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首页> 外文期刊>International journal of surgical pathology >Neuroendocrine tumor of the pancreas in a patient with tuberous sclerosis: A case report and review of the literature
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Neuroendocrine tumor of the pancreas in a patient with tuberous sclerosis: A case report and review of the literature

机译：结节性硬化症患者胰腺神经内分泌肿瘤：一例报道并文献复习

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摘要

A rare case of pancreatic neuroendocrine neoplasm in a patient with tuberous sclerosis complex is described. The patient was a 31-year-old man who had multiple congenital subependymal nodules, bilateral cortical tubers, and seizures of difficult control. A 2.3 cm × 2 cm well-delimitated solid tumor in the tail of the pancreas was discovered during a monitoring abdominal computed tomography. A distal pancreatectomy was performed. Histologically, the tumor was formed by uniform cells with moderated cytoplasm arranged in a combined trabecular and nested pattern. The nuclear features were bland, and mitosis was infrequent. There was no vascular invasion. Immunoreactivity for cytokeratine AE1/AE3, chromogranin A, and synaptophysin confirmed the neuroendocrine nature of this neoplasia. Pancreatic hormones were negatives. One of the 5 lymph nodes isolated from the peripancreatic adipose tissue was positive for metastases. Small series and case reports have documented that in tuberous sclerosis many endocrine system alterations might occur, affecting the function of the pituitary, parathyroid, and other neuroendocrine tissue, including islet cells of the pancreas. However, the true association of these pathological conditions remains uncertain. As far as we know, there are 10 cases reported of pancreatic neuroendocrine tumors in a setting of tuberous sclerosis complex, in which 2 cases resulted in malignant, nonfunctioning pancreatic neuroendocrine tumors.
机译：描述了结节性硬化症患者胰腺神经内分泌肿瘤的罕见病例。该患者是一名31岁的男子，患有多个先天性室管膜下结节，双侧皮质块茎和癫痫发作，难以控制。在监测腹部计算机断层扫描期间，在胰腺尾部发现了一个2.3 cm×2 cm界限清楚的实体瘤。进行了远端胰腺切除术。从组织学上讲，肿瘤是由均匀的细胞质形成的，其中的细胞质呈小梁和巢状结合排列。核特征平淡，有丝分裂很少见。没有血管侵犯。对细胞角蛋白AE1 / AE3，嗜铬粒蛋白A和突触素的免疫反应证实了这种肿瘤的神经内分泌性质。胰腺激素为阴性。从胰周脂肪组织分离的5个淋巴结之一转移阳性。小系列研究和病例报告表明，在结节性硬化症中，可能发生许多内分泌系统改变，从而影响垂体，甲状旁腺和其他神经内分泌组织的功能，包括胰腺的胰岛细胞。然而，这些病理状况的真正关联仍不确定。据我们所知，在结节性硬化复合物中，有10例报告了胰腺神经内分泌肿瘤，其中2例导致了恶性，无功能的胰腺神经内分泌肿瘤。

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