PMID- 25243504 OWN - NLM STAT- MEDLINE DCOM- 20150122 LR - 20141022 IS - 0026-4946 (Print) IS - 0026-4946 (Linking) VI - 66 IP - 6 DP - 2014 Dec TI - Endocrine cancer syndromes: an update. PG - 533-47 AB - Endocrine neoplasms comprise a variety of benign and malign tumors that arise from the endocrine glands or neuroendocrine tissues. Although most endocrine neoplasms are sporadic, others are secondary to mutations of many known tumor-predisposing genes. Endocrine cancer syndromes, including Multiple Endocrine Neoplasia type 1 (MEN1), Multiple Endocrine Neoplasia type 2 (MEN2A and MEN2B), Multiple Endocrine Neoplasia type 4 (MEN4) syndromes, and inherited syndromes with different endocrine neoplasms (von Hippel-Lindau disease, Carney complex, Neurofibromatosis type 1, others) are heterogeneous group of cancer susceptibility syndromes that affect one or more of the endocrine glands or neuroendocrine tissues. Genetic studies and researches as well as technological possibilities allowed for detection of new endocrine cancer syndromes and genes leading to tumor susceptibility. In addition, early detection of children at risk for endocrine cancer syndromes using molecular analysis methods provided opportunity to regular monitoring of potential malignancies and timely intervention for these cases (e.g. early prophylactic thyroidectomy in MEN2). This review will describe the clinical, genetic, diagnostic and therapeutic options for endocrine cancer syndromes based on the current literature data. FAU - Anik, A AU - Anik A AD - Dokuz Eylul University Department of Pediatric Endocrinology Izmir, Turkey - ayhanabaci@gmail.com. FAU - Abaci, A AU - Abaci A LA - eng PT - Journal Article PT - Review DEP - 20140922 PL - Italy TA - Minerva Pediatr JT - Minerva pediatrica JID - 0400740 SB - IM MH - Early Detection of Cancer MH - Endocrine Gland Neoplasms/diagnosis/*pathology/therapy MH - Genetic Predisposition to Disease MH - Genetic Testing/methods MH - Humans MH - Molecular Diagnostic Techniques MH - Multiple Endocrine Neoplasia/diagnosis/*pathology/therapy MH - Mutation MH - Neoplastic Syndromes, Hereditary/diagnosis/pathology/therapy MH - Neuroendocrine Tumors/diagnosis/*pathology/therapy EDAT- 2014/09/23 06:00 MHDA- 2015/01/23 06:00 CRDT- 2014/09/23 06:00 PHST- 2014/09/23 06:00 [entrez] PHST- 2014/09/23 06:00 [pubmed] PHST- 2015/01/23 06:00 [medline] AID - R15Y9999N00A140027 [pii] PST - ppublish SO - Minerva Pediatr. 2014 Dec;66(6):533-47. Epub 2014 Sep 22.