PMID- 15713725
OWN - NLM
STAT- MEDLINE
DCOM- 20050609
LR  - 20161124
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 90
IP  - 5
DP  - 2005 May
TI  - Thymic neuroendocrine carcinoma (carcinoid) in multiple endocrine neoplasia type 
      1 syndrome: the Italian series.
PG  - 2603-9
AB  - Neuroendocrine tumors may occur in the setting of multiple endocrine neoplasia 
      type 1 (MEN1) syndrome. Among these, a probably underestimated prevalence of well 
      differentiated neuroendocrine thymic carcinoma (carcinoid), a neoplasm 
      characterized by very aggressive behavior, has been described. We report 
      characterization of the seven Italian cases in which this association occurred 
      among a series of 221 MEN1 patients (41 sporadic and 180 familial cases; 
      prevalence, 3.1%). All of the patients were male, and six of seven (85%) were 
      heavy smokers. No associated hormonal hypersecretion was detected. The first 
      diagnosis was between the second and fifth decades. Familial clusters were 
      present in three of seven (42.8%). No genotype-phenotype correlation was found. 
      All seven cases were associated with hyperparathyroidism. In one patient, 
      prophylactic thymectomy revealed a small nodular lesion suggestive of a thymic 
      carcinoid, providing evidence that preventive thymectomy might prevent additional 
      growth of an occult thymic carcinoid. These findings confirm that thymic 
      carcinoids are associated with a very high lethality, with a near-total 
      prevalence in smoker males. Therefore, prophylactic thymectomy should be 
      considered at neck surgery for primary hyperparathyroidism in MEN1 male patients, 
      especially for smokers, and, due to the frequent familial clusters distribution 
      of this pathology, in subjects with affected relatives presenting this feature. 
      Thus, we recommend screening every patient affected with a neuroendocrine thymic 
      neoplasm for MEN1 syndrome.
FAU - Ferolla, P
AU  - Ferolla P
AD  - Department of Internal Medicine and Endocrine Sciences, Thoracic Surgery, 
      Institute of Pathology, University of Perugia, Via E. Dal Pozzo, 06100 Perugia, 
      Italy. pferolla@tin.it
FAU - Falchetti, A
AU  - Falchetti A
FAU - Filosso, P
AU  - Filosso P
FAU - Tomassetti, P
AU  - Tomassetti P
FAU - Tamburrano, G
AU  - Tamburrano G
FAU - Avenia, N
AU  - Avenia N
FAU - Daddi, G
AU  - Daddi G
FAU - Puma, F
AU  - Puma F
FAU - Ribacchi, R
AU  - Ribacchi R
FAU - Santeusanio, F
AU  - Santeusanio F
FAU - Angeletti, G
AU  - Angeletti G
FAU - Brandi, M L
AU  - Brandi ML
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20050215
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
SB  - IM
MH  - Adult
MH  - Carcinoid Tumor/diagnostic imaging/*genetics/therapy
MH  - Humans
MH  - Hyperparathyroidism/etiology
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnostic imaging/*genetics/therapy
MH  - Positron-Emission Tomography
MH  - Thymus Neoplasms/diagnostic imaging/*genetics/therapy
EDAT- 2005/02/17 09:00
MHDA- 2005/06/10 09:00
CRDT- 2005/02/17 09:00
PHST- 2005/02/17 09:00 [pubmed]
PHST- 2005/06/10 09:00 [medline]
PHST- 2005/02/17 09:00 [entrez]
AID - jc.2004-1155 [pii]
AID - 10.1210/jc.2004-1155 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2005 May;90(5):2603-9. doi: 10.1210/jc.2004-1155. Epub 
      2005 Feb 15.