Hirai Hiroyuki, Midorikawa Sanae, Suzuki Shinichi, Sasano Hironobu, Watanabe Tsuyoshi, Satoh Hiroaki
Department of Nephrology, Hypertension, Diabetology, Endocrinology, and Metabolism, Fukushima Medical University, Japan.
Intern Med. 2016;55(20):2985-2991. doi: 10.2169/internalmedicine.55.7071. Epub 2016 Oct 15.
We herein present the findings of a 42-year-old woman with either adrenal pheochromocytoma or intraadrenal paraganglioma that simultaneously secreted somatostatin, thus mimicking insulin-dependent diabetes mellitus. Pheochromocytoma was clinically diagnosed based on scintigraphy, elevated catecholamine levels, and finally a histopathological analysis of resected specimens. The patient had diabetic ketosis, requiring 40 U insulin for treatment. Following laparoscopic adrenalectomy, insulin therapy was discontinued and the urinary c-peptide levels changed from 5.5-9.0 to 81.3-87.0 μg/day. Histologically, somatostatin immunoreactivity was detected and the somatostatin levels were elevated in the serum-like fluid obtained from the tumor. Clinicians should be aware of the possible occurrence of simultaneous ectopic hormone secretion in patients with pheochromocytoma.
我们在此报告一名42岁女性的病例,其患有肾上腺嗜铬细胞瘤或肾上腺内副神经节瘤,同时分泌生长抑素,从而表现出胰岛素依赖型糖尿病的症状。嗜铬细胞瘤通过闪烁扫描、儿茶酚胺水平升高以及最终对切除标本的组织病理学分析进行临床诊断。该患者出现糖尿病酮症,需要40单位胰岛素进行治疗。腹腔镜肾上腺切除术后,胰岛素治疗停止,尿C肽水平从5.5 - 9.0微克/天变为81.3 - 87.0微克/天。组织学检查发现肿瘤获取的血清样液中存在生长抑素免疫反应性,且生长抑素水平升高。临床医生应意识到嗜铬细胞瘤患者可能会出现同时异位激素分泌的情况。