Mixed Adenoneuroendocrine Carcinoma Research Articles

Amrubicin in patients with platinum-refractory metastatic neuroendocrine carcinoma and mixed adenoneuroendocrine carcinoma of the gastrointestinal tract

Although the same treatment strategy as used for small cell lung cancer, including second-line chemotherapy, is generally applied to metastatic neuroendocrine carcinoma (NEC) and mixed adenoneuroendocrine carcinoma (MANEC) of the gastrointestinal tract (GIT; GIT-NEC/MANEC), the efficacy of amrubicin (AMR) for GIT-NEC/MANEC is not well known. We retrospectively analyzed platinum-refractory GIT-NEC/MANEC patients who received AMR between February 2004 and July 2012 at the National Cancer Center Hospital. The AMR dose administered was 30-45 mg/m on days 1-3 every 3-4 weeks. The overall response rate according to Response Evaluation Criteria in Solid Tumors guidelines, version 1.0, progression-free survival, overall survival, and adverse events by National Cancer Institute-Common Terminology Criteria for Adverse Events guidelines, version 4.0 were evaluated. Nineteen patients received AMR. The response rate for 16 assessable patients was 18.8% (95% confidence interval, 4.1-45.7), the median progression-free survival was 3.8 months (2.3-5.3), and the median overall survival was 7.7 months (7.1-8.2). Grade 3/4 neutropenia occurred in 52.6% of patients and febrile neutropenia occurred in 10.5%. Other nonhematological toxicities were mild and treatment-related deaths were not observed. AMR may have a modest effect, with tolerable toxicities, on patients with platinum-refractory GIT-NEC/MANEC. Further prospective evaluations are warranted.

Neuroendocrine Tumors of the Large Intestine: Clinicopathological Features and Predictive Factors of Lymph Node Metastasis.

A new histological classification of neuroendocrine tumors (NETs) was established in WHO 2010. ENET and NCCN proposed treatment algorithms for colorectal NET. Retrospective study of NET of the large intestine (colorectal and appendiceal NET) was performed among institutions allied with the Japanese Society for Cancer of the Colon and Rectum, and 760 neuroendocrine tumors from 2001 to 2011 were re-assessed using WHO 2010 criteria to elucidate the clinicopathological features of NET in the large intestine. Next, the clinicopathological relationship with lymph node metastasis was analyzed to predict lymph node metastasis in locally resected rectal NET. The primary site was rectum in 718/760 cases (94.5%), colon in 30/760 cases (3.9%), and appendix in 12/760 cases (1.6%). Patients were predominantly men (61.6%) with a mean age of 58.7 years. Tumor size was <10 mm in 65.4% of cases. Proportions of NET G1, G2, G3, and mixed adeno-neuroendocrine carcinoma (MANEC) were 88.4, 6.3, 3.9, and 1.3%, respectively. Of the 760 tumors, 468 were locally resected, and 292 were surgically resected with lymph node dissection. Rectal NET showed a higher proportion of NET G1, and colonic and appendiceal NET was more commonly G3 and MANEC. Of the 292 surgically resected cases, 233 NET G1 and G2 located in the rectum were used for the prediction of lymph node metastasis. Lymphatic and blood vessel invasion were independent predictive factors of lymph node metastasis. NET G2 cases showed more frequent lymph node metastasis than that seen in NET G1 cases, but this was not an independent predictor of lymph node metastasis. Of the 98 surgically resected cases <10 mm in size, we found 9 cases with lymph node metastasis (9.2%). All cases were NET G1, and eight of the nine cases were positive either for lymphatic invasion or blood vessel invasion. Using the WHO classification, we found NET in the large intestine showed a tumor-site-dependent variety of histological and clinicopathological features. Risk of lymph node metastasis in rectal NET was confirmed even in lesions smaller than 10 mm. Concordant assessment of vascular invasion will be required to estimate lymph node metastasis in small lesions.

Appendiceal Mixed Adeno-Neuroendocrine Carcinoma: A Population-Based Study of the Surveillance, Epidemiology, and End Results Registry.

Mixed adeno-neuroendocrine carcinoma (MANEC) is a rare pathological diagnosis recently defined by the World Health Organization (WHO) in 2010. Prior to the definition by the WHO, tumors with both adenocarcinoma and neuroendocrine components were given multiple pathological designations making it difficult to characterize the disease. The aim of our study is to better characterize MANEC to better understand its natural history to influence patient care and positively impact outcomes. The surveillance, epidemiology, and end results program database was queried for all patients aged 18 years or older between 1973 and 2012 who had the diagnosis composite carcinoid (n = 249) of the appendix. Composite carcinoid tumors refer to tumors that have both adenocarcinoma and carcinoid tumor components present, consistent with that pathological diagnosis MANEC. For comparison, the database was also queried for carcinoid tumor of the appendix (n = 950), signet ring cell carcinoma of the appendix (n = 579), and goblet cell carcinoid (GCC) tumors of the appendix (n = 944). The data were retrospectively reviewed, and clinicopathological characteristics, treatment regimens, and survival data were obtained. The median age of diagnosis of MANEC tumors was 58 years of age. Eighty percent of patients were White, and 49% were female. Fifty-four percent of patients underwent hemicolectomy and 31% had partial/subtotal colectomy as their surgical management. Median overall survival for MANEC was 6.5 years (95% CI 4.5-9.7), which was statistically significantly shorter (p < 0.0001) in comparison to 13.8 years (95% CI 12.1-16.5) for GCC, 2.1 years (95% CI 1.8-2.3) for signet ring cell carcinoma, and 39.4 years (95% CI 37.1-NA) for carcinoid tumors. MANEC is a more aggressive clinical entity than both GCC of the appendix and carcinoid tumors of the appendix. Based on these findings, patients with MANEC tumors should undergo aggressive multidisciplinary cancer management.

Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs): Unifying the Concept of a Heterogeneous Group of Neoplasms.

The wide application of immunohistochemistry to the study of tumors has led to the recognition that epithelial neoplasms composed of both a neuroendocrine and nonneuroendocrine component are not as rare as traditionally believed. It has been recommended that mixed neuroendocrine-nonneuroendocrine epithelial neoplasms are classified as only those in which either component represents at least 30% of the lesion but this cutoff has not been universally accepted. Moreover, since their pathogenetic and clinical features are still unclear, mixed neuroendocrine-nonneuroendocrine epithelial neoplasms are not included as a separate clinicopathological entity in most WHO classifications, although they have been observed in virtually all organs. In the WHO classification of digestive tumors, mixed neuroendocrine-nonneuroendocrine neoplasm is considered a specific type and is defined as mixed adenoneuroendocrine carcinoma, a definition that has not been accepted for other organs. In fact, this term does not adequately convey the morphological and biological heterogeneity of digestive mixed neoplasms and has created some misunderstanding among both pathologists and clinicians. In the present study, we have reviewed the literature on mixed neuroendocrine-nonneuroendocrine epithelial neoplasms reported in the pituitary, thyroid, nasal cavity, larynx, lung, digestive system, urinary system, male and female genital organs, and skin to give the reader an overview of the most important clinicopathological features and morphological criteria for diagnosing each entity. We also propose to use the term "mixed neuroendocrine-nonneuroendocrine neoplasm (MiNEN)" to define and to unify the concept of this heterogeneous group of neoplasms, which show different characteristics mainly depending on the type of neuroendocrine and nonneuroendocrine components.

Recent Updates on Neuroendocrine Tumors From the Gastrointestinal and Pancreatobiliary Tracts.

-Gastrointestinal (GI) and pancreatobiliary tracts contain a variety of neuroendocrine cells that constitute a diffuse endocrine system. Neuroendocrine tumors (NETs) from these organs are heterogeneous tumors with diverse clinical behaviors. Recent improvements in the understanding of NETs from the GI and pancreatobiliary tracts have led to more-refined definitions of the clinicopathologic characteristics of these tumors. Under the 2010 World Health Organization classification scheme, NETs are classified as grade (G) 1 NETs, G2 NETs, neuroendocrine carcinomas, and mixed adenoneuroendocrine carcinomas. Histologic grades are dependent on mitotic counts and the Ki-67 labeling index. Several new issues arose after implementation of the 2010 World Health Organization classification scheme, such as issues with well-differentiated NETs with G3 Ki-67 labeling index and the evaluation of mitotic counts and Ki-67 labeling. Hereditary syndromes, including multiple endocrine neoplasia type 1 syndrome, von Hippel-Lindau syndrome, neurofibromatosis 1, and tuberous sclerosis, are related to NETs of the GI and pancreatobiliary tracts. Several prognostic markers of GI and pancreatobiliary tract NETs have been introduced, but many of them require further validation. -To understand clinicopathologic characteristics of NETs from the GI and pancreatobiliary tracts. -PubMed (US National Library of Medicine) reports were reviewed. -In this review, we briefly summarize recent developments and issues related to NETs of the GI and pancreatobiliary tracts.

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report.

Mixed adenoneuroendocrine carcinomas (MANECs) are rare biphasic tumour types, which are morphologically recognisable as both gland-forming and neuroendocrine neoplasms. Within the gastrointestinal tract, MANECs occur predominantly in the stomach or colorectum. The present study described a case of a MANEC originating from the ampullary region. The patient presented with widespread metastatic disease. Biopsy samples obtained from the ampullary primary tumour disclosed a complex lesion with adenocarcinoma and neuroendocrine small cell carcinoma components, positive for the intestinal transcription factor caudal type homeobox-2 and for neuroendocrine markers, including chromogranin A, synaptophysin, cluster of differentiation 56/neural cell adhesion molecule. By contrast, biopsy samples obtained from metastatic tissue revealed pure neuroendocrine carcinoma. As exemplified by this true mixed tumour, tumour heterogeneity evolves as the major challenge in oncology today, with potentially severe implications for the choice of chemotherapy. The assessment of metastatic sites may render valuable diagnostic information that is crucial for clinical decision-making and patient management.

Primary mixed adenoneuroendocrine carcinoma of gall bladder

Introduction: Primary mixed adenoneuroendocrine carcinomas are rare in gall bladder. They account for 0.5 % of all neuroendocrine tumours. Case report: A 59 year-old female patient presented to the outpatient with complaints of pain right hypochondrium, vomiting and decreased appetite. On abdomen examination, patient had hard palpable gallbladder. Haemogram and liver function tests were normal. In view of elevated renal parameters, magnetic resonance imaging abdomen (MRI) was done. MRI showed a gall bladder lesion at the fundus measuring 6.5 × 6.1 cm, which was hyperintense on T2 and hypointense on T1 weighted imaging with infiltration of Segment V of liver. Metastatic workup was negative. Patient underwent Radical cholecystectomy with lymphadenectomy with uneventful postoperative recovery .Histopathology showed poorly differentiated mixed adenoneuroendocrine carcinoma of gall bladder, pT3N1m0, stage III B with 2/17 positive nodes. Microscopy showed large pleomorphic cells with round to pleomorphic nuclei, prominent chromocenters with moderate eosinophilic to clear cytoplasm. Immunohistochemistry was positive for CK, CMA, Synaptophysin and Chromogranin. Patient received 4 cycles of Cisplatin and Etoposide as adjuvant chemotherapy. Follow -up 8 months after surgery, showed no evidence of local recurrence or metastatic disease. Conclusion: Primary mixed adenoneuroendocrine carcinomas are rare subtype of gallbladder malignancies. Radical surgery with adjuvant chemotherapy still remains the main stay of treatment in patients without metastatic disesase.

Mixed Adeno-neuroendocrine Carcinoma: An Aggressive Clinical Entity.

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 54years. 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 4.1years, which was statistically significantly different (p≤0.05) compared with carcinoid tumors (13.4years), GCC (15.4years), and signet ring carcinoma (2.2years). 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.

Prognostic value of (18)F-fluorodeoxyglucose positron emission tomography in patients with gastric neuroendocrine carcinoma and mixed adenoneuroendocrine carcinoma.

Gastric neuroendocrine carcinomas (NEC) and mixed adenoneuroendocrine carcinoma (MANEC) are very rare, aggressive tumors of the stomach. We aimed to examine predictive role of pretreatment (18)F-FDGPET/CT-assessed metabolic parameter of primary tumors and metastases in patientswith gastric NEC and MANEC. We conducted a review of the 27 patients with histopathologically confirmed NECs (n=10) and MANEC (n=17) of thestomachat our institution between January 2005 and December 2012. All patients underwent (18)F-FDG-PET examination at diagnosis. Metabolic parameters [SUVmax, SUVmean, metabolic tumor volume (MTV) and total lesion glycolysis (TLG)] of the primary tumor and metastases on baseline PET/CT were analyzed. The median follow-up duration was 39.4months (95% CI 20.0-58.1months) and the median overall survival (OS) was 25.7months (95% CI 14.1-37.2months). All gastric lesions were well visualized (average SUVmax=12.0, range 3.0-41.8). When subjects were divided into two groups by ROC cut-off value of 210.9 and 612, patients with high TLG in primary lesion and metastases showed poorer prognosis compared to low TLG patients (P=0.09, P=0.002, respectively). In the sub-analysis of patients with metastasis (n=12), patients with high TLG in whole body tumor showed significantly shorter OS compared to those with low TLG (31.7±11.4 vs. 7.2±2.1months, P=0.006). (18)F-FDG PET/CT is useful in evaluating prognosis of advanced gastric cancer with neuroendocrine carcinoma components. Baseline MTV of primary gastric cancer with metastatic disease, and MTV, TLG of metastases may be prognostic markers in patients with gastric NEC and MANEC.

Whole exome sequencing and gene expression profiling of neuroendocrine carcinoma in the stomach.

57 Background: Neuroendocrine carcinoma (NEC) in the stomach is a rare disease with the incidence of about 0.6 % of all gastric cancer and is well known as a highly malignant tumor with poor survival. Despite the malignant phenotype of this disease, the global gene expression profiling of gastric NEC has not yet been elucidated. We have started a comprehensive molecular profiling project that analyzes genome and transcriptome of tumor obtained from cancer patients admitted to Shizuoka Cancer Center from January 2014, setting a goal to get 3,000 samples in 3 years. We had already evaluated more than 1,500 samples from various types of malignancies, including 111 samples from gastric cancer. Here, we performed deep sequencing of 409 cancer-related genes for gastric NEC patients, adding whole-exome sequencing and gene expression profiling, to identify a gene variant of gastric NEC. Methods: Surgically-resected fresh tumor samples and peripheral blood were analyzed by whole-exome sequencing (Ion Proton, Life Technologies) and gene expression profiling (DNA microarray, Agilent Technologies). A total of 111 patients with gastric cancer were evaluated until August 2015, including 6 gastric NEC (5.4 %). We compared single nucleotide variants (SNVs) and gene expression profiles between gastric NEC and gastric adenocarcinoma. Results: All the gastric NEC patients were male with median age of 69 years (59–79 years). According to classification of TNM 7th, there were two patients with stage IA, one with IIA, one with IIIB and two with IIIC. Three of them had adenocarcinoma components constituted &gt; 30% of the respective tumors, fulfilling the criteria for mixed adenoneuroendocrine carcinoma (MANEC), as defined by the WHO classification. There was no specific SNV for NEC. However, gene expression profiling identified several specific genes expressing in NEC; most of the highly expressed genes were also known to be expressed in neuroendocrine cells. Furthermore, CPLX2 and SCG3, which had been reported to be expressed in various neuroendocrine tumors, were included. Conclusions: Gastric NEC could be characterized by specific gene expression including those expressed in neuroendocrine cells and neuroendocrine tumors.

Gastric Mixed Adenoneuroendocrine Carcinoma with Squamous Differentiation: A Case Report.

The occurrence of gastric mixed adenoneuroendocrine carcinoma (MANEC) is relatively rare, although a few cells of gastric adenocarcinoma frequently show neuroendocrine differentiation [1]. We herein present one of only a few reported cases of gastric MANEC with trilineage histologic differentiation, composed of adenocarcinoma, large cell neuroendocrine carcinoma (NEC), and squamous cell carcinoma (SqCC).

Mixed Adenoneuroendocrine Carcinoma Is a Rare but Important Tumour Found in the Oesophagus.

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract that consists of a dual adenocarcinomatous and neuroendocrine differentiation, each component representing at least 30% of the tumour. We report a case of a 68-year-old man who presented with two-month history of postprandial pain and vomiting. Gastric endoscopy revealed a polypoid mass in the lower part of the oesophagus. In contrast to the majority of these tumours, this biopsy was immunohistochemically positive for chromogranin A, and synaptophysin and Ki-67 index was 50% and the tumour was diagnosed as poorly differentiated neuroendocrine carcinoma of the oesophagus. The patient underwent surgery and lower oesophagus resection was performed. Based on the histopathology and immunohistochemistry of the tumour in the oesophagogastrectomy specimen, a mixed adenoneuroendocrine carcinoma (MANEC) was diagnosed. The objective of this case report is to advocate for the focus on the MANEC diagnosis as such patients need to be referred to a centre of excellence with expertise in NET tumours, to have the correct diagnostic work-up, treatment, and secondary diagnostic procedures performed at progression, as this will have paramount influence of the choice of treatment.

Common Hepatic Duct Mixed Adenoneuroendocrine Carcinoma Masquerading as Cholangiocarcinoma.

Bile duct mixed adenoneuroendocrine carcinoma (MANEC) is a rare entity. It is defined as having mixed elements of both neuroendocrine tumors (NET) and an adenocarcinoma element, the lesser component forming at least 30% of the tumor. It is a subtype of neuroendocrine carcinoma (NEC) showing both gland-forming epithelial tumor cells and neuroendocrine cells. It is generally misdiagnosed as cholangiocarcinoma on imaging studies. The preoperative pathological workup from the endoscopic retrograde cholangiography brush cytology usually misses the NET/NEC component since it often lies deeper in the tumor. However, it is reported that it is the NEC component that defines the prognosis of the tumor; hence, it is vital to identify the NEC component. We present a rare case of common hepatic duct (CHD) MANEC that was preoperatively misdiagnosed as cholangiocarcinoma.

Mixed Adenoneuroendocrine Carcinoma Causing Colonic Intussusception.

Colonic intussusception is a rare cause of intestinal obstruction in adults and is caused by a malignant lesion in about 70% of cases. Early diagnosis and treatment are essential. We present a 64-year-old male patient with right colonic intussusception caused by a mixed adenoneuroendocrine carcinoma (MANEC), presenting as a giant pedunculated polyp (54 mm of largest diameter). The patient underwent right colectomy with primary anastomosis and adjuvant chemotherapy. The diagnosis of intussusception of the colon in adults is difficult because of its rarity and nonspecific clinical presentation. In this case, the cause was a rare histological type malignant tumor (MANEC).

A Previously Undescribed Presentation of Mixed Adenoneuroendocrine Carcinoma.

We report a case of mixed adenoneuroendocrine carcinoma (MANEC) of stomach with tubular adenoma and well-differentiated neuroendocrine tumor (WD-NET) in the primary tumor in the stomach giving rise to biphenotypic regional nodal metastases. A 35-year-old woman with abdominal pain was found to have a 1.8-cm gastric lesion, diagnosed as WD-NET (intermediate grade) on the biopsy. The resection specimen contained residual WD-NET; there was also a gastric adenoma adjacent to the NET and nodal metastasis with both adeno- and neuroendocrine components. The tumor was classified as MANEC. Of note, the entire gastric tissue was submitted and multiple deeper levels of the adenomatous lesion were examined; no adenocarcinoma was present in the primary lesion. While association of gastric adenoma with neuroendocrine neoplasm is rare, presence of biphenotypic metastasis originating from such a lesion is highly unusual and to the best of our knowledge has not been reported.

胆管原発腺内分泌細胞癌の 1 例

胆管原発腺内分泌細胞癌の 1 例

根治的切除が可能であった横行結腸MANECの1例

根治的切除が可能であった横行結腸MANECの1例

Endocrine cell carcinomas of the colon and rectum: a clinicopathological evaluation.

Endocrine cell carcinoma, according to the Japanese classification criteria for colorectal cancer, corresponds to neuroendocrine carcinoma (NEC) and mixed adenoneuroendocrine carcinoma (MANEC), as defined in the 2010 World Health Organization (WHO) classification. We retrospectively reviewed the clinical features of patients with these tumors diagnosed and treated at our institution. The clinicopathological features of endocrine cell carcinomas of the colon and rectum diagnosed by neuroendocrine markers from January 2000 to December 2012 were retrospectively evaluated in 12 patients. Surgical specimens were obtained from eight of the 12 patients. MANEC was diagnosed in six patients and NEC in one. One tumor was unclassifiable. The tumors were not resected in four patients, and all died within 3months. Of the eight patients who underwent resection, four received an R0 resection, two of whom underwent adjuvant chemotherapy and survived more than 5years. One patient who underwent an R2 resection and continuous chemotherapy survived for 53months. One patient with NEC underwent surgery and radiotherapy, and died 17months later. Most endocrine cell carcinomas of the colon and rectum reviewed were MANECs. Though their prognosis was generally poor, chemotherapy may be effective in some patients.

Neuroendocrine Carcinoma of Gall Bladder: A Series of 19 Cases with Review of Literature.

Neuroendocrine neoplasms of the gall bladder (GB) are rare tumors classified as grade 1 and 2 neuroendocrine tumor (NET), neuroendocrine carcinoma (large cell or small cell type), and mixed adeno-neuroendocrine carcinoma (MANEC). Primary neuroendocrine carcinomas (NEC) of GB are rare with grave prognosis not withstanding radical surgical treatment. Chemotherapy forms the primary management due to the advanced stage at presentation, as complete surgical resection is generally not possible. The overall median survival is 4-6 months despite aggressive management. Only 73 cases of small cell carcinoma (SCC) and 10 cases of large cell neuroendocrine carcinoma (LCNEC) have been reported in English literature till now. We present a series of 19 cases of NEC of GB with their pathological and clinical features and response to treatment. The objective of this study was to evaluate NECs of the GB for their clinical behavior, prognosis, and treatment outcome in terms of survival and to segregate NECs into further subcategories, that is 20-50 % and >50 %, based on MIB-1 index. We retrospectively searched the clinical and pathological database over 2.5 years (January 2012 to June 2014) through existing electronic hospital medical records to collect cases of NEC arising in GB. A total of 447 cases of gall bladder pathologies were analyzed, of which 19 cases were diagnosed as NEC. All relevant investigations were documented and response to treatment/therapy was evaluated clinically and radiologically. Tumor specimen was obtained by fine-needle aspiration cytology and/or biopsy and classified using the latest World Health Organisation (WHO) classification (2010) via cytopathologic, histopathologic, and immunohistochemical visage. Nineteen patients diagnosed as NEC of GB were evaluated in the study, with 16 having SCC, 2 having LCNEC, and 1 with MANEC. All patients presented in advanced clinical stage III or IV, along with distant metastasis and showed progressive disease during therapy. Ki67 index was between 20 and 50 % in 5 cases and >50 % in 8 cases. Follow-up was available in 14 cases with a median survival of 3 months, and 2 of these patients capitulated to their illness, before commencement of treatment. The remaining 5 cases were lost to follow-up. Out of these, 2 had deranged liver function test and no chemotherapy could be administered, and the other 3 refused treatment. NECs are aggressive with generally poor prognosis, characterized by insidious onset and advanced clinical stage of presentation. A radical approach to treatment with chemotherapy is the best form of palliation. Role of radiotherapy remains undefined due to paucity of data.

Retrospective analysis of the clinicopathological characteristics of gastrointestinal neuroendocrine neoplasms.

The aim of the present study was to analyze and summarize the clinicopathological characteristics and factors affecting prognosis for patients with gastrointestinal neuroendocrine neoplasms (GINENs). Retrospective analysis was conducted on the clinicopathological data of 74 patients who were diagnosed with GINEN, and immunohistochemical methods were used to detect the expression levels of relevant markers [synaptophysin (Syn), chromogranin A (CgA) and Ki-67]. Among the 74 cases with GINEN, there were 39 males and 35 females, with an average age of 56.9 years. There were 32 neoplasms in the rectum, 29 in the stomach, 6 in the colon, 2 in the small intestine and 5 in the appendix. All 74 cases underwent surgical resection. According to the World Health Organization Classification of Tumors of the Digestive System (2010), the diagnosis of the 74 cases showed 41 cases (55.4%) of neuroendocrine tumor (NET; 25 cases of G1 and 16 cases of G2), 21 cases (28.4%) of neuroendocrine carcinoma (NEC) and 12 cases (16.2%) of mixed adenoneuroendocrine carcinoma (MANEC). Additionally, 19 cases had metastasis to lymph nodes. During 10-34 months of follow-up, 15 patients had distant metastasis and 24 patients succumbed, and the accumulative survival rate in 1 or 2 years was 87.8 and 74.3%, respectively. Six factors, namely neoplasm size, depth of invasion, lymph node metastasis, distant metastasis, pathological type and the expression or lack of expression of CgA, significantly affected the survival time of patients. Definitive diagnosis of GINEN mainly relies on pathological diagnosis. GINENs with different histopathological types and grading have different clinicopathological characteristics and prognosis: NETs are mainly early lesions with a good prognosis, whereas NECs and MANECs have high malignancy and strong invasion with a worse prognosis.