ReviewManagement of Carcinoid Tumors
Section snippets
Classification
Carcinoid tumors are classified by pathologic features as typical carcinoid (TC; < 2 mitoses/2 mm2 of viable tumor) or atypical carcinoid (AC; 2 to 10 mitoses, necrosis, or architectural disruption) [1]. In 1998 the criterion for TC was changed from fewer than 5 to fewer than 2 mitoses/2 mm2 to enhance reproducibility and prognostic differentiation [1]. This resulted in reassignment of about 30% of carcinoids and better 10-year survival (87% vs 73% for TC and 35% vs 9% for AC) [1]. Overall, 16%
Clinical Presentation
Bronchopulmonary carcinoids have a wide age distribution (range, 4 to 86 years) [8, 9], with an equal gender distribution [2]. The late peak of the bimodal distribution is only partly explained by an increase in AC tumors in older patients [10, 11, 12, 13] (Fig 1) [14, 15, 16, 17, 18, 19]. The chance of AC is about 25% in patients aged older than 50 years and less than 10% in patients younger than 30 [14, 15, 16].
One-third of patients are asymptomatic (usually peripheral tumors); cough,
Paraneoplastic Syndromes
Carcinoid syndrome is seen at presentation in only 0.7% of patients (17 of 2496) with a bronchopulmonary carcinoid [9, 14, 16, 17, 18, 19, 20, 21, 22, 23, 27, 28, 29, 30, 31, 32, 33]. During follow-up, this syndrome, which consists of episodic flushing and diarrhea, will develop in another 2% to 5% [14, 28, 20, 34]. These patients almost always have increased urinary levels of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) [20, 35, 36]. Patients with bronchopulmonary carcinoids
Imaging Studies
A chest computed tomography (CT) with intravenous contrast is essential for central tumors to visualize extrabronchial tumor, hilar nodes, adjacent vessels, and to distinguish tumor from atelectatic lung. Peripheral carcinoids are typically homogeneous, rounded, and sharply demarcated (not spiculated), and grow slowly. They usually occur deep within the lung parenchyma, making a wedge resection difficult. Node enlargement by CT has a false-positive rate of 20% to 40%, whereas the false-negative
Diagnostic Tests
Bronchoscopy of a central carcinoid typically reveals a smooth reddish-brown growth, often covered by mucosa. Mucoepidermoid tumors are 10 to 20 times less common but have a similar appearance (sometimes more tan/gray), location (lobar/segmental bronchi), and age distribution (often young adults) [8]. Primary NSCLC or SCLC typically appears irregular and friable.
Biopsy of a carcinoid is safe, with risks similar to bronchoscopy in general [2, 8], despite historical dogma to the contrary. Data on
General Aspects
A clinical diagnosis of carcinoid tumor can be made reliably without a tissue biopsy specimen in most patients with a central tumor (eg, a lobar or segmental endobronchial tumor in a patient aged <40). Only mucoepidermoid tumors have a similar clinical constellation. Fortunately, the evaluation and treatment approach is similar for mucoepidermoid and carcinoid tumors [8].
A clinical diagnosis is more difficult with a peripheral tumor. Peripheral carcinoids are usually round, sharply demarcated,
General Aspects
The mainstay of treatment of carcinoids is surgical resection. For TC tumors, excellent long-term survival is consistently seen (90% or higher survival at 5 and 10 years) [2]. Recurrence occurs in only 3% to 5%, and only 15% of deaths are due to carcinoid tumors [2]. For AC tumors, survival is clearly worse (about 70% at 5 years and 50% at 10 years) 25% experience recurrence, and most deaths are due to recurrence [2]. Almost all recurrences involve distant sites for both TC and AC [2].
Nodal
Endobronchial Resection for Typical Carcinoids
Earlier reports of endobronchial removal of carcinoids demonstrated frequent recurrence (87% of 30 patients with follow-up) [14, 17, 20, 21, 26]. However, a retrospective study in 1995 reported no recurrences in 5 patients (follow-up of 2 to 21 years) and no residual tumor at surgical resection after initial endobronchial removal in 6 patients [60]. In prospective studies, complete resection was achieved in about 50% of selected patients, with recurrence in 5% (5 of 59, median follow-up of 8
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