Snaith Jennifer R, McLeod Duncan, Richardson Arthur, Chipps David
Department of Diabetes and Endocrinology, Westmead Hospital, Westmead, New South Wales, Australia.
Department of Diabetes and Endocrinology, St Vincent's Hospital, Darlinghurst, New South Wales, Australia.
Endocrinol Diabetes Metab Case Rep. 2020 Dec 24;2020. doi: 10.1530/EDM-20-0091.
Insulinomatosis is a rare cause of hyperinsulinaemic hypoglycaemia. The ideal management approach is not known. A 40-year-old woman with recurrent symptomatic hyperinsulinaemic hypoglycaemia was diagnosed with an insulinoma. A benign 12 mm pancreatic head insulinoma was resected but hypoglycaemia recurred 7 years later. A benign 10 mm pancreatic head insulinoma was then resected but hypoglycaemia recurred within 2 months. Octreotide injections were trialled but exacerbated hypoglycaemia. After a 2-year interval, she underwent total pancreatectomy. A benign 28 mm pancreatic head insulinoma was found alongside insulin-expressing monohormonal endocrine cell clusters (IMECCs) and islet cell hyperplasia, consistent with a diagnosis of insulinomatosis. Hypoglycaemia recurred within 6 weeks. There was no identifiable lesion on MRI pancreas, Ga-68 PET or FDG PET. Diazoxide and everolimus were not tolerated. MEN-1 testing was negative. Insulinomatosis should be suspected in insulinomas with early recurrence or multifocality. De novo lesions can arise throughout the pancreas. Extensive surgery will assist diagnosis but may not provide cure.
Insulinomas are usually benign and managed surgically. Insulinomatosis is characterised by multifocal benign insulinomas with a tendency to recur early. It is rare. Multifocal or recurrent insulinomas should raise suspicion of MEN-1 syndrome, or insulinomatosis. Insulinomatosis is distinguished histologically by insulin-expressing monohormonal endocrine cell clusters (IMECCs) and tumour staining only for insulin, whereas MEN-1 associated insulinomas stain for multiple hormones. The ideal treatment strategy is unknown. Total pancreatectomy may not offer cure.
胰岛细胞瘤病是高胰岛素血症性低血糖症的罕见病因。目前尚不清楚理想的治疗方法。一名40岁复发性症状性高胰岛素血症性低血糖症女性被诊断为胰岛素瘤。切除了一个12毫米的良性胰头胰岛素瘤，但7年后低血糖症复发。随后又切除了一个10毫米的良性胰头胰岛素瘤，但2个月内低血糖症再次复发。试用了奥曲肽注射，但低血糖症加重。间隔2年后，她接受了全胰切除术。发现一个28毫米的良性胰头胰岛素瘤，同时伴有表达胰岛素的单激素内分泌细胞簇（IMECCs）和胰岛细胞增生，符合胰岛细胞瘤病的诊断。6周内低血糖症复发。胰腺MRI、Ga-68 PET或FDG PET均未发现可识别的病变。二氮嗪和依维莫司不耐受。MEN-1检测为阴性。对于早期复发或多灶性胰岛素瘤应怀疑胰岛细胞瘤病。胰腺各处均可出现新生病变。广泛手术有助于诊断，但可能无法治愈。
胰岛素瘤通常为良性，通过手术治疗。胰岛细胞瘤病的特征是多灶性良性胰岛素瘤，易于早期复发。该病罕见。多灶性或复发性胰岛素瘤应怀疑MEN-1综合征或胰岛细胞瘤病。胰岛细胞瘤病在组织学上的特征是表达胰岛素的单激素内分泌细胞簇（IMECCs）和仅胰岛素染色的肿瘤，而MEN-1相关胰岛素瘤为多种激素染色。理想的治疗策略尚不清楚。全胰切除术可能无法治愈。
