Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type IIa: Retrospective French Multicentric Study

Abstract

Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) type IIa is rare, occurring in 20% to 30% of the patients. The aim of this study was to evaluate clinical findings, surgical therapy, and outcome for 56 patients affected by PHPT among 249 MEN-IIa patients collected from 84 families assembled by the Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group). This retrospective study was based on cases registered by the GETC (20 participating centers) from 1969 to 1994. Characteristics of PHPT in 56 patients (31 women, 25 men) with MEN-IIa were reviewed. All but two underwent cervicotomy. The median age at diagnosis was 37.6 years. PHPT was found concomitantly with medullary thyroid carcinoma (MTC) or pheochromocytoma in 43 patients (77%). PHPT was asymptomatic in 68% of the patients. Serum calcium levels ranged from 2.20 to 3.70 mmol/L (median 2.82 mmol/L; normal 2. 10-2.60 mmol/L). The number of parathyroid glands removed at surgery was 0 (n = 2), 1 (n = 24), 2 (n = 5), > 2 (n = 12), 4 (n = 11). Pathology (initial surgery) consisted of 24 adenomas, 4 double adenomas, and 25 hyperplasia. Cure after initial surgery was obtained in 89%, including a 22% incidence of hypoparathyroidism. There were 6 cases (11%) with persistent PHPT. With a mean follow-up of 6.4 years, five patients (9%) had recurrent PHPT. The results indicate that MEN-IIa-related PHPT is generally associated with mild, often asymptomatic hypercalcemia. Despite recurrences encountered 5 to 15 years after the first cervicotomy, resection of only macroscopically enlarged glands generally appears sufficient. Subtotal or total parathyroidectomy with autotransplantation is associated with a high rate of hypoparathyroidism.

Accuracy of parathyroid imaging: comparison of planar scintigraphy, spect, spect/ct and c-11 methionine pet for the detection of parathyroid adenomas and glandular hyperplasia

“…Hyperparathyroidism that presents after a period of more than six months of normocalcemia following surgery is called "recurrent hyperparathyroidism" and is commonly due to continuing growth of the remaining parathyroid glands (3). Secondary hyperparathyroidism is a compensatory hypertrophy of all parathyroid glands due to hypocalcemia, as occurs in renal failure or with vitamin D deficiency (4), whereas tertiary hyperparathyroidism describes the development of autonomous function of parathyroid tissue after longstanding secondary hyperparathyroidism (5)(6)(7)(8).…”

First Description of Parathyroid Disease in Multiple Endocrine Neoplasia 2A Syndrome

“…This multiple gland disorder arises from a germ-line mutation affecting parathyroid cells and not from an extra-glandular stimulus. Each parathyroid gland is prone to hyperplasia and recurrences of hyperparathyroidism have been reported in 11-40% of those afflicted [8,11] and persistence in 11% [11]; but persistence was present in only 3-6% following more extensive operations [12]. The age of onset of parathyroid hyperfunction has been seen in patients as young as 9 [13] and as old as 70 [10], but most commonly in individuals between 17 and 49 years of age [8].…”

Diagnosis and management of medullary thyroid carcinoma

“…The diagnosis of HPT and indications for parathyroid surgery in MEN2 are similar to those for sporadic HPT [113][114][115][116]. Although fewer than four parathyroid glands may be enlarged, the consensus is that all four glands should be identified at parathyroid surgery [3].…”