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神经节细胞性副神经节瘤:一种罕见病例,所有 3 种成分(神经节细胞、神经节和嗜铬细胞)均转移至肝脏和淋巴结。

Gangliocytic paraganglioma: a rare case with metastases of all 3 elements to liver and lymph nodes.

机构信息

Department of Pathology, Sunnybrook Health Sciences Centre, Toronto M4N 3M5, Canada.

出版信息

Ann Diagn Pathol. 2011 Dec;15(6):467-71. doi: 10.1016/j.anndiagpath.2010.07.009. Epub 2010 Oct 30.

Abstract

Gangliocytic paragangliomas are a rare but well-described neuroendocrine tumor with a proclivity for the duodenal area. It is usually a locally infiltrative lesion with a good prognosis. Rare cases that spread to lymph nodes have been documented. A 52-year-old female was found to have a duodenal tumor, enlarged regional lymph nodes, and multiple small nodules in the liver. The duodenal tumor was a classic triphasic gangliocytic paraganglioma. Two regional lymph nodes and one of the nodules in the liver were noted to contain all 3 elements constituting a gangliocytic paraganglioma. This is the second case of gangliocytic paraganglioma with liver spread and is also noteworthy because of the presence of all 3 elements in the metastases. The presence of lymph node and liver metastases should not result in overtreatment as such behavior is not necessarily fraught with a bad clinical outcome. It is probably best to regard such cases as having "uncertain malignant potential" and advocate careful follow-up.

摘要

神经节细胞副神经节瘤是一种罕见但特征明确的神经内分泌肿瘤,倾向于发生在十二指肠区域。它通常是一种局部浸润性病变,预后良好。已有文献记录罕见的淋巴结转移病例。一名 52 岁女性被发现患有十二指肠肿瘤、区域性淋巴结肿大和肝脏内多个小结节。十二指肠肿瘤为典型的三相神经节细胞副神经节瘤。两个区域性淋巴结和肝脏内的一个结节均含有构成神经节细胞副神经节瘤的所有 3 种成分。这是第二例肝脏转移的神经节细胞副神经节瘤,值得注意的是,转移灶中存在所有 3 种成分。淋巴结和肝转移的存在不应导致过度治疗,因为这种行为不一定会导致不良的临床结局。最好将此类病例视为“恶性潜能不确定”,并主张进行仔细的随访。

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