PMID- 19350320
OWN - NLM
STAT- MEDLINE
DCOM- 20091020
LR  - 20211020
IS  - 1432-2323 (Electronic)
IS  - 0364-2313 (Linking)
VI  - 33
IP  - 6
DP  - 2009 Jun
TI  - Screening of patients with multiple endocrine neoplasia type 1 (MEN-1): a 
      critical analysis of its value.
PG  - 1208-18
LID - 10.1007/s00268-009-9983-8 [doi]
AB  - BACKGROUND: Screening of multiple endocrine neoplasia type 1 (MEN-1) patients is 
      widely recommended because one-fifth succumb to malignant neoplasms. However, 
      recommendations for screening modalities and intervals are based mostly on 
      nonprospective data. METHODS: Thirty-five of 48 MEN-1 patients were evaluated at 
      least twice by an annual screening program in a single-center, prospective, 
      nonrandomized study between 1997 and 2006. The screening program comprised 
      anamnesis, clinical examination, imaging procedures, and extensive biochemical 
      evaluations. Prospectively diagnosed lesions were evaluated separately from 
      nonprospectively diagnosed lesions at first evaluation. RESULTS: The median age 
      of the patients was 45 years (range = 15-70) at initial assessment. They were 
      followed for a median of 72 months (range = 24-108) by a median of 6 (range = 
      2-10) evaluations. The vast majority of lesions were nonprospectively diagnosed 
      at initial evaluation: 13 of 17 patients had primary hyperparathyroidism (pHPT), 
      24 of 29 had pancreatic endocrine tumors (PETs), and 4 of 4 had carcinoids. Vice 
      versa adrenal lesions were mostly prospectively detected (18/23). Malignancy was 
      observed in 10 patients (28%) in the initial assessment and without symptoms in 5 
      patients (9 PETs, 3 carcinoids). Endoscopic ultrasound (EUS) of 29 patients 
      detected 88 PETs which were followed for 157 patient years. The mean annual 
      growing rate was 13.28 +/- 28.23 mm with respect to the baseline tumor diameter 
      of 9 mm. In 35 patients the mean incidence of newly diagnosed PETs was 0.52/year. 
      Adrenal lesions were invariably nonfunctional. A mean change in diameter of 6.7 
      +/- 23.44% was monitored and malignant transformation was absent. CONCLUSIONS: 
      Most lesions are detected at initial screening, particularly malignant tumors. 
      Computed tomography of the abdomen and chest did not identify additional lesions. 
      The interval between screenings could be extended to 3 years based on annually 
      calculated growth rates and the incidence of MEN-1-associated lesions. The 
      assessment of calcium, gastrin, and prolactin is sufficient for biochemical 
      screening in MEN-1.
FAU - Waldmann, Jens
AU  - Waldmann J
AD  - Department of General Surgery, Philipps-University Marburg, Baldingerstrasse, 
      35033, Marburg, Germany. jwaldman@med.uni-marburg.de
FAU - Fendrich, Volker
AU  - Fendrich V
FAU - Habbe, Nils
AU  - Habbe N
FAU - Bartsch, Detlef K
AU  - Bartsch DK
FAU - Slater, Emily P
AU  - Slater EP
FAU - Kann, Peter H
AU  - Kann PH
FAU - Rothmund, Matthias
AU  - Rothmund M
FAU - Langer, Peter
AU  - Langer P
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Biomarkers, Tumor/analysis
MH  - Clinical Protocols
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/epidemiology/genetics
MH  - Practice Guidelines as Topic
MH  - Prospective Studies
MH  - Young Adult
EDAT- 2009/04/08 09:00
MHDA- 2009/10/21 06:00
CRDT- 2009/04/08 09:00
PHST- 2009/04/08 09:00 [entrez]
PHST- 2009/04/08 09:00 [pubmed]
PHST- 2009/10/21 06:00 [medline]
AID - 10.1007/s00268-009-9983-8 [doi]
PST - ppublish
SO  - World J Surg. 2009 Jun;33(6):1208-18. doi: 10.1007/s00268-009-9983-8.