Review

Authors: Tetsuhide Ito, Lingaku Lee, Robert T Jensen...

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

Review

Authors: Russell G Robertson, William J Geiger, Nancy B Davis...

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

Review

Authors: Scott N Pinchot, Kyle Holen, Rebecca S Sippel...

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

Authors: E Baudin, M Caplin, R Garcia-Carbonero...

Topics: Carcinoid Tumor; Follow-Up Studies; Humans; Lung; Lung Neoplasms; Prognosis; Thymus Neoplasms

PubMed: 33482246
DOI: 10.1016/j.annonc.2021.01.003

Review

Authors: S Janmohamed, S R Bloom

The use of long-acting and potent somatostatin analogues is a major advance in the management of carcinoid tumours. In addition to providing effective symptom relief in malignant carcinoid syndrome, octreotide can also be used for diagnostic purposes. Despite its expense, octreotide is the current agent of choice for the treatment of this condition while analogues with different receptor specificities and pharmacokinetics hold promise for the future. Gastric carcinoids have aroused interest because of their experimental association with chronic hypergastrinaemia, a condition now commonplace because of the widespread use of H2-blockers and proton-pump inhibitors. This subject is reviewed. The slow evolution of many tumours demands prolonged follow-up and the active use of a variety of palliative interventions. These include measures such as hepatic and cardiac surgery, which might be deemed inappropriate for patients with other types of metastatic malignancy. Interferons may have a role when first-line treatments have failed. Chemotherapy is, generally, of limited value.

Topics: Animals; Antineoplastic Agents, Hormonal; Carcinoid Heart Disease; Carcinoid Tumor; Female; Humans; Interferons; Liver Neoplasms; Male; Muridae; Octreotide; Paraneoplastic Endocrine Syndromes; Prognosis; Stomach Neoplasms

PubMed: 9156122
DOI: 10.1136/pgmj.73.858.207

Review

Authors: Kenna Koehler, Wade T Iams

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

Review

Authors: Shahnaz D Vellani, Anthony Nigro, Shangari Varatharajan...

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

Authors: M J Varas-Lorenzo, F Muñoz-Agel, J C Espinós-Pérez...

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

Authors: Fabian Kosko, Alicja Dębska-Ślizień, Piotr Skonieczny...

Topics: Humans; Granulomatosis with Polyangiitis; Lung Neoplasms; Lung; Carcinoid Tumor

PubMed: 37818987
DOI: 10.20452/pamw.16582

Authors: Alexandra Chrisoulidou

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