Bronchial Typical Carcinoid Tumors
Section snippets
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
References (37)
- et al.
Neuroendocrine tumors of the bronchopulmonary tract: a reappraisal of their classification after 20 years
Surg Clin North Am
(2002) - et al.
for the EUROCARE Working Group: Survival from rare cancer in adults
Lancet Oncol
(2006) - et al.
The p16/cyclin D1/Rb pathway in neuroendocrine tumors of the lung
Hum Pathol
(2003) - et al.
Giant bronchial carcinoid tumors: a multidisciplinary approach
Ann Thoracic Surg
(2001) - et al.
Management of ectopic ACTH syndrome due to thoracic carcinoids
Ann Thoracic Surg
(1990) - et al.
Bronchopulmonary carcinoid tumors associated with Cushing’s Syndrome: a more aggressive variants of typical carcinoid
J Thoracic Cardiovasc Surg
(1997) - et al.
Pulmonary carcinoid tumors with Cushing’s syndrome: an aggressive variant or not?
Ann Thoracic Surg
(2005) - et al.
Acromegaly caused by ectopic growth hormone-releasing hormone secretion by a carcinoid bronchial tumor: a rare entity
J Thoracic Cardiovasc Surg
(2004) - et al.
Intraoperative detection of a bronchial carcinoid with a radiolabeled somatostatin analog
Chest
(2002) - et al.
High-resolution CT in patients with intraluminal typical bronchial carcinoid tumors treated with bronchoscopic therapy
Chest
(2000)