PMID- 29201451 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2072-1439 (Print) IS - 2077-6624 (Electronic) IS - 2072-1439 (Linking) VI - 9 IP - Suppl 15 DP - 2017 Nov TI - Neuroendocrine tumors of the thymus. PG - S1484-S1490 LID - 10.21037/jtd.2017.10.83 [doi] AB - Primary neuroendocrine tumors of the thymus (NETTs) are rare and biologically very aggressive neoplasms, usually located in the anterior mediastinal space. They are more frequently observed in males, in their fourth/fifth decades of life. In 50% of cases, NETTs are associated with endocrinopaties [Cushing's syndrome, acromegaly or Multiple Endocrine Neoplasia-1 (MEN-1) syndrome]. NETTs very often present with invasion of the surrounding mediastinal anatomical structures. Surgery, even in advanced stages, is the mainstay of treatment: a compete resection through a median sternotomy or a combined access (sternotomy + thoracotomy) should be always attempted. Induction chemotherapy (+/- radiotherapy) is usually administered in advanced neoplasms, with the aim to achieve tumor shinkage, increasing, therefore, the chance to obtain a complete resection. Postoperative radiotherapy (+/- chemotherapy) is administered in case of invasive lesions, or incomplete resections. NETTs long-term outcome is poor, even in case of completely resected tumors, due to high risk of recurrence or distant metastases development. Prognosis mainly depends on tumor stage, invasivity, completeness of resection, possible association with endocrinopaties and recurrence/distant metastases development. FAU - Filosso, Pier Luigi AU - Filosso PL AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Ruffini, Enrico AU - Ruffini E AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Solidoro, Paolo AU - Solidoro P AD - San Giovanni Battista Hospital, Service of Pulmonology, Via Genova, Torino, Italy. FAU - Roffinella, Matteo AU - Roffinella M AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Lausi, Paolo Olivo AU - Lausi PO AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Lyberis, Paraskevas AU - Lyberis P AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Oliaro, Alberto AU - Oliaro A AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. FAU - Guerrera, Francesco AU - Guerrera F AD - Department of Surgical Sciences, University of Torino, Corso Dogliotti, Torino, Italy. LA - eng PT - Journal Article PT - Review PL - China TA - J Thorac Dis JT - Journal of thoracic disease JID - 101533916 PMC - PMC5690945 OTO - NOTNLM OT - Thymus OT - diagnosis OT - neuroendocrine carcinoma OT - outcome OT - recurrences OT - surgery OT - symptoms COIS- Conflicts of Interest: The authors have no conflicts of interest to declare. EDAT- 2017/12/05 06:00 MHDA- 2017/12/05 06:01 PMCR- 2017/11/01 CRDT- 2017/12/05 06:00 PHST- 2017/12/05 06:00 [entrez] PHST- 2017/12/05 06:00 [pubmed] PHST- 2017/12/05 06:01 [medline] PHST- 2017/11/01 00:00 [pmc-release] AID - jtd-09-S15-S1484 [pii] AID - 10.21037/jtd.2017.10.83 [doi] PST - ppublish SO - J Thorac Dis. 2017 Nov;9(Suppl 15):S1484-S1490. doi: 10.21037/jtd.2017.10.83.