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Current Opinion in Endocrinology,... Feb 2018To review recent advances and controversies in all aspects of carcinoid-syndrome. (Review)
Review
PURPOSE OF REVIEW
To review recent advances and controversies in all aspects of carcinoid-syndrome.
RECENT FINDINGS
Over the last few years there have been a number of advances in all aspects of carcinoid syndrome as well as new therapies. These include new studies on its epidemiology which demonstrate it is increasing in frequency; increasing insights into the pathogenesis of its various clinical manifestations and into its natural history: definition of prognostic factors; new methods to verify its presence; the development of new drugs to treat its various manifestations, both initially and in somatostatin-refractory cases; and an increased understanding of the pathogenesis, natural history and management of carcinoid heart disease. These advances have generated several controversies and these are also reviewed.
SUMMARY
There have been numerous advances in all aspects of the carcinoid-syndrome, which is the most common functional syndrome neuroendocrine tumors produce. These advances are leading to new approaches to the management of these patients and in some cases to new controversies.
Topics: Carcinoid Tumor; Humans; Malignant Carcinoid Syndrome; Medical Oncology; Neuroendocrine Tumors; Somatostatin
PubMed: 29120923
DOI: 10.1097/MED.0000000000000376 -
International Journal of Cancer Jun 2022Lung carcinoid tumors, also referred to as pulmonary neuroendocrine tumors or lung carcinoids, are rare neoplasms of the lung with a more favorable prognosis than other...
Lung carcinoid tumors, also referred to as pulmonary neuroendocrine tumors or lung carcinoids, are rare neoplasms of the lung with a more favorable prognosis than other subtypes of lung cancer. Still, some patients suffer from relapsed disease and metastatic spread. Several recent single-cell studies have provided detailed insights into the cellular heterogeneity of more common lung cancers, such as adeno- and squamous cell carcinoma. However, the characteristics of lung carcinoids on the single-cell level are yet completely unknown. To study the cellular composition and single-cell gene expression profiles in lung carcinoids, we applied single-cell RNA sequencing to three lung carcinoid tumor samples and normal lung tissue. The single-cell transcriptomes of carcinoid tumor cells reflected intertumoral heterogeneity associated with clinicopathological features, such as tumor necrosis and proliferation index. The immune microenvironment was specifically enriched in noninflammatory monocyte-derived myeloid cells. Tumor-associated endothelial cells were characterized by distinct gene expression profiles. A spectrum of vascular smooth muscle cells and pericytes predominated the stromal microenvironment. We found a small proportion of myofibroblasts exhibiting features reminiscent of cancer-associated fibroblasts. Stromal and immune cells exhibited potential paracrine interactions which may shape the microenvironment via NOTCH, VEGF, TGFβ and JAK/STAT signaling. Moreover, single-cell gene signatures of pericytes and myofibroblasts demonstrated prognostic value in bulk gene expression data. Here, we provide first comprehensive insights into the cellular composition and single-cell gene expression profiles in lung carcinoids, demonstrating the noninflammatory and vessel-rich nature of their tumor microenvironment, and outlining relevant intercellular interactions which could serve as future therapeutic targets.
Topics: Carcinoid Tumor; Carcinoma, Neuroendocrine; Endothelial Cells; Humans; Lung; Lung Neoplasms; Neuroendocrine Tumors; Prognosis; Tumor Microenvironment
PubMed: 35262195
DOI: 10.1002/ijc.33995 -
American Family Physician Aug 2006Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that often are indolent and may not become clinically apparent until there has been metastatic spread or... (Review)
Review
Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that often are indolent and may not become clinically apparent until there has been metastatic spread or evidence of carcinoid syndrome. Recent evidence has revealed that the overall incidence of carcinoid tumors has been steadily increasing, and although the disease was thought to be relatively benign, it is now considered one of increasing malignancy. Carcinoid tumors derive from different embryonic divisions of the gut: foregut carcinoid tumors commonly originate in the lungs, bronchi, or stomach; midgut carcinoid tumors in the small intestine, appendix, or proximal large bowel; and hindgut carcinoid tumors in the distal colon or rectum. Carcinoid syndrome, although rare, is most associated with midgut carcinoid tumors. The diagnosis of a carcinoid tumor often is coincidental with surgery performed for another reason. Treatment and prognosis are dependent on the location of the primary tumor and the degree and extent of metastasis at the time of diagnosis.
Topics: Carcinoid Tumor; Diagnosis, Differential; Digestive System Neoplasms; Humans; Malignant Carcinoid Syndrome; Prognosis; Respiratory Tract Neoplasms
PubMed: 16913162
DOI: No ID Found -
The Oncologist Dec 2008Carcinoid tumors are rare, slow-growing neuroendocrine tumors arising from the enterochromaffin cells disseminated throughout the gastrointestinal and bronchopulmonary... (Review)
Review
Carcinoid tumors are rare, slow-growing neuroendocrine tumors arising from the enterochromaffin cells disseminated throughout the gastrointestinal and bronchopulmonary systems. Though they have been traditionally classified based on embryologic site of origin, morphologic pattern, and silver affinity, newer classification systems have been developed to emphasize the considerable clinical and histopathologic variability of carcinoid tumors found within each embryologic site of origin. These neoplasms pose a diagnostic challenge because they are often innocuous at the time of presentation, emphasizing the need for a multidisciplinary diagnostic approach using biochemical analysis, standard cross-sectional imaging, and newer advances in nuclear medicine. Similarly, treatment of both primary and disseminated carcinoid disease reflects the need for a multidisciplinary approach, with surgery remaining the only curative modality. The prognosis for patients with these tumors is generally favorable; however, it can be quite variable and is related to the location of the primary tumor, extent of metastatic disease at initial presentation, and time of diagnosis.
Topics: Carcinoid Tumor; Humans; Incidence; Intestinal Neoplasms; Liver Neoplasms; Lung Neoplasms; Stomach Neoplasms
PubMed: 19091780
DOI: 10.1634/theoncologist.2008-0207 -
Cancer Medicine Apr 2023Neuroendocrine tumors (NETs) are epithelial malignancies that can arise from multiple tissues. Gastrointestinal (GI) NETs are the most common; in this review of... (Review)
Review
Neuroendocrine tumors (NETs) are epithelial malignancies that can arise from multiple tissues. Gastrointestinal (GI) NETs are the most common; in this review of extra-abdominal carcinoid tumors, we focus our discussion on bronchial and thymic carcinoid tumors. Bronchial carcinoid tumors comprise a quarter of all NETs and less than 2% of all lung cancers. Thymic carcinoid tumors are extremely rare, accounting for 5% of thymic tumors. Both bronchial and thymic carcinoid tumors are histologically classified as either typical or atypical based on their mitotic rate (less than 2 or 2-10 mitoses per 10 high-powered fields (HPF), respectively). Both bronchial and thymic carcinoids can present with symptoms of obstruction and potentially carcinoid syndrome. The gold standard of management of bronchial and thymic carcinoid tumors is surgical resection. For patients with advanced disease, first-line systemic therapy is generally somatostatin analog monotherapy with octreotide or lanreotide. In patients with refractory disease, therapy generally involves peptide receptor radioligand therapy, everolimus, or cytotoxic chemotherapy. There are ongoing, prospective trials comparing the mainstays of systemic therapy for these patients, as well as ongoing evaluations of immune checkpoint inhibitors and multi-kinase inhibitors. Prognosis for both bronchial and thymic carcinoid tumors depends on histologic grade, local versus invasive disease, and extent of metastases. Herein we provide a summary of the pathophysiologic and clinical background, the current state of the field in diagnosis and management, and note of key ongoing prospective trials for patients with bronchial and thymic carcinoid tumors.
Topics: Humans; Prospective Studies; Carcinoid Tumor; Bronchial Neoplasms; Neuroendocrine Tumors; Abdomen
PubMed: 36560885
DOI: 10.1002/cam4.5564 -
Annals of Oncology : Official Journal... Apr 2021
Topics: Carcinoid Tumor; Follow-Up Studies; Humans; Lung; Lung Neoplasms; Prognosis; Thymus Neoplasms
PubMed: 33482246
DOI: 10.1016/j.annonc.2021.01.003 -
Hellenic Journal of Nuclear Medicine 2023The carcinoid syndrome (CS) is a constellation of symptoms attributed to hypersecretion of amines, prostaglandins and polypeptides. The cardinal symptoms of CS are...
The carcinoid syndrome (CS) is a constellation of symptoms attributed to hypersecretion of amines, prostaglandins and polypeptides. The cardinal symptoms of CS are flushing, diarrhea and bronchospasm; however, CS may present with various symptoms and signs, as: Skin: cutaneous flushes, cyanosis, pellagra, Gastrointestinal: diarrhea, nausea, abdominal cramps, vomiting, Heart: tricuspid and pulmonic valve thickening causing right heart failure, edema, Respiratory: wheezing, dyspnea.
Topics: Humans; Carcinoid Heart Disease; Malignant Carcinoid Syndrome; Diarrhea; Carcinoid Tumor
PubMed: 37658565
DOI: No ID Found -
Molecules (Basel, Switzerland) Feb 2023Evasion of innate immunity represents a frequently employed method by which tumor cells survive and thrive. Previously, the development of immunotherapeutic agents... (Review)
Review
Evasion of innate immunity represents a frequently employed method by which tumor cells survive and thrive. Previously, the development of immunotherapeutic agents capable of overcoming this evasion has realized pronounced clinical utility across a variety of cancer types. More recently, immunological strategies have been investigated as potentially viable therapeutic and diagnostic modalities in the management of carcinoid tumors. Classic treatment options for carcinoid tumors rely upon surgical resection or non-immune pharmacology. Though surgical intervention can be curative, tumor characteristics, such as size, location, and spread, heavily limit success. Non-immune pharmacologic treatments can be similarly limited, and many demonstrate problematic side effects. Immunotherapy may be able to overcome these limitations and further improve clinical outcomes. Similarly, emerging immunologic carcinoid biomarkers may improve diagnostic capabilities. Recent developments in immunotherapeutic and diagnostic modalities of carcinoid management are summarized here.
Topics: Humans; Carcinoid Tumor; Biomarkers; Immunologic Factors
PubMed: 36903295
DOI: 10.3390/molecules28052047 -
Revista Espanola de Enfermedades... Sep 2010carcinoid tumors (CTs) represent the commonest neuroendocrine tumors. Those in the gastrointestinal tract are diagnosed in surgical specimens, clinically, and using...
OBJECTIVE
carcinoid tumors (CTs) represent the commonest neuroendocrine tumors. Those in the gastrointestinal tract are diagnosed in surgical specimens, clinically, and using imaging techniques (endoscopy, echoendoscopy, CT, Octreoscan, etc.). The goal of this retrospective study was to review a personal series of gastrointestinal carcinoid tumors, and to compare it to those in the literature.
PATIENTS AND METHODS
the medical records of 40 Caucasian patients with over 50 gastrointestinal carcinoid tumors (including multiple cases) who were seen for a period of 16 years (1994-2009) were reviewed.
RESULTS
mean age at presentation was 52 years, 50% were females, and mean tumor size was 9.9 mm. Most were gastroduodenal (42.5%) or rectal (30%), and were treated endoscopically. Metastases and carcinoid syndrome (CS) were seen in 5% of patients. Survival at study endpoint was 85%.
CONCLUSIONS
age and gender were consistent with the literature. There was an increase in gastroduodenal (multifocal) and rectal carcinoids, likely because the series was essentially endoscopical in nature (bias). There was a lower rate of CS and higher survival, likely due to earlier diagnosis and treatment.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carcinoid Tumor; Female; Gastrointestinal Neoplasms; Humans; Male; Middle Aged; Retrospective Studies; Young Adult
PubMed: 20883069
DOI: 10.4321/s1130-01082010000900004 -
The American Journal of Case Reports Apr 2022BACKGROUND Carcinoid tumors are well-differentiated tumors that develop from neuroendocrine cells. They are rare tumors and occur most commonly in the gastrointestinal...
BACKGROUND Carcinoid tumors are well-differentiated tumors that develop from neuroendocrine cells. They are rare tumors and occur most commonly in the gastrointestinal tract, followed by the pulmonary system. They usually present with abdominal pain or cough or persistent pneumonia. They are usually diagnosed with computed tomography (CT) or magnetic resonance imaging (MRI) of the chest or abdomen. The cornerstone of treatment is surgical resection. There are rare reported cases of carcinoid tumor presenting in the pancreas as gastrinoma or insulinoma and are associated with multiple endocrine neoplasia type 1 (MEN1). CASE REPORT We report a case of an otherwise healthy 9-year-old girl who presented with manifestations of Cushing syndrome (easy fatigability, weakness, back pain, easy bruising, hirsutism, acne, skin discoloration [pigmentation], and blurred vision). She was diagnosed with incidental carcinoid tumor in the pancreas based on hypertension and typical stigmata. She underwent distal pancreatectomy and splenectomy. The histopathology showed a well-differentiated neuroendocrine tumor with G2. The diagnosis of concurrent Cushing syndrome and carcinoid syndrome can be challenging, as it is rare. It is important to screen for MEN syndrome when Cushing syndrome occurs in a child, as there is a high rate of transition to malignancy. They are usually diagnosed with ACTH, cortisol, and imaging. CONCLUSIONS Incidental hypertension in children is not common and mandates further investigation and clinical work-up to look for endocrinopathies such as Cushing syndrome and carcinoid syndrome. As the literature on such cases is scant, further reporting of cases is needed.
Topics: Carcinoid Tumor; Child; Cushing Syndrome; Female; Humans; Hypertension; Multiple Endocrine Neoplasia Type 1; Pancreas; Pancreatic Neoplasms
PubMed: 35393387
DOI: 10.12659/AJCR.936029