PMID- 15531478
OWN - NLM
STAT- MEDLINE
DCOM- 20041202
LR  - 20220408
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 89
IP  - 11
DP  - 2004 Nov
TI  - Cutaneous tumors in patients with multiple endocrine neoplasm type 1 (MEN1) and 
      gastrinomas: prospective study of frequency and development of criteria with high 
      sensitivity and specificity for MEN1.
PG  - 5328-36
AB  - Multiple endocrine neoplasm type 1 (MEN1) is associated with parathyroid, 
      pancreatic, and pituitary tumors. Although most patients present with 
      hyperparathyroidism, the diagnosis can be difficult, because a significant 
      proportion present with other endocrinopathies or may lack a family history, and 
      other MEN1 manifestations may be mild. Recently, multiple cutaneous lesions 
      (angiofibromas and collagenomas) were reported to be frequent in MEN1 patients, 
      and it was proposed that their discovery suggested the diagnosis of MEN1. The 
      purpose of this study was to prospectively assess the frequency and 
      sensitivity/specificity of various cutaneous criteria for MEN1 in 110 consecutive 
      patients with gastrinomas with or without MEN1. All patients had hormonal and 
      functional studies to determine MEN1 status (48 with MEN1, 62 without MEN1), 
      dermatological evaluation, and tumor imaging studies. Angiofibromas and 
      collagenomas were more frequent in MEN1 patients (64% vs. 8% and 62% vs. 5%; P < 
      0.00001) and were multiple in 77-81% of the MEN1 patients. Lipomas occurred in 
      17%. The presence of these skin lesions did not correlate with age, disease 
      duration, or other MEN1 features. Angiofibromas or collagenomas (single or 
      multiple) had 50-65% sensitivity for MEN1 and 92-100% specificity. The 
      combination criterion of multiple angiofibromas (more than three) and any 
      collagenomas had the highest sensitivity (75%) and specificity (95%). This 
      criterion has greater sensitivity than pituitary or adrenal disease and is 
      comparable to hyperparathyroidism in some studies of patients with MEN1 with 
      gastrinoma. This criterion should have sufficient sensitivity/specificity to be 
      clinically useful.
FAU - Asgharian, Behnam
AU  - Asgharian B
AD  - Digestive Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases, National Institutes of Health, Building 10, Room 9C-103, 10 
      Center Drive, MSC 1804, Bethesda, Maryland 20892-1804, USA.
FAU - Turner, Maria L
AU  - Turner ML
FAU - Gibril, Fathia
AU  - Gibril F
FAU - Entsuah, Laurence K
AU  - Entsuah LK
FAU - Serrano, Jose
AU  - Serrano J
FAU - Jensen, Robert T
AU  - Jensen RT
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
SB  - IM
MH  - Adult
MH  - Age Factors
MH  - Aged
MH  - Aged, 80 and over
MH  - Female
MH  - Gastrinoma/*pathology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*pathology
MH  - Prospective Studies
MH  - Sensitivity and Specificity
MH  - Skin Neoplasms/*pathology
EDAT- 2004/11/09 09:00
MHDA- 2004/12/16 09:00
CRDT- 2004/11/09 09:00
PHST- 2004/11/09 09:00 [pubmed]
PHST- 2004/12/16 09:00 [medline]
PHST- 2004/11/09 09:00 [entrez]
AID - 89/11/5328 [pii]
AID - 10.1210/jc.2004-0218 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2004 Nov;89(11):5328-36. doi: 10.1210/jc.2004-0218.