Beard Rachel E, Finkelstein Sydney D, Borhani Amir A, Minervini Marta I, Marsh J Wallis
Department of Surgery, Division of Hepatobiliary and Pancreatic Surgery, University of Pittsburgh Medical Center, PA, USA.
Interpace Diagnostics, Pittsburgh, PA, USA.
Int J Surg Case Rep. 2017;37:26-32. doi: 10.1016/j.ijscr.2017.05.039. Epub 2017 Jun 7.
Mixed hepatocellular and cholangiocarcinoma tumors (MHCC) are described in the literature, as are the more rare mixed adenoneuroendocrine carcinomas (MANC) of hepatobiliary origin. Only two cases of tumors with characteristics of all three histologies/phenotypes have been previously described in one Chinese study.
Herein we report clinical, microscopic and molecular features of a 25cm mixed hepatic tumor with hepatocellular, cholangiocarcinoma and neuroendocrine differentiation arising in an otherwise healthy 19-year-old North American Caucasian male without any identifiable risk factors.
The patient underwent multimodality imaging and the tumor was biopsied preoperatively, and it was initially interpreted to be hepatocellular carcinoma fibrolamellar type. A left trisegmentectomy with lymphadenectomy was performed and the tumor was definitively diagnosed based on the surgically resected specimen. Integrated microscopic and molecular features defined the differing biological aggressiveness of growth pattern components. Cases in the literature of MHCC and rare cases of MANC have largely undergone aggressive surgical resection as well, however the majority of studies on mixed hepatic tumors to date reflect Eastern patient cohorts and populations with underlying liver disease, thereby limiting extrapolation on management or outcomes in this case.
This is one of the only reports of a hepatic tumor arising from hepatocellular carcinoma, cholangiocarcinoma and neuroendocrine lineages. Increased awareness of this tumor type may optimize improve future management.
文献中描述了混合性肝细胞和胆管癌肿瘤（MHCC），以及更为罕见的肝胆源性混合性腺神经内分泌癌（MANC）。此前在一项中国研究中仅报道过两例具有所有三种组织学/表型特征的肿瘤病例。
在此，我们报告了一名19岁北美白种男性的临床、显微镜检查和分子特征，该男性身体健康，无任何可识别的危险因素，其肝脏出现了一个25厘米的混合性肿瘤，具有肝细胞、胆管癌和神经内分泌分化特征。
患者接受了多模态成像检查，并在术前对肿瘤进行了活检，最初被诊断为纤维板层型肝细胞癌。实施了左三段切除术及淋巴结清扫术，并根据手术切除标本对肿瘤进行了明确诊断。综合显微镜检查和分子特征确定了生长模式成分不同的生物学侵袭性。文献中的MHCC病例以及罕见的MANC病例大多也接受了积极的手术切除，然而，迄今为止，大多数关于混合性肝脏肿瘤的研究反映的是东方患者队列以及有潜在肝脏疾病的人群，因此限制了对此病例管理或预后的推断。
这是仅有的关于源自肝细胞癌、胆管癌和神经内分泌谱系的肝脏肿瘤的报告之一。提高对这种肿瘤类型的认识可能会优化未来的管理。
