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Carcinoid Tumors RUSSELL G Carcinoid Tumors RUSSELL G. ROBERTSON, M.D., Northwestern University, Chicago, Illinois WILLIAM J. GEIGER, M.D., and NANCY B. DAVIS, M.D. Medical College of Wisconsin, Milwaukee, Wisconsin 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 carci- noid 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 divi- sions 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 associ- ated 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. (Am Fam Physician 2006;74:429-34. Copyright © 2006 American Academy of Family Physicians.) arcinoid tumors are rare, slow- greater than 2 percent but less than 15 per- growing tumors that originate in cent); poorly differentiated endocrine car- the cells of the neuroendocrine sys- cinoma (PI greater than 15 percent); mixed tem. The nerve cells rarely undergo exocrine-endocrine tumors; and tumor-like C hyperplasia or neoplastic transformation, lesions. Table 12,4,5 provides an overview of whereas cells in the endocrine glands and in the incidence rate, average age at onset, asso- disseminated sites in the mucous membranes ciated symptoms, likelihood of metastasis, and skin may undergo a transformation com- and commonness of carcinoid syndrome monly known as carcinoid tumors.1 There for carcinoid tumors in different locations are three main areas of origin for carcinoid of the body. tumors: foregut carcinoid tumors start in the Carcinoid tumors pose a diagnostic chal- lungs, bronchi, or stomach; midgut carcinoid lenge because they often are asymptomatic. tumors start in the small intestine, appendix, The overall prevalence of carcinoid tumors or proximal large bowel; and hindgut carci- in the United States is estimated to be one noid tumors start in the distal colon or rec- to two cases per 100,000 persons.3,4 Because tum.2 The appendix is the most common site many carcinoid tumors are indolent, their of carcinoid tumors, followed by the rectum, true prevalence may be higher.5 Data derived ileum, lungs, bronchi, and stomach.3 from a five-decade analysis of 13,715 carci- Historically, the classification and distribu- noid tumors revealed an overall increase in tion of carcinoid tumors has been according incidence over the past 30 years, with 67.2 per- to their derivation from different embry- cent of patients having a five-year survival rate onic divisions of the gut. More recently, regardless of the site of the tumor.2 Prognosis the World Health Organization varies widely depending on the location and presented new classifications of stage of the tumor.1,2 The disease is considered There has been an overall gastroenteropancreatic neuro- to be more aggressive and to have a worse increase in incidence of car- endocrine neoplasms1 based on prognosis than was thought previously.2 cinoid tumors over the past their malignant potential: well- 30 years, with 67.2 percent differentiated endocrine tumor Clinical Presentation of patients having a five- (proliferation index [PI] less Many carcinoid tumors are found during year survival rate. than 2 percent); well-differen- surgery for other reasons, usually at appen- tiated endocrine carcinoma (PI dectomy or surgery for acute pancreatitis, Downloaded from the American Family Physician Web site at www.aafp.org/afp. Copyright © 2006 American Academy of Family Physicians. For the private, noncommercial use of one individual user of the Web site. All other rights reserved. Contact [email protected] for copyright questions and/or permission requests. SORT: KEY RECOMMENDATIONS FOR PRACTICE Evidence Clinical recommendation rating References 5-Hydroxyindoleacetic acid and serum chromogranin A are C 13, 17 recommended as initial tests for patients with vasoactive symptoms and suspected carcinoid tumors. A multimodal approach is recommended for imaging and may include C 13 CT, MRI, somatostatin receptor scintography using indium-111 labeled octreotide, and endoscopic ultrasonography. Patients with carcinoid tumors of the gastrointestinal tract should be C 21, 22 evaluated for second primary malignancy. CT = computed tomography; MRI = magnetic resonance imaging. A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evi- dence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, see page 363 or http://www.aafp.org/afpsort.xml. but also surgery for bowel obstruction or CARCINOID SYNDROME diseases of the female reproductive tract.6,7 A carcinoid tumor often is only considered If there are symptoms, they usually are after the onset of carcinoid syndrome, which vague, nonspecific, and organ-related, typically does not occur until the tumor has causing relatively long delays in diagnosis. metastasized to the lungs or liver.9 The symp- The average time from symptom onset toms of carcinoid syndrome include flushing to diagnosis is more than nine years.8 (pale, purplish, or red), diarrhea (watery Symptoms from the tumor can range from and explosive), tachycardia or hypotension, mild abdominal discomfort to intermit- bronchospasm, telangiectasia, and right- tent intestinal obstruction.9 Occasionally, sided heart disease or failure.1,9,10 Symptoms a carcinoid tumor can be the lead point for often are precipitated by exertion or by an intermittent intestinal intussuception.9 eating or drinking (especially items high in These tumors characteristically present at tyramine [e.g., blue cheeses, chocolate] or age 50 to 60.8 ethanol [e.g., red wine]).1,9,10 TABLE 1 Characteristics of Carcinoid Tumors by Location Approximate age Carcinoid Location Percent at presentation Symptoms Metastasis at diagnosis syndrome (%) Rectum 26 60 Rectal bleeding, pain, Tumor size < 1 cm: < 5 constipation 5 percent Tumor size > 2 cm: majority Small intestine 25 60 to 70 Abdominal pain, small bowel Majority present with 5 to 7 obstruction metastasis, usually to lymph nodes or liver Lungs, bronchi, 23 50 Recurrent pneumonia, cough, < 15 percent < 5 and trachea hemoptysis, chest pain Appendix 12 40 to 50 Appendicitis caused by tumor < 5 percent < 5 presence; incidental discovery during other pelvic procedures Stomach 7 60 to 70 Anemia, abdominal pain < 10 percent 5 to 10; also Zollinger-Ellison syndrome Colon 7 70 Pain, anorexia, weight loss > 66 percent < 5 Information from references 2, 4, and 5. 430 American Family Physician www.aafp.org/afp Volume 74, Number 3 ◆ August 1, 2006 Carcinoid Tumors Carcinoid tumors contain many neuro- Diagnosis secretory granules that are capable of the Although a significant percentage of car- synthesis, storage, and release of substances, cinoid tumors are innocuous at the time including serotonin, histamine, prostaglan- of presentation, there are useful diagnostic dins, kallikrein, bradykinins, substance P, techniques that facilitate identification and gastrin, corticotrophin, and neuron-specific localization. Because the presentation varies enolase.9,11 The most prominent of these with the embryologic origin of the tumor, substances is serotonin (i.e., 5-hydroxytryp- there are differing approaches to the diagno- tamine). Degradation of 5-hydroxytrypta- sis. The indolent nature of carcinoid tumors mine results in 5-hydroxyindoleacetic acid makes test selection complex. For patients (5-HIAA), which is excreted in the urine. with vasoactive symptoms, measuring the When released in the systemic circulation, urinary excretion of 5-HIAA and serum 5-hydroxytryptamine can result in the chromogranin A level is recommended.13 For symptoms of carcinoid syndrome; the exci- those with symptoms of bowel dysmotility tation of smooth muscle leads to increased syndromes, computed tomography (CT) and gastrointestinal motility, bronchoconstric- magnetic resonance imaging (MRI) may be tion, platelet aggregation, or vascular con- helpful and often will note the coincidental striction and dilatation.12 presence of hepatic metastases that may be The lungs and liver metabolize many of the first clue as to the presence of the primary the substances secreted by carcinoid tumors, tumor.9,11 thus preventing their release into the sys- temic circulation until metastases develop.9 URINALYSIS The syndrome is variable: patients may not The biochemical properties of carcinoid have all symptoms, and the symptoms may tumors reflect the presence of neurosecre- vary in intensity and timing.9 Carcinoid tory granules that these tumors share with syndrome occurs in only 10 percent of all other similarly classified tumors referred to patients with carcinoid tumors,8 and it is as APUDomas (Amine Precursor Uptake and most often associated with midgut tumors.5 Decarboxylation).14 Carcinoid tumors are Table 210 is a differential diagnosis of carci- classified as biochemically typical
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