Clinicopathological features and prognosis of gastroenteropancreatic neuroendocrine neoplasms in a Chinese population: a large, retrospective single-centre study.

BACKGROUND: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are the most common type of neuroendocrine tumors, accounting for more than half of neuroendocrine neoplasms (NENs). We performed a retrospective study in our center to investigate the clinicopathological features, risk factors of metastasis, and prognosis of GEP-NENs in a Chinese population. METHODS: Four hundred forty patients with GEP-NENs treated at the First Affiliated Hospital of Zhengzhou University between January 2011 and March 2016 were analyzed retrospectively. Multivariate logistic regression was performed to identify independent risk factors for metastasis of the tumors. The Kaplan-Meier method was used for survival analysis, and log-rank tests for comparisons among groups. RESULTS: Primary sites were the stomach (24.3%), rectum (24.1%), pancreas (20.5%), esophagus (12.3%), unknown primary origin (UPO-NEN) (8.0%), duodenum (6.1%). Three hundred eighty-nine of the 440 GEP-NENs cases (88.4%) were non-functional tumors, and patients had non-specific symptoms, which could have led to delay in diagnosis and treatment. Neuroendocrine tumor, neuroendocrine carcinoma, and mixed adenoendocrine carcinoma were 56.8%, 33.2% and 3.2%, respectively, of the cases. One hundred thirty (29.5%) of the tumors were G1, 120 (27.3%) G2, and 190 (43.2%) G3. The immunohistochemical positive rate of synaptophysin was 97.7% and of chromogranin 48.7%. Logistic regression analysis revealed that the diameter and pathological classification of tumors were the most important predictors for metastasis. The median survival time was 34 months for patients with well-differentiated neuroendocrine tumors grade G3 and 11 months for poorly differentiated neuroendocrine carcinoma. The median survival time of patients with localized disease, regional disease, and distant disease was 36 months, 15 month, and 6 months, respectively. CONCLUSIONS: This study constitutes a comprehensive analysis of the clinicopathological features of GEP-NENs in a Chinese population. GEP-NENs may occur at any part of the digestive system. The diameter and pathological classification of tumor are the most important predictors for metastasis. The prognosis is poor for patients with poorly differentiated neuroendocrine cancers and distant metastases.

Upregulation of transgelin is an independent factor predictive of poor prognosis in patients with advanced pancreatic cancer.

Transgelin is a known actin-binding protein, which plays a role in regulating the functions of smooth muscle cells or fibroblasts. Recent evidence indicates that transgelin is involved in diverse human cancers, yet its role in pancreatic cancer remains unclear. We therefore evaluated the expression characteristics and function of transgelin in pancreatic cancer. Immunohistochemical analysis of benign (n = 30 patients) and malignant (n = 114 patients) pancreatic ductal cells showed significantly higher transgelin staining in malignant cells. Lymph node metastasis (P = 0.026) and diabetes (P = 0.041) were shown to significantly correlate with transgelin protein expression. Patients with high transgelin expression showed a shorter 5-year overall survival and a lower tumor-specific survival than those with low transgelin expression. Multivariate analysis revealed that transgelin was an independent factor affecting pancreatic tumor-specific survival (P = 0.025). In vitro, RNA interference-mediated transgelin knockdown resulted in inhibition of pancreatic cancer cell proliferation, migration and invasion. Depletion of transgelin expression could suppress pancreatic tumorigenicity and tumor growth in vivo, and produce enhanced cytotoxic effects of gemcitabine on pancreatic cancer cells both in vitro and in vivo. Our results indicate that transgelin plays a promoting role in tumor progression, and appears to be a novel prognostic marker for advanced pancreatic cancer.

Epithelioid inflammatory myofibroblastic sarcoma with recurrence after extensive resection: significant clinicopathologic characteristics of a rare aggressive soft tissue neoplasm.

A case of epithelioid inflammatory myofibroblastic scarcoma (EIMS) developing in an 8-year-old boy who presented with a bulky intra-abdominal occupying lesion with recurrence undergoing a radical resection was reported. Histologically, the tumor cells arranged in cords, strands or sheets of round-to-epithelioid cells with a vesicular nuclear chromatin pattern, prominent nucleoli and weakly eosinophic or basophilic cytoplasm embedded in the abundant myxoid stroma with lymphocytes infiltration. They were positive for ALK, Desmin, SMA, CD30, but negative for AE1/AE3, LCA, CD2, CD3, CD5, CD7, S-100, CD34, CD31, EMA, MyoD1, and myogenin. An elevated proliferation index was demonstrated by Ki-67 comparing the first and the second lesion. Fluorescence in situ (FISH) showed the presence of chromosomal translocation involving ALK. This case show EIMS is a rare variant of inflammatory myofibroblatic tumor with aggressive biological behavior and unfavourable prognosis. To be familiar with its significant clinicopathologic characteristics could prompt us to take it into consideration when facing the relevant dieases.