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

Neuroendocrine neoplasms (NENs) are prevalent neuroendocrine differentiated epithelial tumors, originating from dispersed cells in the endocrine system, portrayed by their peptide producing ability, triggering a range of hormone related syndromes (1). Numerous scholars narrated gastroenteropancreatic NENs (GEP-NENs) related management ranging from: epidemiology of, to diagnosis and pathology in Caucasian patients (American, European) (2-4). Asian NEN cases are infrequent with scarce data reported regarding Asian patients especially in China. The Surveillance, Epidemiology and End Results (SEER) database in the US concurs 61% of NENs to be GEP-NENs, ranging in predominance from the rectum (17.7%), small intestine (17.3%), colon (10.1%), pancreas (7.0%), stomach (6.0%), and appendix (3.1%) (2). Internationally, gastroesophageal junction (GEJ) NENs are infrequently reported. In China, a 10-year multi-center (Level 3 hospitals) retrospective study from 2001–2010 assembled patient information diagnosed with GEP-NENs, sharing common occurrence in Chinese patients to be in the pancreas (31.5%) and rectum (29.6%). Neither of these studies reported NENs of GEJ (5). WHO distributed an updated classification of NENs in 2010, revising NENs definition to include all tumors involving neuroendocrine differentiation (6). Digestive neuroendocrine carcinoma (NEC) as well as mixed adeno-NEC (MANEC) is extremely rare and highly malignant neoplasm which is classified as classes 3 and 4. NEC/MANEC are a very aggressive type of cancer, especially making it a diagnostic challenge for clinicians when the tumor located in GEJ (7). This present study rationale was to implement an in-hospital (PUMCH) analysis of GEJ-NEC/MANEC. With intention to share new evidence regarding progression and management of such rare tumors