Brathwaite Shayna, Rock Jonathan, Yearsley Martha M, Bekaii-Saab Tanios, Wei Lai, Frankel Wendy L, Hays John, Wu Christina, Abdel-Misih Sherif
Department of General Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Ann Surg Oncol. 2016 Jul;23(7):2281-6. doi: 10.1245/s10434-016-5179-2. Epub 2016 Mar 10.
Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare pathologic diagnosis recently defined by the World Health Organization in 2010. Due to poor understanding of MANEC as a clinical entity, there is significant variation in the management of these patients. The purpose of our study was to characterize MANEC to develop evidence-based treatment strategies.
The Ohio State University patient database was queried for the diagnosis of MANEC and 46 patients were identified. For comparison, the database also was queried for goblet cell carcinoid (GCC) of the appendix, signet ring cell carcinoma, and carcinoid/neuroendocrine tumor of the appendix. Charts were then retrospectively reviewed for clinicopathologic characteristics, patient treatment, and survival data.
The mean age of diagnosis of MANEC was 54 years. Eighty-seven percent of MANEC arose from the appendix, with 28 % of patients undergoing appendectomy and 35 % undergoing right hemicolectomy as their index operation. Immunohistochemical staining was positive for chromogranin (82 %), synaptophysin (97 %), and CD56 (67 %). Sixty-seven percent of patients presented with stage IV disease and 41 % had nodal metastases. Overall survival was 4.1 years, which was statistically significantly different (p ≤ 0.05) compared with carcinoid tumors (13.4 years), GCC (15.4 years), and signet ring carcinoma (2.2 years).
MANEC is a more aggressive clinical entity than both GCC of the appendix and carcinoid/neuroendocrine tumors of the appendix. Based on these findings, we recommend patients with MANEC tumors undergo aggressive multidisciplinary cancer management and close surveillance.
混合性腺神经内分泌癌（MANEC）是世界卫生组织于2010年新定义的一种罕见病理诊断。由于对MANEC作为一种临床实体的认识不足，这些患者的治疗方式存在显著差异。我们研究的目的是对MANEC进行特征描述，以制定基于证据的治疗策略。
查询俄亥俄州立大学患者数据库以获取MANEC诊断信息，共识别出46例患者。为作比较，该数据库还查询了阑尾杯状细胞类癌（GCC）、印戒细胞癌以及阑尾类癌/神经内分泌肿瘤。然后回顾性分析病历以获取临床病理特征、患者治疗及生存数据。
MANEC的平均诊断年龄为54岁。87%的MANEC起源于阑尾，28%的患者首次手术为阑尾切除术，35%为右半结肠切除术。免疫组化染色嗜铬粒蛋白阳性率为82%，突触素为97%，CD56为67%。67%的患者诊断时为IV期疾病，41%有淋巴结转移。总体生存期为4.1年，与类癌肿瘤（13.4年）、GCC（15.4年）和印戒细胞癌（2.2年）相比，差异有统计学意义（p≤0.05）。
MANEC是一种比阑尾GCC和阑尾类癌/神经内分泌肿瘤更具侵袭性的临床实体。基于这些发现，我们建议MANEC肿瘤患者接受积极的多学科癌症管理及密切监测。
