Kotsis T, Voros D, Paphiti A, Frangou M, Mallas E
2nd Department of Surgery, Aretaeion Hospital, Medical School, University of Athens, Greece.
Dig Surg. 2000;17(6):636-640. doi: 10.1159/000051976.
Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped Schwann cells.
A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy.
The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100.
Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of benign nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.
十二指肠神经节细胞性副神经节瘤是一种特殊的神经内分泌肿瘤，常因上覆黏膜频繁溃疡，以偶然的影像学表现或胃肠道出血的形式被发现。大多数病变为带蒂的黏膜下肿物，具有独特的组织学特征，由内分泌细胞、神经节细胞和梭形施万细胞组成。
报告一例50岁女性十二指肠神经节细胞性副神经节瘤患者，表现为黑便。小肠灌肠造影显示在十二指肠第二或第三段有一个带蒂肿物。上消化道内镜检查发现一个带蒂的溃疡性肿物，随后经计算机断层扫描得以证实。通过十二指肠切开术将肿瘤简单切除。
肿瘤大小为4.5×3×1.5cm。肿瘤的免疫组化和超微结构特征将其归类为典型的十二指肠神经节细胞性副神经节瘤，神经元特异性烯醇化酶、嗜铬粒蛋白、生长抑素、胃泌素和S100呈阳性细胞反应。
术后46个月，患者情况良好，无复发迹象。大多数报道的十二指肠神经节细胞性副神经节瘤为良性。然而，4例孤立病例出现淋巴结受累这一事实表明，需要对淋巴结转移进行全面检查，以及进行全面的术后随访。
