Carcinoid

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Carcinoid
Classification and external resources
Template:Px Picture of a carcinoid tumor that encroaches into lumen of the small bowel (centre of image). Pathology specimen. The prominent folds are plicae circulares, a characteristic of small bowel.
ICD-10	C75, E340
ICD-9	209.60
ICD-O:	M8240/3
OMIM	114900
DiseasesDB	2040
MedlinePlus	000347
eMedicine	med/271
MeSH	D002276

File:Carcinoid cancer.jpg

Primary site of a carcinoid cancer of gut

Carcinoid (also carcinoid tumor) is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system.

In 2000, the World Health Organization redefined "carcinoid", but this new definition has not been accepted by all practitioners. This has led to some complexity in distinguishing between carcinoid and other neuroendocrine tumors in the literature. According to the American Cancer Society, the 2000 WHO definition states:

The WHO now divides these growths into neuroendocrine tumors and neuroendocrine cancers. Neuroendocrine tumors are growths that look benign but that might possibly be able to spread to other parts of the body. Neuroendocrine cancers are abnormal growths of neuroendocrine cells which can spread to other parts of the body.

Carcinoid tumors have more benign features than neuroendocrine cancers.

Presentation

They are most commonly found in the midgut at the level of the ileum (75 – 87% cases). The next most common affected area is the respiratory tract with 28% cases of all cases — per PAN-SEER data (1973 – 1999).

Gastrointestinal

Carcinoid tumors are apudomas that arise from the enterochromaffin cells throughout the gut. Over two-thirds of carcinoid tumors are found in the gastrointestinal tract. Some sources list the appendix as the most common site.

Lung

Main article: Typical lung carcinoid tumor

Carcinoid tumors are also found in the lungs.

Other sites / metastases

Carcinoid metastasis can lead to carcinoid syndrome. This is due to the over-production of many substances, including serotonin, which is released into the systemic circulation, and which can lead to symptoms of cutaneous flushing, diarrhea, bronchoconstriction, and right-sided cardiac valve disease. It is estimated that less than 10% of carcinoid patients will develop carcinoid syndrome.

History

They were first characterized in 1907 by Siegfried Oberndorfer, a German pathologist at the University of Munich, who coined the term karzinoide, or "carcinoma-like", to describe the unique feature of behaving like a benign tumor despite having a malignant appearance microscopically. The recognition of their endocrine-related properties were later described by Gosset and Masson in 1914, and these tumors are now known to arise from the enterochromaffin (EC) and enterochromaffin-like (ECL) cells of the gut.

Some sources credit Otto Lubarsch with the discovery.

Symptoms

Most carcinoids are asymptomatic through the natural lifetime and are discovered only upon surgery for unrelated reasons; these are called coincidental carcinoids. But all carcinoids are considered to have malignant potential.

About 10% of carcinoids secrete excessive levels of a range of hormones, most notably serotonin (5-HT), causing:

• Flushing
• Diarrhea
• Wheezing
• Abdominal cramping
• Peripheral edema

The outflow of serotonin can cause a depletion of tryptophan leading to niacin deficiency. Niacin deficiency, aka pellagra, is associated with dermatitis, dementia, and diarrhea.

This constellation of symptoms is called carcinoid syndrome or (if acute) carcinoid crisis. Occasionally, haemorrhage or the effects of tumor bulk are the presenting symptoms. The most common originating sites of carcinoid is the small bowel, particularly the ileum; carcinoid tumors are the most common malignancy of the appendix. Carcinoid tumors may rarely arise from the ovary or thymus.

Treatment

Surgery, if feasible, is the only curative therapy. If the tumor has metastasized (most commonly, to the liver) and is considered incurable, there are some promising treatment modalities, such as radiolabeled octreotide^{[How to reference and link to summary or text]} (e.g. Lutetium (¹⁷⁷Lu) DOTA-octreotate) or the radiopharmaceutical 131I-mIBG (meta iodo benzyl guanidine,^{[citation needed]}, for arresting the growth of the tumors and prolonging survival in patients with liver metastases, though these are currently experimental.

Chemotherapy is of little benefit and is generally not indicated. Octreotide or Lanreotide (somatostatin analogues) may decrease the secretory activity of the carcinoid, and may also have an anti-proliferative effect. Interferon treatment is also effective, and usually combined with somatostatin analogues.

While the metastatic potential of a coincidental carcinoid is probably low, the current recommendation is for follow up in 3 months with CT or MRI, labs for tumor markers, like serotonin, and a history and physical, annual physicals thereafter.

Carcinoid tumors are the most common malignant tumor of the appendix, but they are most commonly associated with the small intestine, and they can also be found in the rectum and stomach. They are known to grow in the liver, but this finding is usually a manifestation of metastatic disease from a primary carcinoid occurring elsewhere in the body. They have a very slow growth rate compared to most malignant tumors.

Goblet cell carcinoid

Main article: Goblet cell carcinoid

This is considered to be a hybrid between an exocrine and endocrine tumor derived from crypt cells of the appendix. Histologically, it forms clusters of goblet cells containing mucin with a minor admixture of Paneth cells and endocrine cells. The growth pattern is distinctive: typically producing a concentric band of tumor nests interspersed among the muscle and stroma of the appendiceal wall extending up the shaft of the appendix. This makes the lesion difficult to suspect grossly and difficult to measure. Small tumor nests may be camouflaged amongst the muscle or in periappendiceal fat; cytokeratin preparations best demonstrate the tumor cells; mucin stains are also helpful in identifying them. They behave in a more aggressive manner than do classical appendiceal carcinoids. Spread is usually to regional lymph nodes, peritoneum, and particularly the ovary. They do not produce sufficient hormonal substances to cause the carcinoid or other endocrine syndromes. In fact, they more closely resemble exocrine than endocrine tumors. The term 'crypt cell carcinoma' has been used for them, and though perhaps more accurate than considering them carcinoids, has not been a successful competitor. The ICD-10 code for goblet cell carcinoid is 8243/3.^{[citation needed]}

See also

• Carcinoid syndrome

• Don Meyer Head coach emeritus of the Northern State University men's basketball team. Professor Meyer found to have carcinoid cancer following an automobile accident in September 2009.

• Derrick Bell, Professor and Legal Scholar, died of carcinoid cancer October 5, 2011.

References

Cunningham JL, Janson ET (2011). The Hallmarks of Ileal Carcinoids. Eur J Clin Invest 41 (12): 1353–60.

External links

• The Carcinoid Cancer Foundation
• Uppsala Centre of Excellence in Neuroendocrine Tumors
• European Neuroendocrine Tumor Society
• NET Patient Foundation
• The Unicorn Foundation
• Caring for Carcinoid Foundation
• NETipedia
• Carcinoid Cancer Community
• Cancer.Net: Carcinoid Tumor
• CarcinoidLink

Template:Epithelial neoplasms Template:Digestive system neoplasia Template:Respiratory and intrathoracic neoplasia