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Carcinoid Tumor

Updated : June 6, 2022





Background

Carcinoid tumors are slow-growing tumors that originate from neuroendocrine cells and are capable of secreting various peptides and neuroamines. The GI tract and the tracheobronchial tree are the most prevalent sites for developing carcinoid tumors, responsible for 60% and 25% cases respectively. Kidneys and ovaries are also organs which are regularly affected.

Carcinoids are most frequently seen in the small intestine. Typically, the word carcinoid is applied to well-differentiated, low- to intermediate-grade neuroendocrine tumors, whereas neuroendocrine carcinoma is applied to rare, poorly differentiated, high-grade neuroendocrine tumors.

Epidemiology

Over time, the incidence rate of carcinoid tumors has increased as the techniques for detecting these tumors have improved. Between 2.5-5 individuals out of 100,000 develop carcinoid tumors annually, and around 35 per 100,000 cases are prevalent every year.

No specific gender is more susceptible to carcinoid tumors, but individuals between 50-70 years of age display the highest incidence.

Anatomy

Pathophysiology

On the basis of their embryologic origins and the vascular supply of the GI tract, carcinoid tumors are typically classed as foregut, midgut, and hindgut carcinoids. Carcinoids can release a variety of vasoactive chemicals, including serotonin, histamine, tachykinins, and prostaglandins.

Foregut carcinoids can release vasoactive amines directly into the systemic circulation, allowing them to cause carcinoid syndrome in the absence of liver metastases. Carcinoids of the embryonic hindgut are rarely accompanied with a hormonal condition.

Etiology

Carcinoid tumors emerge from enterochromaffin cells of the aerodigestive tract and they have an endodermal origin. Most Gastrointestinal carcinoids arise in the small intestine, and the rectum, stomach, and appendix are also common sites.

It has been hypothesized that paracrine drugs and growth factors stimulate cell proliferation and provide a favorable environment for alterations in oncogenes and tumor suppressor genes.

Genetics

No genetic basis has been found for patients with carcinoid tumors, but some patients of familial MEN1 disease have a slightly higher risk of developing carcinoid tumors than the average individual.

Prognostic Factors

There isn’t much information available about prognostic factors in patients with carcinoid tumors. One study conducted in 1997 called, “Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center” identified two factors which suggested a poor prognosis in patients with carcinoid tumors in their midgut.

Older age was the first factor, but advanced age is nevertheless associated with lower overall survival. The other prognostic marker was plasma chromogranin A level below 5000 micrograms/l.

Based on the SEER database, information is available about the 5-year survival rates of patients with grade I or II gastrointestinal carcinoid tumors from between 2011-2017.

The figures are as follows:

  • Localized: 97%
  • Regional: 95%
  • Distant: 66%

Although the 5-year survival rate of distant carcinoid tumors is considerably lower than regional or localized tumors, the overall 5-year survival rate for patients with carcinoid tumors in stage I or II is 94%.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/carcinoid-tumor

https://www.ncbi.nlm.nih.gov/books/NBK448101/

https://pubmed.ncbi.nlm.nih.gov/9296223/

https://www.cancer.org/cancer/gastrointestinal-carcinoid-tumor/detection-diagnosis-staging/survival-rates.html

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Carcinoid Tumor

Updated : June 6, 2022




Carcinoid tumors are slow-growing tumors that originate from neuroendocrine cells and are capable of secreting various peptides and neuroamines. The GI tract and the tracheobronchial tree are the most prevalent sites for developing carcinoid tumors, responsible for 60% and 25% cases respectively. Kidneys and ovaries are also organs which are regularly affected.

Carcinoids are most frequently seen in the small intestine. Typically, the word carcinoid is applied to well-differentiated, low- to intermediate-grade neuroendocrine tumors, whereas neuroendocrine carcinoma is applied to rare, poorly differentiated, high-grade neuroendocrine tumors.

Over time, the incidence rate of carcinoid tumors has increased as the techniques for detecting these tumors have improved. Between 2.5-5 individuals out of 100,000 develop carcinoid tumors annually, and around 35 per 100,000 cases are prevalent every year.

No specific gender is more susceptible to carcinoid tumors, but individuals between 50-70 years of age display the highest incidence.

On the basis of their embryologic origins and the vascular supply of the GI tract, carcinoid tumors are typically classed as foregut, midgut, and hindgut carcinoids. Carcinoids can release a variety of vasoactive chemicals, including serotonin, histamine, tachykinins, and prostaglandins.

Foregut carcinoids can release vasoactive amines directly into the systemic circulation, allowing them to cause carcinoid syndrome in the absence of liver metastases. Carcinoids of the embryonic hindgut are rarely accompanied with a hormonal condition.

Carcinoid tumors emerge from enterochromaffin cells of the aerodigestive tract and they have an endodermal origin. Most Gastrointestinal carcinoids arise in the small intestine, and the rectum, stomach, and appendix are also common sites.

It has been hypothesized that paracrine drugs and growth factors stimulate cell proliferation and provide a favorable environment for alterations in oncogenes and tumor suppressor genes.

No genetic basis has been found for patients with carcinoid tumors, but some patients of familial MEN1 disease have a slightly higher risk of developing carcinoid tumors than the average individual.

There isn’t much information available about prognostic factors in patients with carcinoid tumors. One study conducted in 1997 called, “Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center” identified two factors which suggested a poor prognosis in patients with carcinoid tumors in their midgut.

Older age was the first factor, but advanced age is nevertheless associated with lower overall survival. The other prognostic marker was plasma chromogranin A level below 5000 micrograms/l.

Based on the SEER database, information is available about the 5-year survival rates of patients with grade I or II gastrointestinal carcinoid tumors from between 2011-2017.

The figures are as follows:

  • Localized: 97%
  • Regional: 95%
  • Distant: 66%

Although the 5-year survival rate of distant carcinoid tumors is considerably lower than regional or localized tumors, the overall 5-year survival rate for patients with carcinoid tumors in stage I or II is 94%.

https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/carcinoid-tumor

https://www.ncbi.nlm.nih.gov/books/NBK448101/

https://pubmed.ncbi.nlm.nih.gov/9296223/

https://www.cancer.org/cancer/gastrointestinal-carcinoid-tumor/detection-diagnosis-staging/survival-rates.html

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