PMID- 11150038
OWN - NLM
STAT- MEDLINE
DCOM- 20010208
LR  - 20190915
IS  - 0039-6060 (Print)
IS  - 0039-6060 (Linking)
VI  - 129
IP  - 1
DP  - 2001 Jan
TI  - Malignant endocrine tumors in patients with MEN 1 disease.
PG  - 91-5
AB  - BACKGROUND: In contrast with multiple endocrine neoplasia type 2, malignancies 
      are of minor importance in multiple endocrine neoplasia type 1 (MEN 1) syndrome. 
      METHODS: The data for 42 patients with MEN 1 syndrome were evaluated. Twelve 
      patients (29%) had 1 or more associated malignancies: malignant gastrinoma (1 
      patient), neuroendocrine tumors of the thymus (2 patients), neuroendocrine tumors 
      of the lung (3 patients), neuroendocrine tumor of the ileum (1 patient), 
      adrenocortical carcinomas (2 patients), and a combination of neuroendocrine 
      tumors of thymus, lung or pancreas (3 patients). RESULTS: Despite suspected MEN 1 
      syndrome in 7 patients, malignancies were detected late in 4 patients and could 
      not be resected curatively. The survival rates for 5 years and 10 years after 
      operation of the malignant tumor were 66% +/- 14% (SE) and 33% +/- 15% (SE), 
      respectively. CONCLUSIONS: In patients at risk for the MEN 1 syndrome, genetic 
      screening is indispensable. Gene carriers have to be followed up closely with 
      hormone analysis and routine examination of the thorax, pancreas, and adrenal 
      glands to detect malignancies as early as possible.
FAU - Dotzenrath, C
AU  - Dotzenrath C
AD  - Department of Surgery, Heinrich-Heine Universitat, Dusseldorf, Germany.
FAU - Goretzki, P E
AU  - Goretzki PE
FAU - Cupisti, K
AU  - Cupisti K
FAU - Yang, Q
AU  - Yang Q
FAU - Simon, D
AU  - Simon D
FAU - Roher, H D
AU  - Roher HD
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Surgery
JT  - Surgery
JID - 0417347
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Female
MH  - Gastrinoma/genetics/surgery
MH  - Humans
MH  - Hyperparathyroidism/genetics
MH  - Lung Neoplasms/genetics/surgery
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/pathology/*surgery
MH  - Mutation
MH  - Pancreatic Neoplasms/genetics/surgery
MH  - Thymus Neoplasms/genetics/surgery
EDAT- 2001/01/10 11:00
MHDA- 2001/03/03 10:01
CRDT- 2001/01/10 11:00
PHST- 2001/01/10 11:00 [pubmed]
PHST- 2001/03/03 10:01 [medline]
PHST- 2001/01/10 11:00 [entrez]
AID - S0039606001457229 [pii]
AID - 10.1067/msy.2001.109500 [doi]
PST - ppublish
SO  - Surgery. 2001 Jan;129(1):91-5. doi: 10.1067/msy.2001.109500.