, Bulent Ecevit University Faculty of Medicine, Zonguldak, Türkiye.
J Pediatr Endocrinol Metab. 2025 Aug 11;38(9):976-985. doi: 10.1515/jpem-2025-0322. Print 2025 Sep 25.
In childhood, endogenous hyperinsulinemic hypoglycemia is most commonly associated with congenital hyperinsulinism due to genetic mutations. Pancreatic neuroendocrine tumors, which have an incidence of approximately four cases per million per year, are rare in children. Detecting small lesions is challenging, and clinical suspicion is crucial for early diagnosis. This report aims to emphasize the importance of endoscopic ultrasonography (EUS) in the clinical approach to endogenous hyperinsulinism. Additionally, a focused literature review was conducted to support the diagnostic and therapeutic aspects discussed in the case.
A 16-year-9-month-old male with no prior medical history was admitted to the emergency department with excessive sweating, speech difficulties, and an inability to wake up, occurring 8 h after a post-training meal. His blood glucose was 35 mg/dL. He had experienced similar episodes previously, resolving with food intake. An extended fasting test revealed hyperinsulinemic hypoglycemia (glucose: 45 mg/dL, insulin: 15.9 µU/mL, ketone: 0.1). Imaging studies (USG, MRI, CT) failed to detect a lesion. EUS identified a 12 × 9 mm pancreatic body lesion, confirmed as a well-differentiated grade 2 neuroendocrine tumor via fine-needle aspiration biopsy. Laparoscopic enucleation verified the insulinoma diagnosis, with no metastasis or MEN-1 findings. Following surgery, the patient remained asymptomatic with no recurrence of hypoglycemia during a 2-year follow-up.
Insulinoma should be considered in cases of hypoglycemia triggered by fasting or exercise due to its intermittent insulin secretion. Given the small size of the pancreas and the anatomical differences in children, EUS is a highly sensitive diagnostic tool for detecting small pancreatic tumors, such as insulinoma.
在儿童期，内源性高胰岛素血症性低血糖最常见于由基因突变引起的先天性高胰岛素血症。胰腺神经内分泌肿瘤每年发病率约为百万分之四，在儿童中较为罕见。检测小病灶具有挑战性，临床怀疑对早期诊断至关重要。本报告旨在强调内镜超声（EUS）在内源性高胰岛素血症临床诊治中的重要性。此外，还进行了针对性的文献综述，以支持病例中讨论的诊断和治疗方面。
一名16岁9个月大、无前科病史的男性因训练后进食8小时后出现多汗、言语困难及无法唤醒而被收入急诊科。他的血糖为35mg/dL。他此前曾有过类似发作，进食后缓解。延长禁食试验显示高胰岛素血症性低血糖（血糖：45mg/dL，胰岛素：15.9µU/mL，酮体：0.1）。影像学检查（超声、磁共振成像、计算机断层扫描）未能检测到病灶。EUS发现胰腺体部有一个12×9mm的病灶，经细针穿刺活检证实为高分化2级神经内分泌肿瘤。腹腔镜摘除术证实为胰岛素瘤诊断，无转移或多发性内分泌腺瘤1型（MEN-1）表现。手术后，患者无症状，在2年随访期间低血糖未复发。
由于胰岛素瘤间歇性分泌胰岛素，对于因禁食或运动引发低血糖的病例应考虑胰岛素瘤。鉴于胰腺体积小以及儿童的解剖差异，EUS是检测小胰腺肿瘤（如胰岛素瘤）的高度敏感的诊断工具。
