Akabane Miho, Kobayashi Yuta, Kinowaki Keiichi, Okubo Satoshi, Shindoh Junichi, Hashimoto Masaji
Division of Hepatobiliary-pancreatic Surgery, Department of Gastroenterological Surgery, Toranomon Hospital, Tokyo 105-8470, Japan.
Department of Diagnostic Pathology, Toranomon Hospital, Tokyo 105-8470, Japan.
World J Clin Cases. 2022 Mar 6;10(7):2222-2228. doi: 10.12998/wjcc.v10.i7.2222.
Primary hepatic neuroendocrine neoplasm (NEN) is a rare condition, and it is difficult to differentiate between primary and metastatic hepatic NENs. Herein, we report a case of primary hepatic NEN that initially mimicked a hemangioma but showed a gradual increase in size on long-term careful observation.
A 47-year-old woman was incidentally diagnosed with a 12-mm liver mass, suspected to be a hemangioma. Since then, regular follow-up had been carried out. Ten years later, she was referred to our institute due to the tumor (located in segment 4) having increased to 20 mm. Several imaging studies depicted no apparent extrahepatic lesion. Positron emission tomography (PET)/computed tomography exhibited significant accumulation in the mass lesion, which made us consider the possibility of malignancy. Left hepatectomy was performed. The histopathological diagnosis was neuroendocrine tumor grade 2, with somatostatin receptor 2a/5 positivity. Postoperative somatostatin receptor scintigraphy (SRS) showed no other site, leading to the diagnosis of NEN of primary hepatic origin. The gradual growth of the hepatic NEN over 10 years suggested that it was likely to be a primary liver tumor.
In this case, positivity on PET and postoperative SRS may have helped determine whether the tumor was primary or metastatic.
原发性肝神经内分泌肿瘤（NEN）是一种罕见疾病，难以区分原发性和转移性肝NEN。在此，我们报告一例原发性肝NEN病例，该病例最初疑似血管瘤，但经长期仔细观察后肿瘤大小逐渐增加。
一名47岁女性偶然被诊断出肝脏有一个12毫米的肿块，怀疑是血管瘤。此后，进行了定期随访。十年后，由于肿瘤（位于第4段）增大至20毫米，她被转诊至我院。多项影像学检查未发现明显的肝外病变。正电子发射断层扫描（PET）/计算机断层扫描显示肿块病变有明显放射性浓聚，这使我们考虑恶性肿瘤的可能性。遂行左肝切除术。组织病理学诊断为2级神经内分泌肿瘤，生长抑素受体2a/5阳性。术后生长抑素受体闪烁扫描（SRS）未发现其他部位有病变，从而诊断为原发性肝源性NEN。肝NEN在10年内逐渐生长，提示其可能为原发性肝肿瘤。
在本病例中，PET阳性及术后SRS可能有助于确定肿瘤是原发性还是转移性。
