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胰腺肝样癌:一例报告并文献复习

Hepatoid Carcinoma of the Pancreas: A Case Report and Review of the Literature.

作者信息

Williams Noelle L, Palmer Joshua D, Bar-Ad Voichita, Anné Pramila Rani, Sama Ashwin R, Weinstein Jonathan C, Rufail Miguel L, Yeo Charles J, Hurwitz Mark D

机构信息

Department of Radiation Oncology, Bodine Center for Cancer Treatment, Sidney Kimmel Cancer Center and Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.

Department of Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.

出版信息

Case Rep Pancreat Cancer. 2015 Nov 1;1(1):3-6. doi: 10.1089/crpc.2015.29001.nlw. eCollection 2015.

Abstract

Hepatoid carcinoma (HC) is a rare extrahepatic malignancy that shares many morphological and serological features with hepatocellular carcinoma. HC has been reported to arise from several organs that are derived from the foregut endoderm, including the stomach, gallbladder, and pancreas. We present a case of an elderly man with hepatoid adenocarcinoma of the pancreatic head with duodenal invasion, presenting with pancreatitis and a gastrointestinal bleed. With only 23 reported cases at the time of our literature search, we discuss the presentation, histopathology, and management of such a rare disease. A 71-year-old man presented initially with abdominal pain and was treated conservatively for pancreatitis. Four months later, he presented with melena and anemia. His examination was noncontributory. Esophagogastroduodenoscopy revealed a friable ampulla of Vater, and a CT scan of the abdomen showed a 4.5 cm pancreatic head mass. Fine needle aspirate revealed an epithelioid neoplasm with hepatoid morphology. Serum α-fetoprotein was normal. Surgical resection confirmed hepatoid adenocarcinoma of the pancreas with positive lymphadenopathy and negative margins. There was no radiographical or gross evidence of distant spread. Observation and adjuvant gemcitabine were discussed as possible options. The patient elected to receive care closer to home and will continue surveillance imaging. With only 23 reported cases, pancreatic HC represents a rare entity within gastrointestinal oncology. There is no clear postoperative adjuvant standard therapy for this likely heterogeneous group of tumors. Although surgical resection is the mainstay of upfront treatment, metastatic disease to the lymph nodes or liver portends a poor prognosis and may warrant treatment such as transarterial embolization, chemotherapy, or radiotherapy.

摘要

肝样腺癌(HC)是一种罕见的肝外恶性肿瘤,与肝细胞癌具有许多形态学和血清学特征。据报道,HC可起源于几个源自前肠内胚层的器官,包括胃、胆囊和胰腺。我们报告一例老年男性,患有十二指肠侵犯的胰头肝样腺癌,表现为胰腺炎和胃肠道出血。在我们进行文献检索时,仅报道了23例病例,我们讨论了这种罕见疾病的临床表现、组织病理学和治疗方法。一名71岁男性最初表现为腹痛,因胰腺炎接受保守治疗。四个月后,他出现黑便和贫血。体格检查无异常发现。食管胃十二指肠镜检查显示十二指肠乳头易碎,腹部CT扫描显示胰头有一个4.5厘米的肿块。细针穿刺活检显示为具有肝样形态的上皮样肿瘤。血清甲胎蛋白正常。手术切除证实为胰腺肝样腺癌,伴有阳性淋巴结转移且切缘阴性。没有影像学或大体证据表明有远处转移。讨论了观察和辅助吉西他滨治疗作为可能的选择。患者选择在离家较近的地方接受治疗,并将继续进行监测成像。由于仅报道了(23)例病例,胰腺HC是胃肠肿瘤学中一种罕见的疾病实体。对于这类可能异质性的肿瘤,目前尚无明确的术后辅助标准治疗方法。虽然手术切除是初始治疗的主要手段,但出现淋巴结或肝转移预示预后不良,可能需要进行如经动脉栓塞、化疗或放疗等治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ac72/6319671/115c9f9f695f/fig-1.jpg

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