Article Abstract

Characteristics and treatment of patients with neuroendocrine carcinoma of the gastroesophageal junction: an analysis of 13 cases

Authors: Yingzhi Qin, Chao Guo, Xiaoyan Chang, Yeye Chen, Lei Liu, Jieshi Zhang, Cheng Huang, Zhijun Han, Dongjie Ma, Hongsheng Liu


Background: Digestive neuroendocrine carcinoma (NEC) as well as mixed adeno-NEC (MANEC) is rare and highly malignant neoplasm which is classified as classes 3 and 4 neuroendocrine neoplasm (NEN) respectively according to the 2010 WHO classification of digestive NEN. The digestive NENs are found more in the rectum or appendix rather than in the stomach. Most gastric NENs belong to well differentiated class 1 or 2 neuroendocrine tumors (NETs, grade 1 or 2 respectively), while gastric NEC or MANEC are even rarer. Carcinoma of proximal stomach, i.e., carcinoma of gastroesophageal junction (GEJ) is controversial in epidemiology, clinicopathology, mechanisms of tumorigenesis, histology, clinical presentations, and staging, mostly between Western populations and Chinese. This article is to investigate the clinical characteristics, pathological classification and treatment of GEJ NEC.
Methods: Clinicopathological data of 13 GEJ-NEC/MANEC patients at Peking Union Medical College Hospital (PUMCH) from January 2006 to January 2017 were reviewed and analyzed retrospectively.
Results: Of 13 patients with pathologically confirmed GEJ-NEC/MANEC who underwent surgical resection, there were 10 cases (76.9%) of male and 3 cases (23.1%) of female. Patient age varied from 46 to 80 (median 64) years. Only 1 case (7.7%) was pathologically diagnosed as GEJ-NEC by pre-operational gastroscopy biopsy and 5 cases (38.5) were diagnosed as poorly differentiated adenocarcinoma. Twelve patients (92.3%) received proximal subtotal gastrectomy and lower esophagectomy. Another 1 patient (7.7%) received total gastrectomy and lower esophagectomy. Post operational histopathology revealed NEC in 8 cases (61.5%) and MANEC in 5 cases (38.5%).
Conclusions: GEJ-NEC/MANEC is difficult to diagnosis before operation and surgery is still the primary treatment for resectable GEJ-NEC/MANEC.