Neuroendocrine neoplasms of the pancreas: clinical, pathologic and cross-sectional imaging analysis according to the 4th edition of the World Health Organization (WHO) classification of tumors of the digestive system (original) (raw)
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Endokrynologia Polska, 2022
This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially Guidelines Beata Kos-Kudła et al. Guidelines persons/year, with the primary lesion most frequently found in the small intestine (37.4%). Since 2000, rectal NENs have been diagnosed more frequently than small intestine NENs [2, 6-8]. The incidence of GEP-NETs in the USA, based on SEER data, was 3.56/100,000 persons/year. In Europe, the incidence of GEP-NETs is also increasing-from 1.33 to 2.33/100,000 persons/year; however, these data come from different registries and are mainly retrospective. Higher incidence is observed among men (5.35/100,000 persons/year) compared to women (4.76/100,000 persons/year) [1, 5-7]. The vast majority of NENs are sporadic, well-differentiated tumours. However, GEP-NETs originating from the pancreas, duodenum, stomach, and much less frequently, from the thymus and lungs sometimes constitute an element of multiple endocrine neoplasia type 1 (MEN-1) syndrome. Pancreatic neuroendocrine tumours (PanNETs) may also be associated with von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex (TSC), and neurofibromatosis (NF). In these congenital diseases, NETs can be multifocal and occur 10-20 years earlier than in sporadic cases. The frequency of the hereditary causes (MEN-1, VHL) is estimated at about 5%. Genome studies revealed the presence of germline mutations in, e.g., MUTYH, CHEK2, and BRCA2 and a propensity to PanNETs in approximately 17% of the studied population [1]. 2. Diagnostics 2.1. Biochemical diagnostics Biochemical diagnostics of NENs involves the following: A. Non-specific markers The most frequently used diagnostic method is determination of the chromogranin A (CgA) concentration in the serum (less frequently in the plasma) [1, 8, 9]. CgA is a relatively stable protein in blood. However, there are two different methods for determining the concentration of CgA: radioimmunoassay (RIA) and enzyme-linked immunosorbent assay (ELISA) in Neuroendocrine neoplasms (NENs) arise from the disseminated system of neuroendocrine cells and can occur in various parts of the body. However, they are most often found in the gastrointestinal tract and lungs. The term NENs includes both well-differentiated neuroendocrine tumours and neuroendocrine carcinomas (NECs), which account for 10-20% of all NENs. The following characteristics of NENs should be considered in the diagnostic and therapeutic process: proliferative activity, presence of somatostatin receptors (SSTRs), tumour growth rate, and extent of the neoplastic disease [1]. in the prevalence of GEP-NENs. Local and regional NENs are diagnosed more frequently than those with distant metastases. The detectability of NENs is increasing, e.g. from 1973 to 2004 the incidence of NENs increased from 2.1 to 5.25 new cases per 100,000
The Neuroendocrine Cancer. Personal Comments and Operational Remarks
Summary Indroduction: Neuroendocrine tumors (NEN) of the gastro-entero-pancreatic tract (GEP) are a group in themselves very heterogeneous of tumors that are different for the site of localization in the digestive tract (foregut, midgut and hindgut), both in relation to the pathological aspects, functional activity and nosographic classification.
A retrospective analysis of neuroendocrine tumour of pancreas: a single institute study
International Journal of Research in Medical Sciences, 2015
Endocrine neoplasms can be divided according to the chemical nature of their secretion products into two groups. Neoplasms that secrete peptide hormones and biogenic amines comprise the first group. The second group includes the tumours that generate steroid hormones. The tumours of the first group are called neuroendocrine neoplasms (NENs) because of the marker proteins that they share with the neural cell systemsynaptophysin and neuron specific enolase and others like chromogranins A, B and C and the proprotein convertases PC2 and PC3. 1,2 The neural cell adhesion molecule CD56 is positive in many NENs, but is not specific for these tumours. 1 Under the electron microscope the NENs show typical neurosecretory granules. Approximately two-thirds of NETs are found in the gastrointestinal tract, one quarter occurs in the lungs, and the remaining cases arise in other endocrine tissues, such as the thyroid. These tumors account for approximately 2% of all malignancies of the gastrointestinal tract. 3,4 NETS in the gastrointestinal tract occur predominantly in the small intestine (44.7%), followed by the rectum (19.6%), appendix (16.7%), colon (10.6%), and stomach (7.2%). 3,5 Pancreatic NENs ABSTRACT Background: The aim of the work was the clinical characteristics and analysis of preliminary results for surgical treatment of pancreatic neuroendocrine tumors (PNETs). This article deals with the classification of the Pancreatic Neuroendocrine Tumors (PNETs) and discusses their presentation, behaviour, treatment and prognosis. Methods: This was a retrospective study of 70 patients of PNET done over a period of 3 years in The Gujarat Cancer and Research Institute, Ahmedabad. 24 patients who underwent surgical treatment for PNET were further evaluated for surgical outcome, 5yr disease free survival and overall survival. Results: In this study of 70 patients, 61(87.14%) were non-functional. Approximately 77% of PNETs were advanced on presentation (57% metastatic and 20% locally advanced). 20 patients had disease resectable on presentation (11 NF + 9 F). These 20 patients belong to stage I and II of TNM staging system. Only 4 out of 40 metastatic diseases had locally resectable tumor. Of 24 patients who underwent surgery, 12 underwent pancreatico-duodenectomy, 6 underwent enucleation and 6 underwent distal pancreatectomy. Conclusions: PNETs are uncommon tumor of pancreatic origin with presentation more commonly in males than females, usually in the 5th decade. Approximately 77% of patients are advanced or metastatic at presentation. Among those resectable, the Overall Survival for FPNETs and NFPNETs was 90% and 94% respectively and 5yr Disease Free Survival for the same was 100% and 84% respectively.
Pancreas, 2011
Objective: To present our experience of 93 neuroendocrine tumors (NETs) in the pancreas and peripancreatic region, with emphasis on how resectability affects long-term survival and the impact of functional status on the survival outcome. Methods: Ninety-three patients with NETs in the pancreas and peripancreatic region were included to compare the clinical features between functional and nonfunctional NETs. Prognostic factors were determined by univariate and multivariate analyses. Results: There were 39 functional (41.9%) and 54 nonfunctional NETs (58.1%). According to World Health Organization (WHO) tumor categories, therewere 57 well-differentiated tumors (61.3%), 26 well-differentiated carcinomas (28%), and 10 poorly differentiated carcinomas (10.8%). Univariate analysis showed that functional status of the tumor, tumor stage, lymph node status, and pathological classification were prognostic factors for both disease-free survival and disease-specific survival. Resectability did not influence the survival outcome, with the resectable and unresectable groups demonstrating a 5-year disease-specific survival of 86.4% and 65.6%, respectively (P = 0.210). Only the WHO pathological classification was an independent prognostic factor after multivariate analysis. Conclusions: Irresectability does not necessarily preclude long-term survival for both functional and nonfunctional NETs. It is the WHO pathological classification, instead of hormonal functional status, that is an independent prognostic factor and has impact on the survival outcome.
Pancreatic Neuroendocrine Tumors: A Literature Review
JOURNAL OF BIOENGINEERING AND TECHNOLOGY APPLIED TO HEALTH, 2020
Pancreatic neuroendocrine tumors (PNETs) affect 1%-3% of patients with pancreatic cancer. This tumor is rare, difficult to diagnose, and clinically laborious. They have an estimated incidence of up to 1 case per 100,000 inhabitants every year. Up on diagnosis, most PNETs are considered malignant, with low healing potential, lesions that are generally unresectable, and a metastasis rate of aproximately 50%. PNETs are classified as functional and non-functional. The tumor functional produces hormones such as gastrin, insulin, somatostatin, glucagon, among others. They are symptomatic due to hormonal hypersecretion and occur in 30% of cases. The other 70% are non-functioning, and despite producing a series of substances and some hormones such as beta HCG and alpha HCG, they are silent tumors, with no significant clinical syndrome. The present study presented scientific evidence about PNETs, the types of pancreas endocrine-tissue tumors, the rate of survival, diagnosis, treatments, and ...
Poorly Differentiated Neuroendocrine Carcinomas of the Pancreas
American Journal of Surgical Pathology, 2014
Background-In the pancreas, poorly differentiated neuroendocrine carcinomas include small cell carcinoma and large cell neuroendocrine carcinoma and are rare; data regarding their pathologic and clinical features are very limited. Design-One hundred and seven pancreatic resections originally diagnosed as poorly differentiated neuroendocrine carcinomas were reassessed using the classification and grading (mitotic rate/Ki67 index) criteria put forth by the WHO in 2010 for the gastroenteropancreatic system. Immunohistochemical labeling for neuroendocrine and acinar differentiation markers was performed. Sixty-three cases were reclassified, mostly as well differentiated neuroendocrine tumor or acinar cell carcinoma, and eliminated. The clinicopathologic features and survival of the remaining 44 poorly differentiated neuroendocrine carcinomas were further assessed. Results-The mean patient age was 59 years (range, 21-82) and the male/female ratio was 1.4. Twenty-seven tumors were located in the head of the pancreas, 3 in the body and 11 in the tail. The median tumor size was 4 cm (range, 2-18). Twenty-seven tumors were large cell neuroendocrine carcinomas and 17 were small cell carcinomas (mean mitotic rate, 37/10 HPF and 51/10 HPF; mean Ki67 index, 66% and 75%, respectively). Eight tumors had combined components, mostly adenocarcinomas. In addition, 2 tumors had components of well differentiated neuroendocrine tumor. Eighty-eight percent of the patients had nodal or distant metastatic disease at presentation and an additional 7% developed metastases subsequently. Follow-up information was available for 43 patients; 33 died of disease with a median survival of 11 months (range, 0-104); 8 were alive with disease, with a median follow-up of 19.5 months (range, 0-71). The 2-year and 5-year survival rates were 22.5% and 16.1%, respectively. Conclusion-Poorly differentiated neuroendocrine carcinoma of the pancreas is a highly aggressive neoplasm, with frequent metastases and poor survival. Most patients die within less than a year. Most (61%) are large cell neuroendocrine carcinomas. Well differentiated neuroendocrine tumor and acinar cell carcinoma are often misdiagnosed as poorly differentiated neuroendocrine carcinoma, emphasizing that diagnostic criteria need to be clearly followed to ensure accurate diagnosis.
Gastrointestinal Tumors
Pancreatic adeno- Mixed neuroendocrine non-endocrine (pMINEN) are extremely rare. [1] They are known to have distal metastasis at presentation and also have a comparatively lower survival rate as compared to similar staged neuroendocrine carcinoma, adenocarcinoma, and small cell lung tumor from which its treatment patterns are extrapolated. [2,3] Also, very less is known about its molecular structure and natural courses. There is a dearth of data about pMINEN in literature also there is a lack of large multicentral trials due to which the MINEN do not have a standard universal management protocol. We here discuss the clinical dilemmas that arise during diagnosis, reporting, and urge to formulate a multi-centric trial to formulate a focused protocolized approach. We here describe our encounter with a pancreatic head lesion which on immunohistochemically turned out to be a pMINEN with moderately differentiating ductal adenocarcinoma and low-grade neuroendocrine tumor. Radical R0 surge...