医学
错构瘤
肌上皮细胞
胰腺
糖尿病
家族史
囊肿
病理
恶性肿瘤
胰腺疾病
内镜超声
放射科
胃肠病学
内科学
内分泌学
免疫组织化学
作者
Jeremy M. Sharib,Jeffrey R Ord,Rex C. Bentley,Chanjuan Shi,Dan G. Blazer
出处
期刊:Pancreas
[Ovid Technologies (Wolters Kluwer)]
日期:2022-08-01
卷期号:51 (7): 830-833
标识
DOI:10.1097/mpa.0000000000002110
摘要
Pancreatic myoepithelial hamartoma is a rare, benign solid and cystic lesion of the pancreas. We present the first case of an adult with a giant myoepithelial hamartoma extending throughout the pancreas in a patient with diabetes in 4 immediate family members. The patient is a 46-year-old man presented with recurrent acute pancreatitis. Computed tomographic imaging showed that the head and body of the pancreas were replaced by a solid-cystic mass with focal calcification. Medical history includes insulin-dependent diabetes mellitus (IDDM) diagnosed at age 30. Endoscopic ultrasound-guided fine-needle aspiration showed pancreatic acinar tissue and smooth muscle without evidence of malignancy. Total pancreatectomy was performed because of the diffuse nature of the cystic disease and preexisting IDDM. The histopathologic diagnosis was consistent with myoepithelial hamartoma. In addition, there was a family history of IDDM and hamartomatous cyst resection in the paternal grandmother. We report the first case of diffuse pancreatic myoepithelial hamartoma with near total replacement of the entire pancreatic parenchyma, and the first reported case associated with a family history of heritable IDDM. Improved knowledge of the genetics, development, and malignant potential of such rare diseases is critical to determine appropriate management for patients.
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