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持续性慢性皮肤病变:一例胰高血糖素瘤延迟诊断病例

Unremitting chronic skin lesions: a case of delayed diagnosis of glucagonoma.

作者信息

Kawsar Hameem I, Habib Alma, Saeed Azhar, Saeed Anwaar

机构信息

Division of Medical Oncology, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS, USA.

出版信息

J Community Hosp Intern Med Perspect. 2019 Nov 1;9(5):425-429. doi: 10.1080/20009666.2019.1671574. eCollection 2019.

Abstract

A 54-year-old Caucasian male with history of hypertension, hyperlipidemia, insulin-dependent diabetes mellitus, and chronic skin rash of 4 years presented to the emergency department with worsening rash and weight loss. Physical examination revealed diffuse erythematous rash, skin ulceration, bullae with associated paresthesia in the lower extremities, trunk, bilateral upper extremities, and palms and soles. A computed tomography (CT) scan with contrast showed a large, heterogenously enhancing pancreatic mass measuring 9.4 × 3.8 cm with surrounding low-attenuation soft tissue thickening. Blood tests showed hemoglobin A1C of 10.0%. Glucagon level was elevated to 2,178 (normal < 80 pg/dl). Endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) from the pancreatic mass was suggestive of pancreatic endocrine tumor. The tumor cells were positive for synaptophysin, chromogranin, CD56, and pan-cytokeratin with focal positivity for glucagon, suggestive of glucagonoma. The patient underwent distal pancreatectomy along with splenectomy and cholecystectomy. The glucagon level normalized to 25 pg/dl within a week of tumor resection, and during his 6-week outpatient follow up, skin rash had completely resolved.

摘要

一名54岁的高加索男性,有高血压、高脂血症、胰岛素依赖型糖尿病病史,慢性皮疹4年,因皮疹加重和体重减轻就诊于急诊科。体格检查发现弥漫性红斑疹、皮肤溃疡、大疱,下肢、躯干、双侧上肢及手掌和足底伴有感觉异常。增强计算机断层扫描(CT)显示胰腺有一个大的、不均匀强化的肿块,大小为9.4×3.8 cm,周围有低密度软组织增厚。血液检查显示糖化血红蛋白A1C为10.0%。胰高血糖素水平升高至2178(正常<80 pg/dl)。胰腺肿块的内镜超声(EUS)引导下细针穿刺活检(FNA)提示胰腺内分泌肿瘤。肿瘤细胞突触素、嗜铬粒蛋白、CD56和全细胞角蛋白呈阳性,胰高血糖素呈局灶性阳性,提示为胰高血糖素瘤。患者接受了胰体尾切除术、脾切除术和胆囊切除术。肿瘤切除术后一周内,胰高血糖素水平恢复正常至25 pg/dl,在6周的门诊随访期间,皮疹完全消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0e/6830193/b928206519a3/ZJCH_A_1671574_F0001_OC.jpg

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