Because of the rarity of adrenocortical carcinoma, survival rates and the prognosis for patients who have undergone operation are not well known. The purpose of the French Association of Endocrine Surgery was to evaluate these factors over an 18-year period. A trend study was associated to assess changes in the clinical and biochemical presentations as well as the surgical evolution. A total of 253 patients (158 women, 95 men) with a mean age of 47 years were included. Cushing syndrome was the main clinical presentation (30%), and hormonal studies revealed secreting tumors in 66% of the cases. Altogether, 72% (n = 182) of patients underwent resection for cure, and 41.5% (n = 105) of them had an extensive resection because of metastatic cancer. A lymphadenectomy was performed in 32.5% (n = 89) of the cases. The operative mortality was 5.5% (n = 14). Patients were given mitotane as adjuvant therapy in 53.8% of the cases (n = 135). The results of staging were stage I in 16 patients (6.3%), stage II (local disease) in 126 patients (49.8%), stage III (locoregional disease) in 57 patients (22.5%), and stage IV (metastases) in 54 patients (21.3%). Neither tumor staging nor the rate of curative surgery changed during the study period. More subcostal incisions were performed, and the use of mitotane increased significantly. The 5-year actuarial survival rates were 38% overall, 50% in the curative group, 66% for stage I, 58% for stage II, 24% for stage III, and 0% for stage IV. Multivariate analysis showed that mitotane benefited only the group of patients not operated on for cure. A better prognosis was found in patients operated on after 1988 (p = 0.04), in those with precursor-secreting tumors (p = 0.005), and in those at local stages of the disease (p = 0.0003). Thus mitotane benefited only patients not operated on for cure. Curative resection, precursor secretion, recent diagnosis, and local stage were favorably associated with survival.

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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.

The most appropriate surgical approach for hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 remains controversial. It has been advocated that reoperations for recurrent disease are easier to perform after total parathyroidectomy (TP) with autotransplantation than after subtotal parathyroidectomy (SP). In view of our large experience in patients with secondary HPT for whom TP with autotransplantation did not simplify reoperations, SP remains our preferred treatment for patients with HPT and multiple endocrine neoplasia type 1. Design: Retrospective cohort study. Setting: Tertiary referral medical center. Patients: A total of 29 consecutive patients (22 women, 7 men; mean age, 42.2 years) with multiple endocrine neoplasia type 1 who underwent definitive cervical exploration for HPT. Main Outcome Measures: Temporary and permanent hypocalcemia, pattern of parathyroid disease, and sites and timing of recurrent HPT. Definitive primary surgery included SP in 21 patients, TP with autotransplantation in 4 patients, and less-than-subtotal parathyroidectomy in 4 selected patients. Results: The mean follow-up was 88.5 months (range, 8-285 months). Four patients died during follow-up; 2 of these deaths were related to multiple endocrine neoplasia. No patients had persistent HPT. Temporary hypocalcemia occurred in 12 SP cases (57%), 4 TP with autotransplantation cases (100%), and 0 less-thansubtotal parathyroidectomy cases. Permanent hypocalcemia requiring long-term treatment occurred in 2 SP cases (10%), 1 TP with autotransplantation case (25%), and 0 less-than-subtotal parathyroidectomy cases. Four patients developed recurrent disease, including 1 with SP, 2 with TP with autotransplantation, and 1 with lessthan-subtotal parathyroidectomy at 57 months, 197 and 180 months, and 164 months, respectively, representing 14% of all of the patients and 43% of patients with more than 10 years of follow-up. Conclusions: Recurrent HPT occurs many years after definitive primary surgery (median, 14.3 years). Surgical treatment should therefore aim to minimize the risk of permanent hypocalcemia and facilitate future surgery. When correctly performed, SP fulfills these objectives.

Among 258 patients operated on for secondary hyperparathyroidism (HPT II) from 1971 to 1988, a total of 33 had one or more reoperations for persistent or recurrent HPT II. These reoperations did not induce any mortality or significant morbidity. After inadequate parathyroidectomy (25 cases), 15 patients were reoperated. Twelve of these had undergone initial surgery at another institution. Three patients died of causes unrelated to their HPT II. The other 12 patients are disease-free. After successful subtotal parathyroidectomy (79 cases), 2 patients (2.5%) had a recurrence 5 and 6 years later, respectively. Currently, the 2 patients remain disease-free. After total parathyroidectomy with autotransplantation (152 cases), 16 patients (10.5%) had reoperations on the grafts. The mean time before reoperation was 2 1/2 years. Hypertrophy of grafted fragments was observed in 4 cases (2.6%), but only 2 of these 4 patients were cured by removal of the grafts. Residual parathyroid tissue or a supernumerary gland in the neck or the mediastinum was suspected in 5 patients, but this could not be confirmed because one had already been reoperated on in the neck without success, another still refuses reoperation, and 3 died. In 6 other patients, the recurrence was debatable and HPT II was not confirmed. In the last 3 patients, the diagnosis was incorrect and aluminum intoxication was proved later. Results of reoperations for persistent or recurrent HPT II depend, first, on a correct diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)