Primary hyperparathyroidism in multiple endocrine neoplasia type IIa: retrospective French multicentric study. Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group), French Association of Endocrine Surgeons

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 fam...

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Published in:World journal of surgery 1996-09, Vol.20 (7), p.808-12; discussion 812-3
Main Authors: Kraimps, J L, Denizot, A, Carnaille, B, Henry, J F, Proye, C, Bacourt, F, Sarfati, E, Dupond, J L, Maes, B, Travagli, J P, Boneu, A, Roger, P, Houdent, C, Barbier, J, Modigliani, E
Format: Article
Language:English
Subjects:
Adenoma - pathology
Adenoma - surgery
Adolescent
Adrenal Gland Neoplasms - pathology
Adrenal Gland Neoplasms - surgery
Adult
Age Factors
Aged
Calcium - blood
Carcinoma, Medullary - pathology
Carcinoma, Medullary - surgery
Child
Female
Follow-Up Studies
France
Humans
Hypercalcemia - etiology
Hyperparathyroidism - etiology
Hyperparathyroidism - genetics
Hyperparathyroidism - pathology
Hyperparathyroidism - surgery
Hyperplasia
Hypoparathyroidism - etiology
Male
Middle Aged
Multiple Endocrine Neoplasia Type 2a - complications
Multiple Endocrine Neoplasia Type 2a - pathology
Multiple Endocrine Neoplasia Type 2a - surgery
Parathyroid Glands - pathology
Parathyroid Glands - transplantation
Parathyroidectomy - methods
Pheochromocytoma - pathology
Pheochromocytoma - surgery
Recurrence
Retrospective Studies
Thyroid Neoplasms - pathology
Thyroid Neoplasms - surgery
Transplantation, Autologous
Treatment Outcome
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Summary: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.
ISSN:0364-2313