PMID- 9920064
OWN - NLM
STAT- MEDLINE
DCOM- 19990203
LR  - 20131121
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 84
IP  - 1
DP  - 1999 Jan
TI  - Screening for multiple endocrine neoplasia type 1 and hormonal production in 
      apparently sporadic neuroendocrine tumors.
PG  - 69-75
AB  - Screening was performed in 130 consecutive patients with apparently sporadic 
      neuroendocrine tumors (NET) to assess the prevalence of multiple endocrine 
      neoplasia type 1 (MEN1) and hormonal production. Screening for MEN1 included 
      measurement of serum calcium and PTH [PTH-(1-84)], gastrin, PRL, and insulin-like 
      growth factor type I (IGF-I) levels. MEN1 genetic testing was performed in 
      patients with two components of the MEN1 syndrome. Screening for hormonal 
      production included measurement of serum neuron-specific enolase (NSE), 
      calcitonin (CT), glycoprotein alpha-subunit (GP alpha), hCG beta-subunit (free 
      hCG beta), and somatostatin levels. Twenty-four-hour urinary free cortisol (UFC) 
      and 5-hydroxyindolacetic acid (5-HIAA) determinations were also performed. Four 
      patients had hyperparathyroidism, none of whom had pituitary or familial disease. 
      Hyperprolactinemia was compatible with a pituitary disease in one patient. No 
      acromegalic feature or any increase in IGF-I was found. Hypergastrinemia, 
      compatible with an associated pancreatic NET, was found in one patient. Genetic 
      screening of the MEN1 gene was performed in five of the six patients with two 
      components of the MEN1 syndrome. A nonsense mutation (Arg108stop) was identified 
      in the tumor of one patient. Elevated NSE, 5-HIAA, CT, GP alpha, free hCG beta, 
      SMS, and nonsuppressible UFC were found in 47%, 46%, 14%, 19%, 12%, 3%, and 6% of 
      NET patients, respectively. Production of CT, GP alpha, and free hCG beta was 
      highly related to the primary site: all but two of these secretions originated in 
      foregut NET. 5-HIAA secretion was found in 27% of foregut-derived and 85% of 
      midgut-derived NET. In conclusion, MEN1 is a rare event in patients presenting 
      with apparently sporadic NET. It occurred mainly in foregut NET and should be 
      screened for by serum calcium and PTH-(1-84) measurements. Routine hormonal 
      measurements should depend on the primary site. NSE, 5-HIAA, CT, and alphaGP 
      should be routinely measured in foregut-derived NET; only serum NSE and 5-HIAA 
      measurements are recommended in midgut-derived NET.
FAU - Baudin, E
AU  - Baudin E
AD  - Nuclear Medicine, Institut Gustave-Roussy, Villejuif, France. baudin@igr.fr
FAU - Bidart, J M
AU  - Bidart JM
FAU - Rougier, P
AU  - Rougier P
FAU - Lazar, V
AU  - Lazar V
FAU - Ruffie, P
AU  - Ruffie P
FAU - Ropers, J
AU  - Ropers J
FAU - Ducreux, M
AU  - Ducreux M
FAU - Troalen, F
AU  - Troalen F
FAU - Sabourin, J C
AU  - Sabourin JC
FAU - Comoy, E
AU  - Comoy E
FAU - Lasser, P
AU  - Lasser P
FAU - DeBaere, T
AU  - DeBaere T
FAU - Schlumberger, M
AU  - Schlumberger M
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (Chorionic Gonadotropin, beta Subunit, Human)
RN  - 0 (Hormones)
RN  - 54-16-0 (Hydroxyindoleacetic Acid)
RN  - 9007-12-9 (Calcitonin)
RN  - EC 4.2.1.11 (Phosphopyruvate Hydratase)
RN  - WI4X0X7BPJ (Hydrocortisone)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Calcitonin/blood
MH  - Chorionic Gonadotropin, beta Subunit, Human/blood
MH  - Female
MH  - Hormones/*biosynthesis
MH  - Humans
MH  - Hydrocortisone/urine
MH  - Hydroxyindoleacetic Acid/urine
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/epidemiology
MH  - Neuroendocrine Tumors/*metabolism
MH  - Phosphopyruvate Hydratase/metabolism
MH  - Prevalence
EDAT- 1999/01/27 00:00
MHDA- 1999/01/27 00:01
CRDT- 1999/01/27 00:00
PHST- 1999/01/27 00:00 [pubmed]
PHST- 1999/01/27 00:01 [medline]
PHST- 1999/01/27 00:00 [entrez]
AID - 10.1210/jcem.84.1.5445 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 1999 Jan;84(1):69-75. doi: 10.1210/jcem.84.1.5445.