Abstract
Parathyroid surgery in the setting of familial disorders resulting in multiglandular disease is challenging for the endocrine surgeon. The aim of surgery is to gain eucalcemia for a long time without producing hypocalcemia. Since the syndromes are rare, sufficient data are lacking in most diseases. MEN1-HPTH should be treated with subtotal or total PTX with autotransplantation. Surgery in MEN2A has to be more tailored to the individual patient and subtotal PTX, total PTX and autotransplantation as well as excision of grossly enlarged glands might be alternatives depending on the patient’s history of the disease. The surgical approach to patients with HPTH-JTS is the same as to MEN2A-HPTH. Nevertheless radical surgery may be required in this special setting, since the incidence of parathyroid carcinoma is comparably high in these patients.
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