Primary hyperparathyroidism in MEN 1 – how radical

Authors: Johnathan G Hubbard Frédéric Sebag Sylvie Maweja JeanFrançois Henry

Publish Date: 2002/02/06

Volume: 386, Issue: 8, Pages: 553-557

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Abstract

Primary hyperparathyroidism is the most common manifestation of MEN 1 syndrome The management of these patients is complex due to the underlying disease process which predisposes patients to persistent and recurrent disease The surgical treatment of patients with MEN 1 and hyperparathyroidism can therefore be considered to be palliative in nature The basic principles of surgery include 1 obtaining and maintaining normocalcaemia for the longest time possible avoiding persistent/recurrent hypercalcaemia 2 avoiding surgically induced hypocalcaemia and 3 facilitating future surgery for recurrent disease Two approaches have been described as the best practice for patients with hyperparathyroidism in MEN 1 subtotal parathyroidectomy leaving a remnant of no more than 60 mg in the neck and total parathyroidectomy with immediate autotransplantation of 10–20 1 mm3 pieces of parathyroid tissue Both approaches should be combined with efforts to exclude supernumerary glands and ectopic parathyroid tissue by including resection of fatty tissue from the central neck compartment and thymectomy in all patients Cryopreservation of parathyroid tissue should be performed whenever facilities are available In patients with persistent or recurrent disease an attempt to obtain total elimination of cervical parathyroid tissue is justified combined with cryopreservation of parathyroid tissue As radical as surgery is for hyperparathyroidism in MEN 1 the surgeon must take steps to avoid permanent hypoparathyroidism which in young patients may be worse than the disease itself

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