Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

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 mm(3) 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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“…A limited resection can be associated with a high risk of recurrence, but an extended operation can cause a permanent hypocalcemia in 13-47% of cases (107). Nevertheless, a measurable risk of recurrence is present also after subtotal parathyroidectomy (108,109,110) and then a subtotal parathyroidectomy (including at least three glands) seems to be the most appropriate treatment for HPT in MEN1 patients.…”

Prospective Study of Surgery for Primary Hyperparathyroidism (HPT) in Multiple Endocrine Neoplasia-Type 1 and Zollinger-Ellison Syndrome

“…3,4 Surgery to control the HPT in MEN-1 patients requires either three and one-half gland parathyroidectomy or fourgland excision with autograft. [5][6][7][8] Patients have a significant rate of persistent HPT with lesser procedures and a high-rate of recurrent HPT as well. 5,[9][10][11][12][13] Careful long-term follow-up studies of these patients are few and the long-term results of surgery have been infrequently reported.…”

Multiple endokrine Neoplasie Typ 1

“…Im Gegensatz zur sporadischen Erkrankung gibt es jedoch einen wesentlichen Unterschied. Der therapeutische Ansatz beim MEN1-pHPT wird aufgrund des genetisch determinierten Proliferationsreizes für das Nebenschilddrüsengewebe von vielen Autoren als prinzipiell palliativ angesehen [13,16]. Synchron oder metachron können mehrere Drüsen erkranken, und verbliebenes Restgewebe kann …”

“…Hubbard et al [16] berichteten über eine Rezidivrate von 44% und eine Persistenzrate von 22% nach stPTX ohne zervikale Thymektomie, allerdings beobachteten sie bei keinem der Patienten eine dauerhafte Hypokalzämie. 2 weitere Studien bestätigten die relativ hohen Rezidivraten nach stPTX.…”

“…Thompson et al [27] berichteten über Langzeitrezidivraten von nur 12,5% nach stPTX inklusive zervikaler Thymektomie, während bei keinem Patienten eine andauernde Hypokalzämie beobachtet wurde. Ähnliche Erfahrungen wurden von Goudet et al [13] und Hubbard et al [16] aus Frankreich berichtet. Je nach Autorengruppe wurden zwischen 60 und 80 mg Nebenschilddrüsen in situ belassen.…”