Ibrahim Mohd Elmugtaba, Abadeer Kerolos, Zhai Qihui Jim, Nassar Aziza
Division of Neurology, Massey Cancer Center, VCU Medical Center, Richmond, VA, USA.
Division of Nephrology and Hypertension, Mayo Clinic, Jacksonville, FL, USA.
Case Rep Pathol. 2017;2017:7931975. doi: 10.1155/2017/7931975. Epub 2017 Feb 20.
We describe a primary hepatic neuroendocrine tumor of a 57-year-old Thai woman who presented in 2004 with a suspicious mass in the left hepatic lobe. She underwent left hepatectomy for the 10.5-cm mass, called , . The tumor recurred in 2007, then called (PHNET), and the patient underwent liver transplant. Because of similarity between the neuroendocrine tumor and a thyroid tumor-specifically, follicular-like characteristics-immunohistochemical stains for thyroglobulin, TTF1, and calcitonin were performed. However, all were negative. All imaging studies revealed no evidence of a primary lesion other than the liver mass. In 2008, the patient's liver transplant failed because of ischemic cholangiopathy, and she underwent a second liver transplant. Seven years later, in 2015, she presented with metastatic neuroendocrine tumor of intermediate grade to the lung, consistent with metastatic PHNET. She underwent left upper-lobe wedge resection to remove the tumor. The patient is alive with no evidence of disease at 13 years after initial diagnosis. This rare variant of PHNET had thyroid-like morphologic characteristics but there is no evidence of primary thyroid tumor or thyroid markers in the primary and recurrent hepatic tumors and lung metastasis.
我们描述了一名57岁泰国女性的原发性肝神经内分泌肿瘤，该患者于2004年因左肝叶可疑肿块就诊。她因一个10.5厘米的肿块接受了左肝切除术，该肿块称为 ， 。肿瘤于2007年复发，当时称为 （PHNET），患者接受了肝移植。由于神经内分泌肿瘤与甲状腺肿瘤存在相似性——具体而言，具有滤泡样特征——因此对甲状腺球蛋白、甲状腺转录因子1（TTF1）和降钙素进行了免疫组化染色。然而，所有染色结果均为阴性。所有影像学检查均未发现除肝脏肿块外的原发性病变证据。2008年，患者因缺血性胆管病导致肝移植失败，随后接受了第二次肝移植。七年后，即2015年，她出现了中度转移性神经内分泌肿瘤，转移至肺部，符合转移性PHNET。她接受了左上叶楔形切除术以切除肿瘤。在初次诊断13年后，患者存活且无疾病证据。这种罕见的PHNET变异体具有甲状腺样形态特征，但在原发性和复发性肝肿瘤及肺转移灶中均无原发性甲状腺肿瘤或甲状腺标志物的证据。
