Wermer syndrome revealed by primary hyperparathyroidism: a case report
Mchirgui Nadia, Hadj Ali Insaf, Khiari Karima, Cheikhrouhou Hela, Lakhoua Youssef & Ben Abdallah Nejib
Multiple endocrine neoplasia type 1 (MEN1) is a rare but misleading disease. The diagnosis is evocated when two main lesions are present (parathyroid, pituitary gland, endocrine pancreas tumor: Gastrinoma). We studied a 36 year-old woman hospitalized in orthopedics for a neck femur fracture. A hypercalcaemia (Ca++=2.84 mmol/l) with elevated PTH=500 pg/ml were discovered before intervention. The bone biopsy concluded to primary hyperparathyroidism lesions.
The history-taking revealed that the propositus had 11 years of secondary amenorrhea without galactorrhea before the time of parathyroidectomy.
Hormonal results: FSH=0.4 mUI/ml, LH<0.1 mUI/ml, oestradiol=20 pg/ml, prolactine=24 947 mUI/l (1133 pg/ml). In front of this tumoral rate of prolactine, the MRI of the pituitary gland had found an enlarged sella turcica related to a macroprolactinoma. After parathyroid adenoma resection, the patient had normalized her calcemia (2.18 mmol/l) and PTH rate (55 pg/ml). For prolactinoma, she had received bromocriptine treatment (5 mg/day) and the rate of prolactine had decreased.
The Wermer syndrome was diagnosed and the genetic diagnosis was on going. An abdominal CT scan did not found any endocrine pancreas abnormality.
We conclude that, as a general rule, any apparently isolated endocrine lesion such hyperparathyroidism must prompt the endocrinologist to look for another endocrine lesion and to look for an abnormal family tree with recurrent monoglandular or pluriglandular lesions.