[Pilot study and preliminary validation of the French version of a quality-of-life questionnaire specific for subjects with growth hormone deficiency]. / Etude pilote et validation préliminaire de la version française d'un questionnaire de mesure de la qualité de vie spécifique des sujets atteints de déficit en hormone de croissance.

This study forms part of a research project seeking to develop a standardized questionnaire by which clinicians can assess the impact of growth hormone (GH) deficiency and its treatment on the "perceived health" or health-related quality of life of adults. The specific aim of this study was to translate and adapt for French patients the AGHDA (Adult Growth Hormone Deficiency Assessment) a standardized health-related quality of life measure for use with GH-deficient adults, initially developed in the United Kingdom, and to collect data which could be used to assess the main psychometric characteristics of its French version the ISPA-HC (Indicateur de Santé Perceptuelle Adulte-Hormone de Croissance). The main properties analyzed are: 1/ The scale's acceptability, as determined by means of face-to-face interviews with a small number of subjects, then by an ad hoc questionnaire administered during a test-retest study; 2/ The scale's reliability, as determined by a test-retest study (with a 15-days interval between tests); 3/ The scale's concurrent validity, as expressed by comparison with scores obtained by means of a generic quality of life scale, the ISPN (the French version of the Nottingham Health Profile). The results of this first trial with the ISPA-HC are conforming to what one can expect from a good instrument. The ISPA-HC has been shown to have very good levels of reliability and internal consistency. Its scores show a close correlation with those of the ISPN (the French version of the Nottingham Health Profile). This instrument can be used to measure variations in the perceived health of subjects with growth hormone deficiency. Its responsiveness to change is to be examined in subsequent studies.

[Anorexia nervosa and Crohn disease: diagnostic intricacies and difficulties. 3 cases]. / Anorexie mentale et maladie de Crohn: intrications et difficultés diagnostiques. Trois cas.

INTRODUCTION: Diagnosis of anorexia nervosa is readily evoked in young girls who associate decreased food intake and weight loss. Among the differential diagnoses of anorexia, it is important to underline the preponderant place occupied by Crohn's disease. OBSERVATIONS: We report three cases of young 18 to 25 year-old girls, initially treated for anorexia nervosa in a psychiatric department. Diagnosis of Crohn's disease was made within 5 to 13 years. The clinical and biological characteristics are reported for each case. COMMENTS: In view of the frequency of digestive disorders concomitant to eating disorders, the distinction between anorexia and digestive disease is particularly delicate. It requires appropriate somatic and biological assessment that, when confronted with clinical and/or biological atypia, may query the initial diagnosis of anorexia.

Molecular markers and long-term recurrences in a large cohort of patients with sporadic adrenocortical tumors.

Genetic alterations, such as loss of heterozygosity (LOH) at the 17p13 and 11p15 loci and overexpression of the insulin-like growth factor (IGF)-II gene, are associated with the malignant phenotype in sporadic adrenocortical tumors. A high risk of recurrence after surgery for adrenocortical tumors is predicted in cases with regional invasion or distant metastases. However, patients with localized tumors also have a high risk of recurrence. Reliable prognostic markers are required to identify subjects at high risk of recurrence. The aim of this study was to assess the prognostic value of three molecular markers (17p13 LOH, 11p15 LOH, and overexpression of the IGF-II gene) by assessing disease-free survival in a large series of adult patients with sporadic adrenocortical tumors. Adult patients (114) were prospectively followed up from diagnosis of the disease to June 1999 or to death. Malignancy was initially diagnosed in 18 patients (McFarlane stage III: n = 1 and stage IV: n = 17). The remaining 96 patients with localized adrenal disease at diagnosis (stage I: n = 60 and stage II: n = 36) were at risk of recurrence. Histological grade was assessed according to Weiss criteria, and tumors were classified into two groups (Weiss score or=4). Tumor samples were analyzed for LOH at the 17p13 and 11p15 loci and for IGF-II gene mRNA content. 17p13 LOH was a strong predictor of shorter disease-free survival in univariate analysis (P = 0.001; relative risk, 27), as were histological grade (Weiss score >or=4; P = 0.00001; relative risk, 15), 11p15 LOH (P = 0.004; relative risk, 9), tumor size (size >5 cm; P = 0.006; relative risk, 18), and overexpression of the IGF-II gene (P = 0.01; relative risk, 5). In a Cox proportional hazards regression model, histological grade (P = 0.04; relative risk, 4.2) and 17p13 LOH (P = 0.009; relative risk, 21.5) were independently associated with recurrence. Molecular markers, particularly 17p13 LOH, are predictive of long-term outcome in patients with sporadic adrenocortical tumors. In patients who have undergone curative surgery, routine assessment of these tumor markers is a useful complement to histological scoring for predicting recurrence and guiding decisions for subsequent follow-up and management.

Evaluation of plasma insulin-like growth factor binding protein-2 as a marker for adrenocortical tumors.

OBJECTIVE: Recent studies have pointed to the role of the IGF system in the pathogenesis of adrenocortical tumors, and it was shown recently that malignant adrenocortical tumors exhibit a high insulin-like growth factor binding protein (IGFBP)-2 content. Circulating markers specific for adrenocortical carcinoma are needed and the aim of this study was to evaluate plasma IGFBP-2 as a marker for these malignant tumors. METHODS: Plasma IGFBP-2 was determined in 51 patients referred to our institutions for adrenocortical tumors. Fifteen patients were in complete remission (group 1), eight patients had preoperative localized tumors (group 2) and 28 patients had metastatic tumors (group 3). Thirty-six healthy volunteers constituted a control group. RESULTS: There was no significant difference in plasma IGFBP-2 concentration between healthy controls and patients with complete remission or localized tumors. In contrast, patients with metastatic disease had significantly higher IGFBP-2 plasma levels than the control group (P<0.001). IGFBP-2 levels in patients with metastatic disease were inversely correlated with survival (R2=0.308; P=0.0026). In patients with localized tumors, there was no correlation between plasma IGFBP-2 concentration and tumor size or histological features. Analysis of individual IGFBP-2 concentrations showed that five patients (17.8%) with metastatic tumors had normal IGFBP-2 levels and two patients (13.3%) in complete remission had high plasma IGFBP-2 levels. The influence of nutrition, hormone secretion and treatment on IGFBP-2 levels was examined. Nutritional status was evaluated by determining IGF-I levels and was found to be normal in 16 patients (61.5%) with high IGFBP-2 levels, suggesting that malnutrition was not responsible for the high IGFBP-2 concentrations in these patients. IGFBP-2 levels did not differ significantly according to tumor secretion or mitotane treatment. In a follow-up study, plasma IGFBP-2 concentration remained stable in patients with complete remission or stabilized disease and was a late marker of tumor progression in patients with progressive metastatic disease. CONCLUSIONS: These results indicate that plasma IGFBP-2 is elevated in patients with malignant adrenocortical tumors and that the major factor affecting IGFBP-2 levels in these patients is tumor stage. However, plasma IGFBP-2 was less sensitive than expected for a tumor marker, which may limit its value in the diagnosis and follow-up of adrenocortical carcinoma.

Hormonal and metabolic effects of radiotherapy in acromegaly: long-term results in 128 patients followed in a single center.

Conventional radiotherapy is usually indicated in acromegaly when surgery fails to normalize GH secretion. However, the benefits of radiotherapy are delayed. This has raised questions about the potency of this treatment for reaching the safe GH level of 2.5 microg/L and for normalizing insulin-like growth factor I (IGF-I) levels, both of which are currently recommended as the therapeutic goal. To evaluate the long-term hormonal and metabolic effects of radiotherapy in acromegaly, a retrospective analysis was undertaken studying 128 patients followed for 11.5+/-8.5 yr (mean +/- SD) in a single center. The preradiation GH levels decreased as a function of time to 50% at 2 yr, 20% at 5 yr, and 10% at 10 yr. Basal GH levels below 2.5 microg/L were obtained in 7% of the patients at 2 yr, 35% at 5 yr, 53% at 10 yr, and 66% at 15 yr. A basal GH level below 2.5 microg/L was associated with suppression of GH below 2 microg/L during an oral glucose tolerance test and normalization of IGF-I levels in 9 of 10 patients. Preradiation GH levels was the sole factor that could predict the delay in GH fall to below 2.5 microg/L (P = 0.008). At the last follow-up, IGF-I levels were normalized in 79% of the patients (37 of 47; mean follow-up, 15.0+/-11.3 yr). In the 32 patients presenting with diabetes mellitus, improvement of glucose tolerance was associated with lower GH levels after treatment (35+/-78 microg/L in the group of 13 patients still presenting diabetes; 9+/-12 microg/L in the group of 4 patients with glucose intolerance; 5+/-8 microg/L in the 14 patients with normal glucose tolerance; P = 0.04). Ten years after termination of radiotherapy gonadotroph, thyreotroph and corticotroph deficiencies were observed in 80%, 78%, and 82% of the patients, respectively. In conclusion, conventional radiotherapy can reduce GH levels below the optimal level of 2.5 microg/L and normalize IGF-I levels in acromegaly. However, the incidence of late hypopituitarism is high, and the delay to obtain this safe GH secretory status can be long, depending on the preradiation GH level. These parameters should be considered when adjuvant therapy is needed after surgery.

Osteoporotic fractures at presentation of Cushing's disease: two case reports and a literature review.

Osteoporosis is a common complication of Cushing's disease/syndrome. Fractures can be the presenting manifestation. We report two cases and review 28 others from the literature. Clinical, laboratory, and absorptiometry data are reviewed; the vertebrae and ribs were the most common fracture sites, and osteoporosis reversal after treatment varied with age, gender, and symptom duration. The pathophysiology, which remains controversial, is discussed. A search for Cushing's disease/syndrome should be part of the routine evaluation of osteoporosis in children and adults, particularly men.

Alterations of the 11p15 imprinted region and the IGFs system in a case of recurrent non-islet-cell tumour hypoglycaemia (NICTH).

BACKGROUND AND OBJECTIVE: Non-islet-cell tumour hypoglycaemia (NICTH) is a rare disorder and has been explained by oversecretion of non mature IGF-II and dysregulation of the IGFs sytem. The mechanisms responsible for tumoural IGF-II overexpression in NICTH have been rarely studied. We report an extensive study of IGF-II and IGFBPs as well as chromosome 11p15 gene expression regulation in a case of a pleural fibrosarcoma in a 63-year-old patient presenting with NICTH. METHODS AND RESULTS: Abnormal high molecular weight precusor forms of IGF-II were present in the patient's serum and were associated with dramatic alterations in the circulating levels of IGF-I, IGF-II and their binding proteins, as well as an inhibition of the somatotroph axis. These alterations returned to normal following complete surgical removal of the tumour. No structural change in chromosome 11p15 region was apparent in the tumour. However, dysregulation of this imprinted region was demonstrated, leading to the loss of imprinting of the IGF-II gene associated with high IGF-II expression, and by contrast decreased level of expression of H19 and p57KIP2 genes. Recurrence of the tumour four years latter was not associated with hypoglycaemia or changes in the levels of circulating IGFs or IGFBPs, despite IGF-II overexpression by the tumour. This suggests that a large tumour volume is required to reach high enough levels to cause changes in the levels of circulating IGFs and IGFBPs, and to cause hypoglycaemia. CONCLUSION: These results suggest that a dysregulation of gene expression and imprinting of chromosome 11p15 region is associated with tumour growth and IGF-II overexpression in non-islet-cell tumour hypoglycaemia.

[Iodine 131 uptake by a bronchogenic cyst in a patient with differentiated carcinoma of the thyroid gland]. / Fixation à l'iode 131 d'un kyste bronchogénique chez une patiente ayant un carcinome différencié thyroïdien.

BACKGROUND: After thyroidectomy for differentiated thyroid carcinoma, extracervical uptake of iodine 131 is suggestive of metastasic dissemination. False positives can however occur. CASE REPORT: Differentiated thyroid carcinoma was found in a female patient with a non-functional nodule. Two years after subtotal thyroidectomy and an ablative dose of iodine 131, the whole body scan showed abdominal mediastinal uptake with low serum thyroglobulin level. Considering the possibility of tumor recurrence or lymph node metastasis, the mass was excised. The histology diagnosis was mediastinal bronchogenic cyst. DISCUSSION: To date, iodine uptake in a bronchogenic cyst has not been reported among other false-positives previously described. The pathophysiology of this iodine 131 uptake in a bronchogenic cyst is still unknown: the presence of NIS symporter or a protein which can organify iodine in the mucus cells of the cyst remains to be proven.

[Ovarian endometriosis cyst with iodine 131 uptake : first case of false positive in the follow up for differentiated thyroid carcinoma]. / Kyste endométriosique ovarien fixant l'iode 131 : premier cas de faux positif dans le suivi d'un cancer différencié de la thyroïde.

A whole body scan is performed after a radioiodine treatment in patients with differentiated thyroid carcinoma. This scan is useful coupled with thyroglobulin level for the patient's management. When unusual uptake is found, investigations have to be done to eliminate thyroid metastasis. A 28-year old woman underwent a total thyroidectomy for micro papillary carcinoma. Two years and a half after, ultrasonography of the neck showed a small lymph node in homolateral side of carcinoma. It was decided to begin treatment with iodine 131. The post-therapeutic scan showed an abnormal pelvic uptake. IRM found no osseous abnormality but an ovarian lesion. After surgery, histological diagnosis was an endometriosis cyst without thyroid or tumoral cells. Abdominal ou pelvic iodine false positive are rare. Ovarian cysts may be the cause of false positive radioiodine uptake. Endometriosis cyst was not previously described and the mecanism of iodine uptake is not clear.

Outcome in patients with adrenal incidentaloma selected for surgery: an analysis of 88 cases investigated in a single clinical center.

OBJECTIVE: The study was designed to evaluate the clinical, endocrinological and radiological parameters used to investigate adrenal incidentalomas and select patients for surgery. DESIGN AND METHODS: An analysis of 88 consecutive patients with adrenal incidentaloma selected for surgery and investigated in a single clinical center was performed. RESULTS: Mean (+/-s.d.) age of the patients was 53+/-14 years. Fourteen (16%) of the adrenal incidentalomas were malignant tumors (2 adrenocortical carcinomas, 3 metastases, 4 adenocarcinomas, 4 sarcomas and 1 mesenchymoma), 10 (11%) were pheochromocytomas, 32 (36%) were non-secretory benign adrenal adenomas and the remaining were benign adrenal (n = 8; 9%) or extra-adrenal (n = 24; 27%) masses. Endocrinological investigations revealed 1 Conn adenoma, 4 tumors responsible for Cushing's syndrome or silent hypercortisolism and 1 androgen secreting tumor. Abnormalities of endocrine evaluations were observed in the 2 malignant adrenocortical carcinomas. Elevated 24-h urinary metanephrine levels were observed in the 9 pheochromocytomas tested. Complications of surgery occurred in 14% of the cases. Regardless of the endocrine status, parameters associated with malignant tumors were: older age (mean age of patients harboring malignant tumors vs patients with benign incidentalomas: 62+/-17 years vs 52+/-13 years, P = 0.005), weight loss (39% vs 7%, P = 0. 005), and mass diameter greater than 60mm (69% vs 15%, P < 0.001). By multiple logistic regression analysis malignant tumors were associated with increased age, diameter greater than 60mm and bilateral masses. CONCLUSION: This study points to a high rate of pheochromocytomas and malignant tumors in patients selected for surgery. This high rate differs from some previous reports and might be explained by the criteria used to select patients for surgery. Among these two groups of tumors, careful systematic endocrinological investigations allow the detection of altered secretion in the vast majority - if not all - malignant tumors of adrenal origin and pheochromocytomas. Only 5% of the incidentalomas below 30 mm selected for surgery in this study were malignant, in keeping with the use of this criteria as an important parameter to select patients with normal hormonal investigations for careful follow-up.

Goldmann perimetry in acromegaly: a survey of 307 cases from 1951 through 1996.

OBJECTIVE: Because visual pathway lesions are a common complication of pituitary tumors, visual field examinations in patients with acromegaly were studied. Proportion and outcome of visual field defects in patients with acromegaly were evaluated. DESIGN: Large, retrospective case series. PARTICIPANTS: We reviewed 307 cases of acromegaly seen from 1951 through 1996 at a single referral center. METHODS: Kinetic visual field testing had been performed with the Goldmann perimeter, and the frequency of visual field defects and their correlation with other clinical manifestations and characteristics of the adenoma were examined. MAIN OUTCOME MEASURES: Repeat visual field examinations. RESULTS: Of the 307 patients included in the analysis, a visual field defect that could be attributed to the pituitary adenoma was observed in 62 (20.2%) during follow-up. Visual field defects were bilateral in 38 (61.3%) of these cases. Patients with visual field abnormalities were significantly younger (P = 0.04), had larger tumors (P < 0.001), had more suprasellar extensions (P < 0.001), and had higher levels of growth hormone in their serum (P = 0.04) than patients free of visual field defects. At the end of the follow-up period, visual field examination remained abnormal in 32 (10.4%). Return to a normal visual field examination after treatment was more frequently observed in patients who were less than 40 years of age at the time of diagnosis (P = 0.004). Secondary empty sella syndrome was the main cause of visual field defects after treatment. Abnormal visual field, either at the time of diagnosis or during follow-up, decreased from 27% of patients between 1951 and 1975 to 15.4% of patients between 1976 and 1996, when modern neuroimaging techniques became available. CONCLUSIONS: Endocrinologic and neuroimaging follow-up of patients with acromegaly should be accompanied by ophthalmic assessment. Factors predictive of visual field defects have been identified.

[Pituitary metastases. 3 cases]. / Les métastases hypophysaires. 3 observations.

OBJECTIVE: Review cases with hyothalamo-hypophyseal metastases which raise complex therapeutic situations. PATIENTS AND METHODS: Files of patients hospitalized from 1992 to 1996 at the Cochin Hospital Endocrinology unit were compared with data reported in the literature. RESULTS: We had patients with pituitary metastases from breast cancer and 69 cases have been reported in the literature: 72 patients, 57% men and 43% women, mean age 57.2 +/- 12.3 years. The primary tumor was identified in 58.3% of the cases. The primary cancer was unknown and revealed by the pituitary metastasis in 41.7% of the cases. The primary tumor was a breast cancer in 31.9% of the cases, and lung cancer in 30.5%. The main clinical signs were vision disorders (40.3%) and diabetes insipisus (29.1%). The principal differential diagnosis was hypophyseal adenoma. Short term prognosis was not good. DISCUSSION: These data are in agreement with the older literature excepting the high prevalence of primary cancers revealed by pituitary metastasis, probably a result of progress in hypophyseal exploration techniques. CONCLUSION: The prevalence of pituitary metastasis will continue to increase with longer survival in cancer patients. Metastasis should thus be entertained as a possibility in patients with a hypothalamo-hypophyseal tumor, particularly after the age of 59, in order to hasten diagnosis and provide early treatment.

[Imaging of adrenal incidentalomas]. / Imagerie des incidentalomes surrénaliens.

Imaging plays an important role in evaluating fortuitously discovered adrenal masses. These unanticipated masses (incidentalomas) are usually discovered at CT examinations. Although most incidentally discovered adrenal tumors are non-hyperfunctioning adenomas, they must be distinguished from primary or secondary malignant lesions. Recent studies have established that both unenhanced CT scans and chemical shift MR imaging can accurately differentiate benign adenomas from nonadenomatous masses by features that reflect the large amount of lipid found in most adenomas and absent in most nonadenomas. We review here the current imaging issues concerning adrenal incidentalomas including diagnostic approaches and management.

[POEMS syndrome with primary adrenocortical insufficiency]. / Syndrome de POEMS avec insuffisance surrénalienne primaire.

BACKGROUND: POEMS syndrome is a multisystem disorder. It usually presents as severe polyneuropathy and monoclonal gammapathy associated with endocrinopathies, organomegaly, skin hyperpigmentation. CASE REPORT: A patient with POEMS syndrome developed primary adrenocortical deficiency revelated by asthenia and hyperpigmentation. Hydrocortisone replacement therapy produced a rapidly beneficial effect on asthenia and hyperpigmentation. DISCUSSION: Although asthenia and skin pigmentation are common in POEMS syndrome, adrenocortical deficiency is seldom reported. This endocrinopathy might be underestimated in POEMS patients. We suggest systematic screening with a rapid ACTH test in all POEMS patients in order to detect underlying adrenal deficiency.

[Diabetes insipidus with a hypothalamo-hypophyseal morphologic anomaly during a pregnancy]. / Diabète insipide avec anomalie morphologique hypothalamo-hypophysaire au cours d'une grossesse.

BACKGROUND: Diabetes insipidus is uncommon in pregnancy. Despite physiological modifications in hydroelectrolytic balance during normal pregnancy, the capacity of the kidney to concentrate urine is preserved, partially due to lower vasopressin secretion. CASE REPORT: A young woman developed diabetes insipidus during the third trimester of normal pregnancy. The disease regressed totally after delivery. However, magnetic resonance imaging revealed a persistent expansive intrasellar image with a high-intensity signal. DISCUSSION: Onset of diabetes insipidus is usually rapidly progressive in pregnancy. Occurring generally during the third trimester in normal pregnancies, diabetes insipidus is generally well tolerated and responds to dDAVP, usually without pituitary abnormally, and regresses after delivery. Two types are distinguished: partially latent diabetes insipidus occurring during pregnancy and due to a central rather than nephrogenic origin; and excessive vasopressinase activity leading to diabetes insipidus usually associated with liver anomalies and high frequency of pre-eclampsia. During normal pregnancy, the size of the anterior pituitary increases and the normal high-intensity signal in the posterior pituitary seen on MRI usually regresses or disappears. In diabetes insipidus, the posterior pituitary hypersignal image generally disappears, reflecting reduced vasopressin storage. Few observations of diabetes insipidus occurring during pregnancy have been reported with morphological explorations. Most have described a "normal" aspect of the pituitary, specifically in the post partum period. In our patient, the weak vasopressin response to the end of water restriction at post partum when the diabetes insipidus symptoms had disappeared would suggest partial central diabetes insipidus revealed by pregnancy. Other pathologies involving this region could also be involved due to the unusual and persistent sellar image, with an expansive process showing a high intensity signal on MRI. An asymptomatic craniopharyngioma cyst was hypothesized and would be more compatible with the observed symptoms.

[Failure of synthcen to diagnose corticotropin insufficiency]. / Pièges de l'épreuve au synacthène immédiat dans l'insuffisance corticotrope.

It is commonly believed that a normal cortisol response to exogenous ACTH (synacthen test) reliably indicates, with the exception of states of acute ACTH deprivation, the integrity of hypothalamic-pituitary-adrenocortical function. Published evidence has shown that the synacthen test is not sensitive enough to reveal partial ACTH deficiency leading to a subnormal ACTH response to stress. We report a patient who maintained a normal response to exogenous ACTH stimulation despite symptomatic chronic ACTH deficiency in unstressed conditions. A normal cortisol response in the synacthen test does not therefore exclude the possibility of clinically relevant ACTH deficiency.

[Bilateral video-endoscopic adrenalectomy in Cushing's disease. Experience in 24 patients]. / Surrénalectomie bilatérale vidéo-endoscopique dans la maladie de Cushing. Expérience chez 24 patients.

The purpose of this study was to compare the results of bilateral laparoscopic adrenalectomy (BLA) to bilateral open adrenalectomy (BOA) in the treatment of Cushing's disease. Twenty-four patients (23 Cushing's disease, 1 congenital adrenal hyperplasia) were divided into 3 groups. Group 1 patients (n = 15) underwent BCA using the lateral transabdominal approach, Group while 2 patients (n = 9) underwent laparoscopic adrenalectomy on one side and conventional open adrenalectomy on the contralateral side. Groups 1 and 2 were compared retrospectively to 15 patients (Group 3) who underwent BOA as part of larger series of 61 patients. There was no difference in the degree of hypercortisolism in the 3 groups. At the beginning of the experience, the duration of surgery was longer in Groups 1 and 2 compared to the open surgery group, but this difference subsequently decreased during the study. There was no difference in intraoperative blood loss or transfusion rate. Group 1 patients experienced fewer wound and intraabdominal complications and less postoperative pain, shorter hospitalization, and quicker recovery than groups 2 and 3 patients. Technically obesity and tissue fragility are easily overcome by the laparoscopic approach. BCA also achieves success rate of hypercortisolism correction. In conclusion, BLA is the surgical procedure of choice for the treatment of Cushing's disease when surgical therapy is indicated.

[Possibilities and limits of surgical treatment of malignant adrenal cortex carcinomas. Apropos of a series of 74 cases]. / Possibilités et limites du traitement chirurgical des cortico-surrénalomes malins. A propos d'une série de 74 cas.

The aim of this study was to report our experience of 74 patients operated on for adrenocortical carcinomas in the last 15 years with particular reference to survival rate and prognostic factors. The tumors were secreting in 78% of the cases and non secreting in 22% of the cases, encompassing stade I: 7%, stade II: 43%, stade III: 21%, stade IV: 29%, according to the MacFarlane classification. All patients were operated on whatever the stade. Adrenalectomy with regional lymphadectomy was performed in 50% of the cases. Other procedures included extended adrenalectomy with nephrectomy (n = 27), hepatectomy (n = 7), and desobstruction or resection of inferior vena cava (n = 13). The resection was curative for 66% of the patients and palliative in 34% of the patients. Local recurrences were operated on in 13 patients. Operative mortality was 4%. Stade I and II had significantly the best actuarial survival rates (78 and 62% at 5 years, respectively), when compared to stade III (27%). Extension to the vena cava was not considered as a contra indication even in cases of massive extension. The survival rate of patients with local recurrences was the same as patients wich stade III: 32% at 5 years. Stade IV tumors had the poorer prognosis with a survival rate of 5% at 3 years. In this group, some patients may have benefited from mitotane. Further studies are mandatory to appreciate the benefit of adjuvant therapy with mitotane and palliative chemotherapy.