PMID- 19081021
OWN - NLM
STAT- MEDLINE
DCOM- 20090113
LR  - 20081216
IS  - 1532-7361 (Electronic)
IS  - 0039-6060 (Linking)
VI  - 144
IP  - 5
DP  - 2008 Nov
TI  - Multimodal treatment of sporadic and inherited neuroendocrine tumors of the 
      thymus.
PG  - 780-5
LID - 10.1016/j.surg.2008.05.012 [doi]
AB  - BACKGROUND: Neuroendocrine tumors of the thymus (NETT) are a rare tumor entity of 
      the anterior mediastinum. They belong to the category of foregut carcinoids and 
      are often associated with the multiple endocrine neoplasia type 1 (MEN1) 
      syndrome. Approximately 180 cases have been reported since their first 
      description. NETT reveal an aggressive behavior leading to rapid local invasion 
      and metastatic spread. An aggressive surgical approach may achieve long-term 
      survival. METHODS: Patients presenting from 1990 to 2005 at the Department of 
      Surgery and the Department of Gastroenterology of the Philipps-University Marburg 
      with neuroendocrine tumors were enrolled in a prospective database with a 
      follow-up until 2005. Fifty MEN1-patients were enrolled in a study and screening 
      program. These databases were retrospectively reviewed identifying all patients 
      with NETT. The clinical features, therapeutical approaches and the outcome were 
      analyzed. RESULTS: Six patients were found with NETT, 4 patients suffered from 
      metastases at the time of presentation. All patients were male, with a median age 
      of 41.3 years at presentation. Four out of these 6 patients revealed MEN1 
      syndrome. All patients underwent tumor resection via sternotomy. Three patients 
      underwent parathyreoidectomy and transcervical thymectomy before the NETT was 
      diagnosed. Median survival was 53 months (range, 24-109). CONCLUSION: Given a 
      frequent association between MEN1 and NETT, all patients with NETT should be 
      screened for MEN1. Since transcervical thymectomy does not prevent all MEN1 
      patients from developing NETT, existing surveillance guidelines for MEN1 should 
      consider CT scan of the thorax on a regular basis.
FAU - Habbe, Nils
AU  - Habbe N
AD  - Department of Surgery, Philipps-University Marburg, Marburg, Germany. 
      habbe@med.uni-marburg.de
FAU - Waldmann, Jens
AU  - Waldmann J
FAU - Bartsch, Detlef K
AU  - Bartsch DK
FAU - Fendrich, Volker
AU  - Fendrich V
FAU - Rothmund, Matthias
AU  - Rothmund M
FAU - Langer, Peter
AU  - Langer P
LA  - eng
PT  - Journal Article
DEP - 20080810
PL  - United States
TA  - Surgery
JT  - Surgery
JID - 0417347
SB  - IM
MH  - Adult
MH  - Cohort Studies
MH  - Combined Modality Therapy
MH  - Databases, Factual
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/epidemiology/*therapy
MH  - Neuroendocrine Tumors/*diagnosis/genetics/*therapy
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Thymus Neoplasms/*diagnosis/genetics/*therapy
MH  - Treatment Outcome
EDAT- 2008/12/17 09:00
MHDA- 2009/01/14 09:00
CRDT- 2008/12/17 09:00
PHST- 2008/01/05 00:00 [received]
PHST- 2008/05/20 00:00 [accepted]
PHST- 2008/12/17 09:00 [entrez]
PHST- 2008/12/17 09:00 [pubmed]
PHST- 2009/01/14 09:00 [medline]
AID - S0039-6060(08)00355-3 [pii]
AID - 10.1016/j.surg.2008.05.012 [doi]
PST - ppublish
SO  - Surgery. 2008 Nov;144(5):780-5. doi: 10.1016/j.surg.2008.05.012. Epub 2008 Aug 
      10.