Gastrointestinal mixed adenoneuroendocrine carcinoma: a population level analysis of epidemiological trends

Wang, Jiakun; He, Aoxiao; Feng, Qian; Hou, Ping; Wu, Junjun; Huang, Zhihao; Xiao, Zhouqing; Sun, Chi; Liao, Wenjun; Wu, Linquan

doi:10.1186/s12967-020-02293-0

“…Patients with distant MiNENs had the worst prognosis, with 5-year OS and CSM rates of 12.3 and 78.6%, respectively. One study found that metastatic gastrointestinal MiNENs had a higher incidence than localized disease (22). In our study, the proportion of patients with an advanced stage MiNENs was approximately 33% and this proportion was even higher in other studies (23,24).…”

Section: Discussionsupporting

confidence: 39%

Survival Profile and Prognostic Factors for Appendiceal Mixed Neuroendocrine Non-neuroendocrine Neoplasms: A SEER Population-Based Study

Zheng

¹

,

Li

²

,

Li

³

et al. 2020

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Introduction: Mixed neuroendocrine non-neuroendocrine neoplasm (MiNEN) is a rare form of neuroendocrine neoplasms (NENs). The purpose of this study was to investigate the characteristics and survival profile of appendiceal MiNENs, with a view of providing robust clinical features of this rare disease. Methods: Patients were selected from the Surveillance, Epidemiology, and End Results database (2004-2016). The prognosis of MiNEN (n = 315) was compared with other histological subtypes including neuroendocrine tumor (NETs) (n = 1734), neuroendocrine carcinoma (NECs) (n = 375), goblet cell carcinoid (GCC) (n = 968), signet ring cell carcinoma (n = 463), mucinous adenocarcinoma (MAC) (n = 2355), and non-mucinous adenocarcinoma (NMAC) (n = 1187) in the appendix. Age-adjusted incidence was calculated using Joinpoint regression. The Cox proportional hazards model and the Fine-Gray competing risk model were used to perform overall survival (OS) and cancer-specific mortality (CSM) analyses, respectively. Results: The age-adjusted incidence of MiNENs increased from 0.01/100,000 personyears in 2004 to 0.07/100,000 person-years in 2016. The 3-, 5-, and 10-year OS rates for MiNENs were 69.5, 57.4, and 43.7%, respectively, and the corresponding CSM rates were 23.1, 36.4, and 45.1%, respectively. Multivariate analysis revealed that the prognosis of MiNENs was worse than that of NETs, NECs, GCC, and MAC but better than that of NMAC and signet ring cell carcinoma. Tumor extension was the only independent factor influencing the prognosis of MiNENs, but tumor size, grade, and surgical approaches were not. Moreover, when compared with local excision or appendectomy, extensive surgery such as hemicolectomy or colectomy did not prolong the survival of individuals with MiNENs. Conclusion: MiNEN is a rare but aggressive tumor with a poor prognosis differing from NENs, GCC and adenocarcinomas. To improve the prognosis of the disease, early diagnosis and comprehensive evaluation are necessary.

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“…Gastrointestinal (GI) tumors are one of the most serious health complications in the world. They mainly include seven major cancers: liver hepatocellular carcinoma, stomach adenocarcinoma, esophageal carcinoma, pancreatic adenocarcinoma, colon adenocarcinoma, cholangiocarcinoma, and rectum adenocarcinoma [ 18 ]. Understanding the molecular markers that predict prognosis of GI tumors can guide clinical management of patients with these cancers.…”

Section: Discussionmentioning

confidence: 99%

Expression of Four Autophagy-Related Genes Accurately Predicts the Prognosis of Gastrointestinal Cancer in Asian Patients

Tang

¹

,

Liang

²

,

Xu

³

et al. 2021

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Gastrointestinal (GI) cancers are among the most fatal diseases in the world. Numerous studies have demonstrated the relationship between autophagy and development of gastrointestinal cancers. However, whether autophagy-related genes can predict prognosis of GI cancers in individuals of Asian ancestry has not been defined. This study, evaluated the prognostic value of autophagy-related genes in gastrointestinal cancer. Expression profile of autophagy-related genes for 296 gastrointestinal cancer patients of Asian ancestry was downloaded from the TCGA database (TCGA-LIHC, TCGA-STAD, TCGA-ESCA, TCGA-PAAD, TCGA-COAD, TCGA-CHOL, and TCGA-READ). The prognostic value of the autophagy-related genes was evaluated using univariate Cox, LASSO, and multivariate Cox regression analyses. The risk score of the autophagy-related gene signature was calculated to assess its predictive prognostic value for GI cancers. Forty-seven differentially expressed autophagy-related genes, in Asian patients with gastrointestinal cancers, were identified. Of the 47 genes, 4 were associated with prognosis of GI cancer (SQSTM1, BIRC5, NRG3, and CXCR4). A prognostic model for GI cancer, based on the expression of the above 4 genes in the training set, showed that cancer patients were stratified into high-risk and low-risk groups ( P < 0.05 ). The utility of the model for overall survival (OS) of GI cancer patients was consistent across the entire set, training set, and test set (entire set: P = 4.568 × 10 − 4 ; train set: P = 5.718 × 10 − 3 ; test set: P = 3.516 × 10 − 2 ). The sensitivity and specificity of the ROC curve of the above prognostic model in predicting the 5-year prognosis of GI cancer was satisfactory (entire set: 0.728; train set: 0.727; test set: 0.733). Analysis of clinical samples validated the overexpression of the 4 genes (SQSTM1, BIRC5, NRG3, and CXCR4) in tumor tissues relative to paired normal tissues, consistent with bioinformatic findings. Expression of the 4 autophagy-related genes (SQSTM1, BIRC5, NRG3, and CXCR4) can accurately predict the prognosis of gastrointestinal tumors in Asian patients.

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“…Since the colon biopsy in our patient was predominantly large cell neuroendocrine carcinoma, which is notorious for its aggressiveness and high metastatic potential [ 20 ], it is very likely to be the culprit of the hepatic lesions, although no biopsy was collected from the liver to confirm this. Wang et al demonstrated a relationship between the size of the primary tumor and the presence of metastatic lesions, regardless of whether the metastasis is to lymph nodes or to distant organs, with primary tumors of more than 2 cm being 2 times more likely to have metastatic lesions [ 25 ].…”

Section: Discussionmentioning

confidence: 99%

Metastatic Mixed Adeno-Neuroendocrine Carcinoma of the Colon to the Liver with Multiple Peritoneal Deposits: A Case Report

AlQattan

¹

,

AlSulaiman

²

,

Aldossary

³

et al. 2021

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Patient: Male, 48-year-old Final Diagnosis: Poorly differentiated mixed adenoneuroendocrine tumor of the colon Symptoms: Abdominal pain • fatigue • weight loss Medication: Azathioprine Clinical Procedure: Colonscopy Specialty: Oncology • Pathology • Surgery Objective: Rare disease Background: Metastatic mixed adeno-neuroendocrine carcinoma (MANEC) is a rare malignancy. It is characterized by the presence of both neuroendocrine and epithelial components, each of which constitute at least 30% of the lesion to establish the diagnosis. Case Report: A 48-year-old man presented with a 1-month history of right upper-quadrant pain and unintentional weight loss of 18 kg. He was also complaining of constipation and fatigue for 6 days. The initial diagnosis from a referring hospital was colon cancer with liver metastasis based on a computed tomography (CT) scan of the chest, abdomen and pelvis. After re-evaluation at our hospital, the scan revealed multiple peritoneal deposits in addition to the previously reported findings. A colonoscopy and biopsy were performed, after which the histo-pathological examination demonstrated a mixed poorly differentiated large cell neuroendocrine carcinoma and adenocarcinoma. Based on the imaging and histopathology reports, he was diagnosed with a poorly differentiated MANEC of the colon with liver metastasis and multiple peritoneal deposits. His lesions were deemed unresectable, and he was referred to the oncology department for palliative care. There he received a total of 9 cycles of cisplatin and etoposide for 8 months. His CT scan showed a regression of the primary tumor indicating a good response to chemotherapy. The patient is still following up with his medical oncologist. Conclusions: Although it is rare, MANEC is a complex neoplasm that requires a high index of suspicion to diagnose due to its nonspecific presentation. It is confirmed through histopathology and immunohistochemistry of the tumor biopsy. Imaging is performed for staging, with most patients presenting at advanced stages with metastases. The only curative option is complete surgical resection of both the primary and metastatic lesion. Many cases, however, are regarded as unresectable and are referred for palliative treatment.

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Gastrointestinal mixed adenoneuroendocrine carcinoma: a population level analysis of epidemiological trends

Cited by 23 publications

References 27 publications

Survival Profile and Prognostic Factors for Appendiceal Mixed Neuroendocrine Non-neuroendocrine Neoplasms: A SEER Population-Based Study

Survival Profile and Prognostic Factors for Appendiceal Mixed Neuroendocrine Non-neuroendocrine Neoplasms: A SEER Population-Based Study

Expression of Four Autophagy-Related Genes Accurately Predicts the Prognosis of Gastrointestinal Cancer in Asian Patients

Metastatic Mixed Adeno-Neuroendocrine Carcinoma of the Colon to the Liver with Multiple Peritoneal Deposits: A Case Report

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