Neuroendocrine neoplasms of the appendix, colon and rectum

Volante, Marco; Grillo, Federica; Massa, Federica; Maletta, Francesca; Mastracci, Luca; Campora, Michela; Ferro, Jacopo; Vanoli, Alessandro; Papotti, Mauro

doi:10.32074/1591-951x-230

Cited by 52 publications

(52 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The histological features of A-NENs are similar to those of NENs of other primary sites, with the exception of tubular NETs. Tubular NETs are rare benign neoplasms with a predominant tubular growth pattern, so it is important not to misdiagnose them as adenocarcinomas[ 61 ]. Of note, goblet cell adenocarcinoma (formerly goblet cell carcinoid) is no longer considered an A-NEN[ 62 ].…”

Section: Clinical Presentation Diagnosis and Treatmentmentioning

confidence: 99%

New insights in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms

Yin¹,

Wu²,

Lai³

2022

WJG

View full text Add to dashboard Cite

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are rare epithelial neoplasms derived from pluripotent endocrine cells along the gastrointestinal tract and pancreas. GEP-NENs are classified into well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas. Despite overlapping morphological features, GEP-NENs vary in molecular biology, epigenetic, clinical behavior, treatment response, and prognosis features and remain an unmet clinical challenge. In this review, we introduce recent updates on the histopathologic classification, including the tumor grading and staging system, molecular genetics, and systemic evaluation of the diagnosis and treatment of GEP-NENs at different anatomic sites, together with some insights into the diagnosis of challenging and unusual cases. We also discuss the application of novel therapeutic approaches for GEP-NENs, including peptide receptor radionuclide therapy, targeted therapy, and immunotherapy with immune checkpoint inhibitors. These findings will help improve patient care with precise diagnosis and individualized treatment of patients with GEP-NENs.

show abstract

Section: Clinical Presentation Diagnosis and Treatmentmentioning

confidence: 99%

New insights in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms

Yin¹,

Wu²,

Lai³

2022

WJG

View full text Add to dashboard Cite

show abstract

“…However, comprehensive information on this subject can be reviewed in a previous study[ 10 ]. Although several biomarkers, including neuron-speciﬁc enolase (NSE), CD57, protein gene product 9.5 (PGP 9.5), insulinoma-associated protein 1 (INSM1) and somatostatin receptor subtype 2A (SSTR2A), have been described to date, the most widely used and reliable neuroendocrine markers are chromogranin A, synaptophysin, and CD56[ 11 , 12 ]. In the nonneuroendocrine component, adenocarcinomas express carcinoembryonic antigen, CA 19-9, cytokeratins 7, 19, and AE 1/3.…”

Section: Diagnosis Of Minenmentioning

confidence: 99%

“…In the nonneuroendocrine component, adenocarcinomas express carcinoembryonic antigen, CA 19-9, cytokeratins 7, 19, and AE 1/3. The immunohistochemical features of other tumors that make up this component are presented below according to their localization in different organs of the GIS[ 10 - 12 ].…”

Section: Diagnosis Of Minenmentioning

confidence: 99%

Mixed neuroendocrine–nonneuroendocrine neoplasms of the gastrointestinal system: An update

Elpek¹

2022

WJG

View full text Add to dashboard Cite

Mixed neuroendocrine-nonneuroendocrine neoplasms (MiNENs) of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment. Over the years, the diagnostic criteria, classification, and clinical behavior of these tumors have been the subjects of ongoing debate, and the various changes in their nomenclature have strengthened the challenges associated with MiNENs. This review is performed to provide an understanding of the key factors involved in the evolution of the designation of these tumors as MiNEN, highlight the current diagnostic criteria, summarize the latest data on pathogenesis and provide information on available treatments. Moreover, this work seeks to increase the awareness about these rare neoplasms by presenting the clinicopathological features and prognostic factors that play important roles in their behavior and discussing their different regions of origin in the gastrointestinal system (GIS). Currently, the MiNEN category also includes tumors in the GIS with a nonneuroendocrine component and epithelial tumors other than adenocarcinoma, depending on the organ of origin. Diagnosis is based on the presence of both morphological components in more than 30% of the tumor. However, this value needs to be reconfirmed with further studies and may be a limiting factor in the diagnosis of MiNEN by biopsy. Furthermore, available clinicopathological data suggest that the inclusion of amphicrine tumors in the definition of MiNEN is not supportive and warrants further investigation. The diagnosis of these tumors is not solely based on immunohistochemical findings. They are not hybrid tumors and both components can act independently; thus, careful grading of each component separately is required. In addition to parameters such as the metastatic state of the tumor at the time of diagnosis and the feasibility of surgical resection, the aggressive potential of both components has paramount importance in the choice of treatment. Regardless of the organ of origin within the GIS, almost MiNENs are tumors with poor prognosis and are frequently encountered in the elderly and men. They are most frequently reported in the colorectum, where data from molecular studies indicate a monoclonal origin; however, further studies are required to provide additional support for this origin.

show abstract

“…Rectal NENs encompass rectal neuroendocrine tumours (NETs) and rare poorly differentiated neuroendocrine carcinomas (NECs) and mixed neuroendocrine-non neuroendocrine neoplasm (MiNEN). The well-differentiated rectal NETs can be sub-classified by tumour cell type to L-cell rectal NETs which are the most common sub-type and the EC-cell rectal NETs [ 6 ••]. The L-cell rectal NETs are commonly small with 75–88% being less than 1 cm in size; on immunohistochemical analysis, they may be chromogranin A (CgA) and CDX-2 negative.…”

Section: Introductionmentioning

confidence: 99%

“…However, they can stain positive for glucagon like peptides (GLP-1) and pancreatic polypeptide (PP). Conversely, the EC-cell rectal NETs have immunohistochemical features similar to small bowel NETs and are often CgA and CDX-2 positive [ 6 ••]. There is some evidence to suggest a non-L-cell immunophenotype is associated with more aggressive clinical behaviour and worse prognosis.…”

Section: Introductionmentioning

confidence: 99%

Rectal Neuroendocrine Neoplasms: Why Is There a Global Variation?

Cope

Srirajaskanthan

2022

Curr Oncol Rep

View full text Add to dashboard Cite

Purpose of Review This review examines the variation in incidence of rectal neuroendocrine tumours across the globe. Rectal neuroendocrine tumours are a common type of gastrointestinal NET with an increasing incidence reported over the last 30 years. Recent Findings There have been a number of publications examining the epidemiology of neuroendocrine tumours across the world. These have utilized a variety of different methodologies to examine both incidence of prevalence of NETs. We review the data published and describe any causative factors and findings regarding the epidemiology of rectal NETs. Summary Rectal NETs account for 1–2% of all rectal cancers and are commonly diagnosed between 50–60 years of age. Most lesions are identified by chance at colonoscopy, commonly during colon cancer screening procedures, which is reflected in part in the age at diagnosis. Most lesions are small in size, < 10 mm and can be managed with endoscopic resection rather than requiring surgery. The highest incidence is reported in people of Asian ethnicity, with a tenfold increased incidence reported in some series compared with white population. There is also an increased incidence in Black and Hispanic population as identified through the Surveillance, Epidemiology and End Results (SEER) database. Endoscopic assessment of lesions is variable globally. Future work to better understand the cause of ethnic variation and development of comprehensive cancer registries would be helpful.

show abstract

Neuroendocrine neoplasms of the appendix, colon and rectum

Cited by 52 publications

References 17 publications

New insights in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms

New insights in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms

Mixed neuroendocrine–nonneuroendocrine neoplasms of the gastrointestinal system: An update

Rectal Neuroendocrine Neoplasms: Why Is There a Global Variation?

Contact Info

Product

Resources

About