Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2007) 14 P151

ECE2007 Poster Presentations (1) (659 abstracts)

Frequency of occurence of MEN1 syndrome in patients admitted with primary hyperthyroidism

Agata Baldys-Waligorska , Grzegorz Sokolowski , Malgorzata Trofimiuk , Filip Golkowski & Bohdan Huszno


Department of Endocrinology, Collegium Medicum of the Jagiellonian University, Krakow, Poland.

Background: Primary hyperparathyroidism (HPT) is the most common endocrinopathy in MEN1 and usually its first clinical manifestation. Yet MEN1 is a rare disease, representing only 2–4% of all cases of HTP. We studied the frequency of MEN1 syndrome in HPT patients admitted to our Department.

Methods: In a retrospective analysis of 84 suspected HPT patients hospitalized in 1999–2006, case reports of 11 patients with suspected MEN1 were analysed. MEN1 was stated if two of the three main MEN1-related endocrine tumours occurred.

Results: HPT diagnosis was confirmed in 69 patients: of mean age 55.4±14.1 yrs. Median values of PTH and total calcium concentration were 57.4 pg/ml (min – 60.6, max – 1580) and 2.95 mmol/l (min – 2,2, max – 4,0), respectively. In parathyroid scintigraphy equivocal tracer accumulation was found in 72% of cases. MEN1 was diagnosed in 9 patients of mean age 51.3±12,0 yrs, in 8 of whom (89.0%) HPT was confirmed. Pituitary adenoma was found in 7 patients: 3 prolactinomas, 1 acromegaly, 1 Cushing disease and 2 non-functioning tumours. In 2 patients pancreatic tumours were diagnosed: somatostatinoma and gastrinoma were confirmed by laboratory tests and immunohistochemistry. Four carcinoids: 3 gastric and one bronchial were found. Mean 5-HIAA (5-hydroxyindoloacetic acid) urine excretion in the carcinoid patients was 144.0 μmol/24hrs (norm: up to 40), mean serum concentration of CgA (chromogranin-A) 728.7 U/L (norm: up to 18.0). Moreover, in the patient with HPT and somatostatinoma concurrent von Recklinghausen’s disease was diagnosed and in the HPT and prolactinoma patient, meningioma was found. Adrenal tumours were observed in two cases: one pheochromocytoma and one non-functioning tumour.

Conclusions: The frequency of MEN1 occurrence in our patients (13%) is much higher than that quoted in the literature (2–4%), clearly, due to referral of complicated cases to our Department. Patients with symptoms atypical for HPT should be screened towards MEN1.

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