scholarly journals Diffuse Endocrine System, Neuroendocrine Tumors and Immunity: What’s New?

2012 ◽  
Vol 95 (4) ◽  
pp. 267-276 ◽  
Author(s):  
Pietro Ameri ◽  
Diego Ferone
2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e22120-e22120
Author(s):  
M. S. O'Dorisio ◽  
P. Navalkele ◽  
T. M. O'Dorisio ◽  
C. F. Lynch

e22120 Background: Neuroendocrine tumors (NET) arising from the diffuse endocrine system are thought to be quite rare in children and young adults. However, a surprising number of young people have been referred to our neuroendocrine tumor clinic and the NCI has targeted NET as a high priority for development of new diagnostic and therapeutic options. This analysis of the SEER database was undertaken to determine the incidence, prevalence, and survival of NET in young people. Their incidence, prevalence, and survival were compared with neuroblastoma, a related pediatric malignancy arising in the neural crest. Methods: The SEER data were obtained from 9 standard SEER registries for the diagnosis years of 1975 to 2004 using SEER*Stat version 6.4.4. ICD-9 codes related to neuroendocrine tumors and to neuroblastoma were characterized as to patient age, gender, racial and ethnic background, stage, grade, histology, incidence, survival, and prevalence. Results: Neuroendocrine tumors occur more often in females among children and young adults with the most common sites being bronchial, ovarian, and breast. The overall incidence of neuroendocrine tumors was lower than for neuroblastoma in the age range 0–30 years. However, the 30 year limited prevalence of neuroendocrine tumors in the 9 SEER registries was 698 compared to 881 for neuroblastoma. This extrapolated to over 7000 children and young adults with neuroendocrine tumors across the United States. Survival rate of young people with neuroendocrine tumors declined from 84% in 1975–1986 to 80% in the 1987–2004 era. Conclusions: These results indicate that neuroendocrine tumors constitute an unrecognized cancer threat to children and young adults. Survival of children and young adults with neuroendocrine tumors has decreased over the past 30 years in the United States. We recommend the establishment of centers of care for children and young adults diagnosed with neuroendocrine tumors with the expectation that earlier diagnosis coupled with targeted therapies will decrease the incidence of metastatic disease and improve survival. No significant financial relationships to disclose.


Author(s):  
S. Rybakov

The literature review is devoted to the analysis of numerous studies that have formed the basis for creating ideas about the diffuse endocrine system. In addition to the main endocrine glands, also there are cellular accumulations and individual cells that produce various hormones and biologically active substances. It is established that, in particular, in the organs of the gastrointestinal tract, starting from the esophagus and to the terminal parts of the large intestine, there are several types of endocrine cells with these functions. Most of them are in the pancreas, antrum of the stomach, duodenum, the initial part of the intestine, in the liver. Excessive secretory activity of these cells can cause the development of pathological clinical syndromes, characterized by the effect of the corresponding hormones. The morphological substrate of such syndromes may be tumors or diffuse hyperplasia of the corresponding cells. The author considers the pancreas as an integral part of the diffuse endocrine system, as specific endocrine functions in it perform numerous accumulations (islets of Langerhans) or individual cells. The review of the numerous neuroendocrine tumors of the gastrointestinal tract examines in detail the characteristics of some of them in historical and clinical aspects, in particular insulinoma as the most common and well-studied tumor. Glucagonoma is also considered as a neuroendocrine tumor derived from α-cells of the pancreas, is a kind of insulinoma antagonist, as it secretes an excess of the hormone glucagon, causing the development of hyperglycemic clinical syndrome in contrast to hypoglycemic, caused by insulin. It is a rare tumor and its consideration after insulinoma can be explained by the antagonistic nature of the action of hormones produced by these tumors. In the group of neuroendocrine tumors of the pancreas, glucagonoma follows the frequency of insulinoma and gastrinoma.


Author(s):  
S. I. Rybakov

The literature review presents the analysis of numerous studies that have formed the basis for creating ideas about the diffuse endocrine system,namely existence of the cellular accumulations and individual cells that produce various hormones and biologically active substances in addition to the main endocrine glands. Several types of endocrine cells with these functionshave been established, in particular, in the organs of the gastrointestinal tract, starting from the esophagus and up to the terminal parts of the large intestine. Most of them are contained in the pancreas, antrum of the stomach, duodenum, the initial part of the intestine, in the liver. Excessive secretory activity of these cells can cause the development of pathological clinical syndromes, characterized by the effects of the corresponding hormones. The tumors or diffuse hyperplasia of the corresponding cellsmay present morphological substrate of such syndromes. The author considers the pancreas as an integral part of the diffuse endocrine system, as specific endocrine functions in it perform numerous accumulations (islets of Langerhans) or individual cells. The review of the numerous neuroendocrine tumors of the gastrointestinal tract examines in detail the characteristics of some of them in historical and clinical aspects, in particular Zollinger—Ellison syndrome as the most common and well-studied tumor. Glucagonoma is also considered as a neuroendocrine tumorderived from α-cells of the pancreas, is a kind of insulinoma antagonist, as it secretes an excess of the hormone glucagon, causing the development of hyperglycemic clinical syndrome in contrast to hypoglycemic, caused by insulin. It is a rare tumor and its consideration after insulinoma can be explained by the antagonistic nature of the action of hormones produced by these tumors. In the group of neuroendocrine tumors of the pancreas, glucagonoma follows the frequency of insulinoma and gastrinoma.


2019 ◽  
Vol 03 (01) ◽  
pp. 003-013
Author(s):  
Sanjit Datta ◽  
Annika Sinha ◽  
Baljendra Kapoor

AbstractGastrointestinal neuroendocrine tumors (GI-NETs), previously classified as carcinoid tumors, are rare cancers that arise from cells of the diffuse endocrine system of the gastrointestinal tract. These tumors most commonly arise from the bronchus, jejunoileum, or colon/rectum. They produce peptide products that can lead to identifiable clinical syndromes such as carcinoid syndrome, which is classically associated with diarrhea, flushing, and heart disease. The latest classifications of GI-NETs include pancreatic NETs, which can produce a wide range of pancreatic hormones leading to syndromes such as Zollinger–Ellison's syndrome. The prognosis for patients with GI-NETs varies widely depending on the size, location, and presence of metastatic disease, with 5-year survival rates ranging from 0 to 15% for poorly differentiated NETs and 10-year survival rates of up to 100% for patients with insulinomas smaller than 2 cm. A wide range of treatment modalities is commonly used to treat GI-NETs, including surgical and endoscopic resection, locoregional therapies, cytotoxic chemotherapy, and somatostatin receptor targeted therapy. The goal of this review is to detail the classification, epidemiology, clinical syndromes, diagnosis, and staging of these tumors and to provide an overview of management strategies.


2021 ◽  
Vol 12 ◽  
Author(s):  
Marta Opalińska ◽  
Anna Sowa-Staszczak ◽  
Helena Olearska ◽  
Magdalena Ulatowska-Bialas ◽  
Aleksandra Gilis-Januszewska ◽  
...  

BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma – ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.Materials and MethodsThe group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.Results70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.ConclusionsThe prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.


Author(s):  
R. Ramachandran ◽  
W. Dhillo

Neuroendocrine cells occur either singly or in small groups in a variety of tissues and organs. Although morphologically and embryologically diverse, they are characterized by a number of unifying features. They have dense core secretory vesicles in the cytoplasm and hormone receptors on the cell membranes. There is evidence of prohormone activity within the cells and they synthesize, store, and secrete hormones. In addition, neuroendocrine cells possess an ability to take up and decarboxylate amine precursors. Components of this diffuse endocrine system are particularly prominent in the gastrointestinal tract, pancreas, C cells of the thyroid, adrenal medulla, parathyroid tissue, respiratory tract, skin, and genitourinary system. Neuroendocrine tumours (NETs) are known to occur in all these tissues. Historically, the diagnosis of NET was made on the basis of characteristic histological findings. The significantly worse prognosis in advanced disease and the availability of multiple therapeutic options have highlighted the need for robust tumour markers that can be used both for diagnosis and follow-up. Currently, a number of normal and abnormal forms of peptides, biogenic amines, and hormones, secreted by NETs, are routinely measured as markers of disease. An ideal tumour marker would be one that is secreted exclusively by the tumour cells and is useful (1) for screening and differential diagnosis of NETs; (2) as a prognostic indicator; (3) as an estimate of tumour burden; and (4) as a surveillance tool. Although none of the currently available markers completely fits the paradigm for an ideal tumour marker, when measured in conjunction with each other, they are useful not only for making a diagnosis but also for monitoring response to therapy and in surveillance post-remission.


2019 ◽  
Vol 25 (2) ◽  
pp. 72-77
Author(s):  
M.I. Sydorenko

In recent decades, diseases of the digestive system that require immediate, both therapeutic and surgical treatment, have become widespread, and it is therefore a natural task to find new and optimize existing technologies and methods for correcting the above-mentioned nosologies. Preclinical studies of such developments are conducted exclusively on laboratory animals and knowledge of the morphological features of their structure for further comparison with the morphology of similar human organs is an urgent task of modern medical and biological science. The purpose of the study was to study the structural organization of the ileum of rabbits in comparative species and to obtain control data on its morphological features. Adequate research methods were used in the work according to the set goal, namely: histological, histochemical, electron microscopic, morphometric and statistical and biopsies of the ileum of 10 rabbits were studied. The correctness of the distribution of traits by each of the variations obtained, the average values for each trait studied, standard errors and standard deviations were evaluated. The significance of the difference of values between independent micrometric values in the normal distribution of features was determined by Student’s criterion. The paper describes the main structural components of the ileum of rabbits and compared with similar structures of the human ileum. The ileum of rabbits, as in humans, has been determined to have four membranes: mucous, submucosal, muscular and serous. The mucous membrane is constructed from the epithelial layer, which is located on the basement membrane and the muscular plate and contains cellular elements (exocrinocytes, enterocytes of various kinds, elements of the diffuse endocrine system associated with the mucous membrane, intraepithelial lymphocytes), blood and lymphatic vessels and nerve endings. The submucosa is composed of loose fibrous connective tissue, which contains collagen and reticular fibers, elements of diffuse lymphoid tissue, blood vessels, and nerve endings. The muscular and serous membranes are constructed in the same way as in the human ileum. Thus, after the study, it was determined that the morphological organization of the ileum of rabbits at the optical and electron microscopic levels has general patterns of structure similar to those in the ileum of the person.


2004 ◽  
Vol 229 (10) ◽  
pp. 1007-1016 ◽  
Author(s):  
Guido Rindi ◽  
Antonio Torsello ◽  
Vittorio Locatelli ◽  
Enrico Solcia

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