OBJECTIVETo investigate clinicopathological features and prognostic factors of appendiceal neuroendocrine neoplasms(a-NEN).

METHODSClinical data of 20 patients diagnosed with a-NEN at Zhongshan Hospital of Fudan University between January 2000 and December 2016 were retrospectively analyzed. Pathological diagnosis was based on the WHO classification criteria of digestive system tumors (2010 edition). Based on the mitotic count and Ki-67 index, a-NENs were divided into grade 1 neuroendocrine tumor (NET G1), grade 2(G2) NET G2) and grade 3 (neuroendocrine carcinoma, NEC). Some special types of a-NEN (e.g. goblet cell carcinoid) and mixed adenoneuroendocrine neoplasms were classified as mixed adenoneuroendocrine carcinoma (MANEC). Follow-up was conducted by telephone or return visits. Univariate analysis was carried out using the Kaplan-Meier method, and the log-rank test was used to draw survival curves.

RESULTSOf 20 patients, 14 were male and 6 were female with median age of 54 years. Seventeen cases presented acute right lower quadrant abdominal pain, 1 chronic right lower quadrant abdominal pain, 1 persistent abdominal discomfort with outburst whole abdominal pain and 1 was found during body check without symptoms. Twenty cases comprised 8 G1 patients, 4 G2 patients, 3 G3 patients, and 5 MANEC patients. When diagnosed, there was 1 patient with liver metastasis, 1 patient with abdominal and pelvic metastases, and 2 patients with postoperative pathological findings of lymph node metastasis. Six patients underwent appendectomy, 12 underwent right hemicolectomy, 1 underwent right hemicolectomy plus small intestine resection, and 1 underwent partial hepatectomy plus right hemicolectomy. The follow-up time was 7-187 months(average, 36 months). The total 1- and 3-year survival rates were 94.7% and 60.2%, respectively. Univariate analysis showed that age >50 years (χ=7.036, P=0.008), pathology grade as MANEC (χ=5.297, P=0.021), and metastasis (χ=6.558, P=0.010) indicated lower 5-year survival rate.

CONCLUSIONSMost a-NEN patients have no typical symptoms, and the main complaint at consultation is acute right lower quadrant abdominal pain. Prognosis is poor for patients with age >50 years, MANEC pathology grade and metastasis.

Appendiceal Neoplasms ; complications ; diagnosis ; surgery ; Carcinoma, Neuroendocrine ; complications ; diagnosis ; therapy ; Female ; Gastrointestinal Neoplasms ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies

Local recurrence after endoscopic piecemeal mucosal resection (EPMR) for colorectal tumors is a crucial issue. However, such recurrence is usually detected within one year and cured with additional endoscopic treatment, which makes EPMR acceptable. Herein, we report a rare case of repeatedly recurrent colon cancer involving the appendiceal orifice after EPMR, which was not cured with additional endoscopic treatments. A 67-year-old man was referred to us for endoscopic treatment of a 25 mm cecal tumor spreading to the appendiceal orifice in May 2002. The tumor was resected with EPMR, showing well differentiated intramucosal adenocarcinoma with a positive lateral cut margin of tubular adenoma. Endoscopic surveillance was conducted and the first local recurrence was detected in August 2006. Although we resected it endoscopically, the second local recurrence was found in September 2007 and we removed it with endoscopic resection again. However, the third local recurrence was detected in March 2008. Although endoscopic resection was performed also for the third recurrence, curative resection was not achieved. In February 2009, laparoscopic assisted colectomy was performed and histopathological examination showed well differentiated adenocarcinoma with deep submucosal invasion. This case is important in considering indication for endoscopic resection in colorectal tumors involving the appendiceal orifice.
Adenocarcinoma/*diagnosis/pathology/surgery ; Aged ; Appendiceal Neoplasms/complications ; Colectomy ; Colonic Neoplasms/*diagnosis/pathology/surgery ; Colonoscopy ; Humans ; Intestinal Mucosa/pathology ; Male ; Neoplasm Recurrence, Local ; Recurrence

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Appendectomy ; Appendiceal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Appendicitis ; etiology ; surgery ; Diagnosis, Differential ; Female ; Granular Cell Tumor ; complications ; metabolism ; pathology ; surgery ; Humans ; Paraganglioma ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism