Bronchial Typical Carcinoid Tumors

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The current WHO classification of lung tumors recognizes bronchial typical carcinoid as low-grade neuroendocrine tumors. These tumors grow slowly but can metastasize to regional nodes (4 to 20%) and more rarely to extrathoracic sites. Symptoms are usually related to local compression and obstruction of the bronchial tree. Paraneoplastic syndrome can be present (carcinoid syndrome, Cushing’s syndrome, acromegaly). Preoperative diagnosis is usually obtained with bronchoscopic biopsy. Computed tomography and somatostatin receptor scintigraphy are useful in the preoperative staging. Only selected cases can be treated endoscopically with laser resection. The complete surgical resection remains the only therapy with curative intent in the majority of patients. Parenchyma-sparing resections are indicated whenever possible. Overall survival after surgery is excellent (5-year rate, 87 to 100%) with low recurrence rate (2 to 11%). N-status and type of resection seem not to affect prognosis. Local relapse can be treated successfully with surgery, whereas distant metastases have a poor prognosis even after chemotherapy.

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Epidemiology

Bronchial carcinoids account for 1 to 2% of all lung malignancies. A large population-based study reported a crude incidence in Europe of 0.2 per 100,000/yr.4 Typical carcinoids are three to four times more frequent than AC. An analysis of Surveillance, Epidemiology, and End Results Program of the National Cancer Institute found an increased incidence of pulmonary neuroendocrine tumors in the last decades, especially for TC.5 It is currently not known if this trend is real or if it is only

Pathologic Changes and Molecular Biology

Both central and peripheral TCs consist of a relatively well-demarcated nodule varying in color from yellowish–white to tan–yellow or brown. Peripheral type may have multiple satellite nodules.

According to the current 2004 WHO classification of lung tumors,3 TC consists of a neuroendocrine neoplasm with no more than 1 mitosis per 10 high-power fields (2 mm2) and without necrosis. Organoid growth pattern, tumor proliferation of relatively monomorphic cells with finely granular chromatin nuclei,

Clinical Setting

Patients with TC are often symptomatic and symptoms can be persistent, even for years, before a definitive diagnosis can be achieved. Basically, the symptoms are related to the location of the lesion. Small peripheral TC are usually asymptomatic and incidentally discovered at chest radiograph. Central-located TC produce symptoms as a result of partial or complete bronchial obstruction or secondary to its high vascular supply. Cough, hemoptysis, and recurrent pulmonary infections in the same

Imaging

The chest radiograph is abnormal in most cases of bronchial carcinoid, but in approximately 10% it is negative. Almost 80% are centrally located tumors that usually present with complete or partial atelectasis and more rarely a hilar mass can be revealed at chest radiograph. Peripheral well-defined pulmonary nodule accounts only for 20% of bronchial carcinoid.16

CT scan (Fig. 2) gives an excellent morphological characterization of peripheral and especially centrally located TC that can be purely

Bronchoscopic Management

Bronchoscopy is the procedure of choice to obtain a preoperative histologic diagnosis. Up to 75% of all bronchial carcinoids arise within the orifice of the tertiary bronchi, appearing as a pink/reddish or yellow tumor with a smooth surface (Fig. 4). Cytologic washing or brushing is generally not useful because the overlying bronchial epithelium is normal and deep biopsy is required. For centrally located lesions the diagnostic accuracy reaches 100%. The high vascular tumor supply has led in

Surgical Management

Surgery is the treatment of choice for TC and the only one offering a real chance of cure. The goal of surgical treatment is to remove the whole neoplastic tissue, preserving as much functional lung tissue as possible. The appropriate surgical procedure is related to the location of the neoplasm. For centrally located tumors (main-stem bronchial tumors or tumors involving bronchus intermedius or lobar bronchial structures) bronchoplastic procedures should be considered whenever possible to

Role of Chemotherapy and Radiotherapy

The excellent results after complete resection in TC do not justify adjuvant therapy even when regional nodal involvement occurs. As reported in Table 1 loco-regional and more rarely systemic metastases after surgery are observed in 2 to 11% of patients with TC, even after decades of the primary treatment. The rarity of bronchial carcinoid tumors has prevented the design of prospective trials and few data are available on chemotherapy in relapsed or advanced-stage TC. Bronchial carcinoids are

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