Dudziak Katarzyna, Rettig Ingo, Adam Patrick, Horger Marius, Neumann Hartmut P, Müssig Karsten
Abteilung für Endokrinologie, Diabetes, Nephrologie, Angiologie und Klinische Chemie, Medizinische Universitätsklinik Tübingen.
Dtsch Med Wochenschr. 2011 Oct;136(43):2196. doi: 10.1055/s-0031-1289124. Epub 2011 Oct 18.
A 48-year-old patient presented with an insufficient control of his diabetes mellitus which was known since 3 years. The antidiabetic medication comprised metformin and exenatide.
Physical examination revealed, beside elevated blood pressure, abdominal purple striae. Endocrine testing was consistent with ectopic Cushing's syndrome. Abdominal CT showed a 5 cm measuring, inhomogeneous, contrast-enhanced mass in the right suprarenal area which was positive on iodine-131 MIBG SPECT. Furthermore, urinary catecholamines were markedly increased.
DIAGNOSIS, TREATMENT AND COURSE: Diagnosis of an ACTH-producing pheochromocytoma was made and an open adrenalectomy was performed. Histology confirmed a pheochromocytoma with potential aggressive clinical behaviour according to the Pheocromocytoma of the Adrenal gland Scaled Score. 6 months after the intervention, glucose control was significantly improved with an HbA1c of 5.5%.
An ACTH-producing pheochromocytoma is a very rare cause of deterioration of glucose control. However, in presence of typical clinical findings an endocrine work-up is warranted.
一名48岁患者,其糖尿病自3年前确诊以来一直控制不佳。抗糖尿病药物包括二甲双胍和艾塞那肽。
体格检查发现,除血压升高外,腹部有紫色条纹。内分泌检查结果与异位库欣综合征相符。腹部CT显示右肾上腺区有一个5厘米大小、不均匀、增强造影的肿块,碘-131间碘苄胍单光子发射计算机断层扫描(131I-MIBG SPECT)呈阳性。此外,尿儿茶酚胺明显升高。
诊断、治疗与病程:诊断为分泌促肾上腺皮质激素(ACTH)的嗜铬细胞瘤,并进行了开放性肾上腺切除术。组织学检查根据肾上腺嗜铬细胞瘤分级评分证实为具有潜在侵袭性临床行为的嗜铬细胞瘤。干预6个月后,糖化血红蛋白(HbA1c)为5.5%,血糖控制显著改善。
分泌ACTH的嗜铬细胞瘤是血糖控制恶化的非常罕见原因。然而,在存在典型临床症状时,有必要进行内分泌检查。