Author(s)

Sara Pusceddu, Milena Vitali, Eva Haspinger, Luca Tavecchio, Riccardo Giovannetti, Andrea Bille, Laura Concas, Marina Garassino, Massimo Milione, Filippo de Braud, Roberto Buzzoni

Medical Oncology Unit, Fondazione IRCCS—Istituto Nazionale dei Tumori, Milan, Italy.
Pathology Unit, Fondazione IRCCS—Istituto Nazionale dei Tumori, Milan, Italy.
Thoracic Surgery Unit, Fondazione IRCCS—Istituto Nazionale dei Tumori, Milan, Italy.

An estimated 25% to 30% of all neuroendocrine tumors (NETs) have their origin in the bronchial tree and into the lungs. Although lung NETs account for less than 1% of all pulmonary malignancies, the incidence of these neoplasms has risen precipitously since the mid 1970s. Currently, according to the 2004 World Health Organization categorization, these tumors are separated into 4 subtypes characterized by increasing biologic aggressiveness: low-grade typical carcinoid (TC), intermediate-grade atypical carcinoid (AC), high-grade large-cell neuroendocrine carcinoma (LCNEC) and small-cell carcinoma (SCLC). Surgery is the treatment of choice for typical and atypical carcinoid lung NETs with loco-regional disease. At diagnosis up to 64% of patients with atypical carcinoid lung NETs present with lymph node metastases, and 5-year survival ranges from 61% to 88%. In contrast, lymph node metastases are present in fewer than 15% of typical carcinoid lung NETs, and 5-year survival exceeds 90%. To date, there is no recognized standard of treatment for advanced carcinoid lung NETs. In recent years only two trials reported intriguing results regarding lung NETs: a phase 2 retrospective study of dacarbazine derivative temozolomide and the phase 3, RADIANT-2 trial in advanced NETs. Successful management requires a multidisciplinary team management. This review is restricted to typical/atypical NETs.

Bronchial; Pulmonary; Carcinoids; Neuroendocrine Tumours

S. Pusceddu, M. Vitali, E. Haspinger, L. Tavecchio, R. Giovannetti, A. Bille, L. Concas, M. Garassino, M. Milione, F. Braud and R. Buzzoni, "Update on Therapeutic Strategy in Lung Carcinoids," Journal of Cancer Therapy, Vol. 4 No. 10, 2013, pp. 1466-1471. doi: 10.4236/jct.2013.410176.