PMID- 20464709
OWN - NLM
STAT- MEDLINE
DCOM- 20100831
LR  - 20170302
IS  - 0423-104X (Print)
IS  - 0423-104X (Linking)
VI  - 61
IP  - 2
DP  - 2010 Mar-Apr
TI  - Insulinoma in a patient with MEN 1 syndrome--9-year follow-up: case report.
PG  - 212-6
AB  - INTRODUCTION: Multiple endocrine neoplasia type 1 (MEN 1) is a rare autosomal 
      dominant inherited endocrine disease characterized by pancreatic, parathyroid, 
      and anterior pituitary tumours. Hypercalcaemia due to parathyroid tumours is 
      usually the first manifestation of MEN 1. Pancreatic islet tumours occur less 
      frequently, among them gastrinomas and insulinomas are the most prevalent. 
      Prolactinomas are a relatively common pituitary presentation of the syndrome. The 
      gene causing MEN 1 is localized in chromosome 11q13 and encodes a protein named 
      menin, which interacts with various proteins involved in transcriptional 
      regulation, cell division, and DNA repair. Various mutations in the menin gene 
      have been described, but so far no strong correlation between genotype and 
      phenotype has been found. CASE REPORT: We report a case of a 31-year-old man, a 
      lawyer, who was diagnosed with MEN 1 syndrome in 1999 at the age of 21 when he 
      was operated because of prolactinoma and hyperparathyroidism. In 2000 insulinoma 
      was suspected and eventually multifocal lesions in the pancreas were revealed. 
      However, the patient did not agree to be operated on. Since then he has been 
      followed up and has been treated with diazoxid. We observed gradual progression 
      of the disease, but the patient remains in relatively good condition. 
      CONCLUSIONS: Careful screening for MEN 1 is important in young patients with 
      pituitary tumours. Regular follow up is crucial even after surgical treatment. 
      The presented patient developed gradual enlargement of insulinomas and 
      reoccurrence of hyperparathyroidism as well.
FAU - Debski, Marcin G
AU  - Debski MG
AD  - Department of Internal Medicine and Endocrinology, Medical University, Warsaw. 
      debskim@wp.pl
FAU - Makowska, Anna
AU  - Makowska A
FAU - Bar-Andziak, Ewa
AU  - Bar-Andziak E
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Poland
TA  - Endokrynol Pol
JT  - Endokrynologia Polska
JID - 0370674
RN  - O5CB12L4FN (Diazoxide)
SB  - IM
MH  - Adult
MH  - Diagnosis, Differential
MH  - Diazoxide/therapeutic use
MH  - Disease Progression
MH  - Follow-Up Studies
MH  - Humans
MH  - Hyperparathyroidism/diagnosis
MH  - Insulinoma/*diagnosis/*drug therapy
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/*drug therapy
MH  - Pancreatic Neoplasms/*diagnosis/*drug therapy
EDAT- 2010/05/14 06:00
MHDA- 2010/09/02 06:00
CRDT- 2010/05/14 06:00
PHST- 2010/05/14 06:00 [entrez]
PHST- 2010/05/14 06:00 [pubmed]
PHST- 2010/09/02 06:00 [medline]
PST - ppublish
SO  - Endokrynol Pol. 2010 Mar-Apr;61(2):212-6.