Carcinoid Lung Tumors

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Carcinoid Lung Tumors
Author: Mary C Mancini, MD, PhD; Chief Editor: Jeffrey C Milliken, MD more... Updated: Dec 5, 2011

Background
Carcinoid tumors of the lung are a fascinating but uncommon group of pulmonary neoplasms. In the past, these tumors were grouped with benign or less aggressive malignant pulmonary tumors. Together they were grouped as a category of neoplasms called bronchial adenomas. This unfortunate label, still used by many today, creates the impression that such tumors are benign neoplasms. Recent study has revealed that carcinoid lung tumors represent the most indolent form of a spectrum of bronchopulmonary neuroendocrine tumors that includes small cell carcinoma of the lung as its most malignant member and several other forms of intermediately aggressive tumors, such as atypical carcinoid.

Posteroanterior chest radiograph of a 37-year-old woman with a carcinoid lung tumor of the left mainstem bronchus and resultant left upper lobe atelectasis.

History of the Procedure
Laennec's description of an intrabronchial mass in 1831 was the first written description of what was likely a bronchial carcinoid tumor. Mueller described the first so-called bronchial adenoma in detail 1882. This probably was a carcinoid tumor because the patient was young and had symptoms of cough with hemoptysis for 8 years. In 1914, Chevalier Jackson performed bronchoscopic resection of a large intrabronchial tumor. At that time, the tumor was labeled with the pathologic diagnosis of endothelioma, but, after reexamination a number of years later, it was

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2 of 8 12/10/2012 10:57 . the idea that a spectrum of neuroendocrine tumors of the bronchopulmonary tree exists has become more accepted. These tumors characteristically grow slowly and tend to metastasize infrequently. arising from stem cells of the bronchial epithelium known as Kulchitsky cells. and Thorsen in 1952 described the first report of carcinoid syndrome and its relation to metastatic carcinoid tumor. In 1939. C. see eMedicine Oncology article Carcinoid Tumor. Since this study. Atypical carcinoid tumors have a more aggressive histologic and clinical picture. In 1944. Ten to 15% of tumors arise in a mainstem bronchus. carry a worse prognosis. Atypical carcinoid tumors appear in slightly older people than typical carcinoids do. The average age of people at occurrence of typical carcinoid tumors is 40-50 years.Carcinoid Lung Tumors http://emedicine. Alexander and Weller classified bronchial adenomas as grade 1 malignancies and reported that they had observed metastases in 2 of 13 cases. Intestinal. Gunter. however. Arrigoni and associates reported a subset of pulmonary carcinoid tumors that had an atypical histologic appearance and behaved more aggressively. it was associated with an intestinal carcinoid tumor.L.medscape. Jackson reported on 12 and 20 cases of bronchial adenomas.) Endocrine syndromes found in association with small cell carcinoma of the lung are found less commonly with carcinoid tumors of the lung. In 1937 and again in 1945. Eloesser performed the first bronchotomy for resection of a bronchial tumor. In 1972. began to question the apparent benign nature of these tumors. Bjork. much less frequently than in cases associated with gastrointestinal carcinoid tumors. The first report of this syndrome in association with a bronchial carcinoid tumor was by Stanford. It is noted much less frequently in association with carcinoids of pulmonary origin than those originating within the gastrointestinal tract. One to 6% of all lung tumors are carcinoid tumors. Eighty to 90% of tumors develop within a bronchus of subsegmental size or greater. The tumor was called a benign carcinoma at the time but was likely a carcinoid or cylindroma. (For more information. Carcinoid syndrome occurs in about 2% of cases of pulmonary carcinoid tumors. Carcinoid tumors occur in equal numbers of males and females.[1] Problem Typical carcinoid tumors of the lung represent the most well differentiated and least biologically aggressive type of pulmonary neuroendocrine tumor.[2] Etiology In the past. and Hobart in 1958.com/article/426400-overview determined to be an adenoma. they currently are understood to be of endodermal origin. pulmonary carcinoid tumors were believed to be derived from neural crest cells. They metastasize at a considerably higher rate than do typical carcinoid tumors and. however. Other experts. Axen. Epidemiology Frequency The gastrointestinal tract is the most common area in which carcinoid tumors arise. however. some endocrine abnormalities have been attributed to both typical and atypical pulmonary carcinoid tumors. however. He stated that none of these exhibited signs of metastases or any other evidence of malignant activity. Davis. but typical carcinoid tumors have been reported in virtually every age group. Bronchopulmonary carcinoid tumors are reported to represent about 10% of all carcinoid tumors. They named this group of tumors atypical carcinoid and reported that the tumors often were larger at presentation and developed distant metastases in as many as 70% of cases. therefore. they rarely appear in the trachea. Atypical carcinoid tumors comprise about 10% of all pulmonary carcinoid tumors. Ten to 20% of tumors are located in the pulmonary periphery. Carcinoid syndrome has been reported in association with very large bronchopulmonary carcinoid tumors or in the presence of metastatic disease. respectively.

At least 50% of pulmonary atypical carcinoid tumors present in the periphery of the lung. If large enough. In addition. producing hyperinflation in the pulmonary parenchyma distal to the tumor. and bronchiectasis. and others. carcinoids are capable of producing a variety of biologically active peptides and hormones. no external environmental toxin or other stimulus has been identified as a causative agent for the development of pulmonary carcinoid tumors. such as 3 of 8 12/10/2012 10:57 . Carcinoids characteristically are vascular tumors and can bleed secondary to bronchial irritation. Presentation About 25% of patients with pulmonary carcinoid tumors are asymptomatic at the time of discovery. especially large ones. Atypical carcinoid tumors can present in the same locations as typical carcinoids. Pathophysiology Local pathophysiology Twenty-five to 39% of patients with a carcinoid pulmonary tumor are asymptomatic. and acidosis.medscape. including serotonin. weakness. In symptomatic patients. Carcinoids developing within large airway structures grow slowly and can become quite large. lethargy.Carcinoid Lung Tumors http://emedicine. the lesion is generally a pulmonary neoplasm of some type. melanocytestimulating hormone (MSH). however. but they occur more commonly as peripheral lesions. This syndrome characteristically occurs in the presence of metastatic disease to the liver. pulmonary abscess.com/article/426400-overview Although these neoplasms are capable of producing a variety of substances. bronchoconstriction. this latter form can create a ball-valve mechanism within the bronchus. these central carcinoids can cause bronchial obstruction. flushing. bronchial carcinoid tumors. can develop convulsions and coma. in severe cases. and is reported in 2-12% of patients with bronchial carcinoid tumors. All of the sequelae resulting from bronchial obstruction can follow. although it more commonly is associated with small cell lung carcinoma. This syndrome is characterized by a constellation of symptoms. and mental confusion and. these tumors are responsible for the development of about 1% of cases of Cushing syndrome. The production of excess circulating AVP creates hyponatremia secondary to water retention. the most common clinical findings are those associated with bronchial obstruction. Ectopic production of ACTH and Cushing syndrome have been reported in association with typical and atypical carcinoid tumors. it is the second most common neuroendocrine syndrome produced by these tumors. Because of their location and size. a few present on a mobile stalk and have a polypoid appearance. hemodynamic instability. most are inactive.[2] Systemic pathophysiology As neuroendocrine tumors. including biologically active peptides and hormones. Unlike carcinoma of the lung. When a patient is found to have an ectopic source of ACTH production. They are one of the differential diagnoses considered in evaluation of a solitary pulmonary nodule. diarrhea. Patients present with weight gain. The vast majority of symptomatic patients have symptoms directly involving the bronchopulmonary tree. recurrent pneumonia. are capable of producing the syndrome in the absence of metastatic disease. Although most tumors are broad-based intrabronchial lesions. Excess serotonin production has been implicated in the development of carcinoid syndrome. The syndrome of inappropriate AVP (arginine vasopressin) secretion or syndrome of inappropriate secretion of ADH (SIADH) can be produced by pulmonary carcinoid tumors. They have a more aggressive nature and a greater tendency to metastasize. adrenocorticotropin hormone (ACTH). Although less than 1% of pulmonary carcinoid tumors produce Cushing syndrome. including persistent atelectasis. antidiuretic hormone (ADH). Peripheral pulmonary carcinoid tumors most often are asymptomatic and usually are discovered incidentally. including tachycardia.

and recurrent or obstructive pneumonitis. Tumors usually are soft masses covered with intact bronchial epithelium. and dyspnea also may be noted. has been utilized with good long-term results. Tumors usually are attached to the bronchus by a broad base. or pneumonectomy. Palliation. Appropriate lymph node dissection also should be performed in these cases. Because surgical resection is the only treatment known to achieve cure. may indicate a more aggressive tumor and a poorer prognosis. Complete tumor removal is extremely unlikely using this method because these obstructing intrabronchial tumors usually have penetrated the bronchus and invaded the local pulmonary parenchyma by the time they are discovered. Tumorlets may represent local metastatic disease or an entirely different histologic abnormality and. Carcinoid syndrome. and a general feeling of ill health. is the goal of this technique. segmentectomy. Carcinoid syndrome is observed most commonly when metastatic disease to the liver is present. Wheezing. Incomplete specimen analysis may have significant bearing on prognostic determination because the histologic features of pulmonary carcinoid tumors must be scrutinized carefully in order to determine whether typical or atypical carcinoid is present. a variety of parenchymal-sparing bronchoplastic procedures. Transbronchial photocoagulation destroys at least a portion of the resected tumor and thwarts thorough analysis of a completely resected specimen. the presence of metastatic disease can produce weight loss.Carcinoid Lung Tumors http://emedicine. not cure. they are polypoid with a distinct stalk. These include preoperative management of symptomatic bronchial obstruction prior to formal resection and palliative treatment in patients who would otherwise not tolerate formal pulmonary resection. Tumors are very vascular and pink to purplish in color. various endocrine or neuroendocrine syndromes can be initial clinical manifestations of either typical or atypical pulmonary carcinoid tumors. Tumors may be associated with the presence of tumorlets. Thoracoscopic or open wedge resection of a peripheral carcinoid tumor should be reserved for patients with limited pulmonary reserve who cannot tolerate anatomic resection.com/article/426400-overview persistent cough. hemoptysis. Resection of distant metastatic lesions is indicated in a select group of patients in whom thorough evaluation has revealed isolated lesions in areas amenable to resection. all pulmonary carcinoid tumors without evidence of distant metastatic disease should be resected completely as long as no contraindication to surgery exists. Total resection should be the primary goal of any form of surgical therapy. but. weakness. chest pain. In addition.medscape. inappropriate secretion of ADH. hypercortisolism and Cushing syndrome. when present. 4 of 8 12/10/2012 10:57 . Patients with marginal pulmonary reserve may be good candidates for complete resection and cure if a bronchoplastic or parenchymal-sparing procedure can be performed. lymph node staging cannot be accomplished. Its limitations are similar to those of bronchoscopic resection with one additional drawback. occasionally. Lymph node dissection should accompany resection. and ectopic insulin production resulting in hypoglycemia are some of the endocrinopathies that can be produced by a pulmonary carcinoid tumor in a patient who is otherwise asymptomatic. increased pigmentation secondary to excess MSH. Relevant Anatomy Gross anatomic features of carcinoid tumors Tumors most commonly are found within the cartilaginous portion of the tracheobronchial tree. Neodymium:yttrium-aluminum-garnet (Nd:YAG) laser photoresection of intrabronchial carcinoid tumors also has been proposed. including sleeve resections. Indications All pulmonary carcinoid tumors should be treated as malignancies. Bronchoscopic resection of an intrabronchial carcinoid tumor only is recommended in selected cases. This form of therapy should not be considered primary and should be reserved for the same types of cases indicated for bronchoscopic resection. While the most commonly used procedures are formal lobectomy. In cases of malignancy. which are small foci of atypical hyperplastic bronchial epithelium in adjacent locations. Although uncommon.

American College of Surgeons. and Southern Surgical Association Disclosure: Nothing to disclose. Phi Beta Kappa. MD. PhD is a member of the following medical societies: American Association for Thoracic Surgery. PhD Professor and Chief of Cardiothoracic Surgery.medscape. University of Utah School of Medicine and Medical Center 5 of 8 12/10/2012 10:57 . Editor-in-Chief. Division of Cardiothoracic Surgery. Specialty Editor Board Francisco Talavera.Carcinoid Lung Tumors http://emedicine.com/article/426400-overview Contraindications Formal resection of carcinoid tumors of the lung only is contraindicated in patients who would not otherwise tolerate the operative procedure or who are found to have widespread metastatic disease. PhD Adjunct Assistant Professor. Department of Surgery. University of Nebraska Medical Center College of Pharmacy. Contributor Information and Disclosures Author Mary C Mancini. Medscape Drug Reference Disclosure: Medscape Salary Employment Shreekanth V Karwande. Professor. Society of Thoracic Surgeons. Louisiana State University School of Medicine in Shreveport Mary C Mancini. MBBS Chair. Department of Surgery. PharmD. American Surgical Association. MD.

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