Xenaki Sofia, Lasithiotakis Konstantinos, Andreou Alexandros, Aggelaki Sofia, Tzardi Maria, Daskalaki Anna, Chalkiadakis George, Chrysos Emmanuel
Department of General Surgery, University General Hospital of Heraklion, Heraklion, 71110 Crete, Greece.
Department of Medical Oncology, University General Hospital of Heraklion, Heraklion, 71110 Crete, Greece.
Case Rep Surg. 2016;2016:3240569. doi: 10.1155/2016/3240569. Epub 2016 Aug 17.
Introduction. Neuroendocrine carcinoma (NEC) of pancreas is a rare tumor with aggressive progression and poor prognosis. Its coexistence with adenocarcinoma poses significant clinical problems and has not been addressed in the literature. Methods. We describe a case of a 51-year-old male who underwent pancreatoduodenectomy due to pancreatic head tumor 1.5 × 1 × 1.4 cm. Histological examination of the specimen revealed a mixed neoplasm: (1) a well differentiated adenocarcinoma, neoplastic blasts of which are extended focally to the submucosa without invading the muscular layer, and (2) a low differentiated NEC consisting of solid clusters and pagetoid formations. All 18 lymph nodes of the specimen were free of neoplastic disease and the surgical margins of the specimen were tumor-free. No adjuvant treatment was administered and two months after the operation the patient developed liver metastasis. FNA cytology of the hepatic lesions revealed low grade carcinoma with neuroendocrine characteristics. Five lines of chemotherapy were administered: VP + CDDP, paclitaxel + ifosfamide + Mesna + CDDP, Folfox + Avastin, Folfiri + Avastin, and CAV. During his treatment he revealed PD and succumbed to his disease 13 months after the operation. Conclusion. Coexistence of NEC with adenocarcinoma of the pancreas is a very rare entity presenting significant challenges regarding its adjuvant treatment and the treatment of distant relapse.
引言。胰腺神经内分泌癌（NEC）是一种罕见的肿瘤，进展迅速且预后不良。它与腺癌共存带来了重大的临床问题，而相关文献中尚未涉及。方法。我们描述了一例51岁男性患者，因胰头肿瘤（1.5×1×1.4厘米）接受了胰十二指肠切除术。对标本进行组织学检查发现是一种混合性肿瘤：（1）高分化腺癌，其肿瘤母细胞局部扩展至黏膜下层但未侵犯肌层；（2）低分化NEC，由实性细胞团和派杰样结构组成。标本的所有18个淋巴结均无肿瘤病变，标本的手术切缘无肿瘤。未进行辅助治疗，术后两个月患者出现肝转移。肝病变的细针穿刺抽吸活检（FNA）细胞学检查显示为具有神经内分泌特征的低级别癌。给予了五种化疗方案：VP + 顺铂（CDDP）、紫杉醇 + 异环磷酰胺 + 美司钠（Mesna） + 顺铂、Folfox + 阿瓦斯汀（Avastin）、Folfiri + 阿瓦斯汀以及CAV。在治疗过程中他出现疾病进展（PD），术后13个月死于该疾病。结论。胰腺NEC与腺癌共存是一种非常罕见的情况，在辅助治疗和远处复发的治疗方面面临重大挑战。
