Subtotal Adrenalectomy Research Articles

Bilateral adrenalectomy with autotransplantation of adrenocortical tissue or unilateral adrenalectomy: treatment options for pheochromocytomas in multiple endocrine neoplasia type 2A.

Surgical strategies for pheochromocytomas in patients with multiple endocrine neoplasia (MEN) type 2 syndrome have been controversial. The purpose of this study is to review the current status of patients with MEN 2 who underwent adrenalectomy with or without adrenal autotransplantation. We studied 15 patients with MEN 2A who underwent adrenal surgery between 1981 and 1992. The follow-up survey included physical examination and biochemical determinations. The median period from initial surgery to follow-up was 54 months (range, 0-145 months). Initial bilateral total adrenalectomy was performed on seven patients, and subtotal adrenalectomy was carried out on two. Among six patients who initially underwent unilateral adrenalectomy, four had remained normotensive (median follow-up, 61 months), whereas the other two patients had to undergo reoperation on the contralateral side because of recurrent symptoms. Two patients were suspected of having had a relapse of the disease after total adrenalectomy. Seven patients underwent adrenal autotransplantation; however, none of them were able to discontinue glucocorticoid replacement therapy. In MEN 2A patients having large pheochromocytomas on only one side, unilateral adrenalectomy can be a suitable alternative to bilateral adrenalectomy in terms of blood pressure control and preservation of adrenocortical function. The attempt to preserve adrenocortical function by autotransplantation is discouraged.

Long term follow-up of Cushing's disease treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy.

Subtotal adrenalectomy was given to 10 adult patients with Cushing's disease, concurrently with or following therapeutic regimen by long term reserpine administration and pituitary irradiation. In the present study, we describe long term follow-up results. Two patients died after the operation due to acute adrenal crisis and pneumonia, respectively. The other 8 patients achieved clinical and biochemical remissions and were followed for long term. Three patients relapsed 9, 14 or 17 years after achieving remission, two patients developed hypopituitarism 12 or 20 years after and one died of cerebral vascular accident at 64 years, 5 years after the remission. The remaining 2 patients maintained remission for 10 or 18 years, respectively. During the remission periods of 0.5 to 20 years with a mean of 10.1 +/- 6.7 years, 6 of 7 patients examined by 1 mg overnight dexamethasone test showed normal suppressibility of plasma cortisol. Provocative tests of plasma GH by l-arginine infusion and/or insulin-induced hypoglycemia were performed in 6 patients in the early remission period. All of 5 patients in the arginine infusion test and 3 of 5 in the insulin-induced hypoglycemia test showed normal responses. Furthermore, to facilitate prediction of long term response or failure to our therapeutic regimen, long term reserpine administration and pituitary irradiation, pretreatment clinical and biochemical characteristics were analyzed retrospectively in 3 divided groups; the present 10 patients treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy, 11 patients achieving long term remission treated by our regimen alone, and 7 patients failed with our regimen alone. There were no significant factors predictive of response to our regimen. These findings suggest that subtotal adrenalectomy does not lead favorable outcome, however, reserpine administration shows usefulness to improve pituitary functions in treating Cushing's disease.

Development of Nelson's syndrome in a patient with recurrent cushing's disease: Analysis of secretory behavior of the pituitary tumor

A markedly cushingoid 32-year-old man presented to Queens Hospital Center with headache, hyperpigmentation, and visual field loss. Twelve years earlier, he had undergone subtotal adrenalectomy for Cushing's disease, but symptoms of hypercortisolemia promptly recurred. Workup revealed the presence of a large, expanding intrasellar mass, plasma ACTH levels between 3,000 and 10,000 pg/ml, and markedly elevated cortisol levels. The secretion of ACTH (mainly ACTH 1-39-like peptide) by the pituitary tumor showed neither diurnal periodicity nor response to a variety of pharmacologic agents known to affect ACTH secretion. The patient demonstrates a rarely observed presentation of Nelson's syndrome, with aggressive adrenotropic pituitary tumor growth even in the presence of chronic hypercortisolemia.

TRILOSTANE IN THE TREATMENT OF PRIMARY ADRENOCORTICAL MICRONODULAR DYSPLASIA (PAMD)

A rare cause of Cushing's syndrome is a pituitary-independant nodular dysplasia of the adrenal cortex. A 4 year old girl presented with advanced clinical features of Cushing's syndrome. Abdominal CT-scan and adrenal scintigraphy revealed bilateral adrenal hyperplasia whereas a cerebral CT-scan was normal. Serial determinations of plasma ACTH together with dynamic tests using dexamethasone, metyrapone and vasopressin indicated a primary, autonomous, adrenal dysfunction. Treatment with Trilostane - an inhibitor of 3β-hydroxysteroid-dehydrogenase - in a dose of 50 mg t.i.d. was effective, and cortisol excretion was instantly reduced to a low normal level with only a slight increase in ACTH. No side-effects of this treatment were seen and the clinical features of the syndrome regressed. The therapeutic index of Trilostane was small, however. Serum levels of adrenal androgens remain increased therefore a long-term treatment in this young girl was considered to be unfavourable. At laparotomy both adrenals were almost normal in size and a subtotal adrenalectomy was performed. Microscopically, the picture was typical for PAMD with normal structure of the cortex between the micronodules. Trilostane seem to be effective in improving clinical and some of the biochemical parameters even in a severe case of PAMD but its use may be limited to a short-term preoperative treatment.

Plasma steroid hormones in Cushing's syndrome: their relation to cause and clinical manifestations (author's transl)

For the purpose of studying the relation of diversities of basic lesion as well as clinical manifestation to hormonal abnormality in Cushing's syndrome, 12 kinds of steroid hormones was simultaneously measured in plasma by using 2 types of Sephadex LH-20 column chromatography in a total of 30 patients comprosing 28 cases of Cushing's syndrome and 2 cases of adrenocortical carcinoma which had abnormal plasma steroid hormone levels without sign or symptom of Cushing's syndrome. In the group of Cushing's syndrome were included cases of pituitary ACTH-dependent hyperplasia (Hp.), adrenocortical adenoma(Ad.), bilateral nodular hyperplasia, ectopic ACTH syndrome as well as recurrent Cushing's syndrome following subtotal adrenalectomy. Twelve steroids measured in plasma were pregnenolone(Preg)., 17-OH pregnenolone(17Preg.), progesterone(Prog.), 17-OH progesterone(17Prog.), 11-deoxycorticosterone (DOC), corticosterone(B), aldosterone(Ald.), 11-deoxycortisol(S), cortisol(F), dehydroepiandrosterone(DHA), androstendione(A-dione) and testosterone(T), including precursors (Prec.: Preg., 17-preg., Prog., 17-prog.) as well as hormones belonging to the 3 systems in the biosynthetic pathways of steroid; i.e., glucocorticoids(Glu.C.'s: S,F), mineralocorticoids(Min.C.'s: DOC, B, Ald.) and sex steroids(And.'s: DHA, A-dione, T). In addition, steroidogenesis in isolated adrenal cells obtained surgically from patients with Cushing's syndrome due to Hp. and Ad, was observed. The results were as follows: (1) In cases due to Hp., plasma levels of Glu.C's and And.'s were slightly elevated, while levels of Min.C.'s were within the normal range. On the whole, however, the 3 systems were well balanced. (2) In cases due to Ad., elevated secretion of Glu.C.'s and Min.C.'s was observed, while secretion of And.'s was depressed. Among the 3 fractions of And.'s, depression of DHA and A-dione was characteristic of Ad.. (3) Elevation of And.'s in Hp. and Min.C.'s in Ad. in addition to elevation of Glu.C.'s was in fair correlation with moderate virilism in Hp. and with hypertension and hypokalemia in Ad. respectively. (4) In vitro experiments revealed that Ad. produces not only F but all 12 steroids hormones and that increased DOC and depressed DHA secretion reflected in their plasma levels were the characteristics of Ad. in steroidogenesis. Furthermore, isolated adenoma cells were found to produce Ald. at a higher rate than normal or hyperplasia adrenal cells. This finding may suggest that an intermediate type between Cushing's syndrome and primary hyperaldosteronism can exist in cases of adenoma. (5) In ectopic ACTH syndrome, plasma levels of Glu.C.'s, Min.C.'s and And.'s were equally but more markedly elevated as compared with Hp.. The increase of B was characteristic of this disorder and, coupled with a marked increase of F, seems to be the main cause of hypokalemic alkalosis frequently associated with this syndrome. (6) Nodular hyperplasia was accompanied by elevated Min.C...

Results of treatment in 68 patients with idiopathic aldosteronism in Japan.

Based on the results of a nationwide questionaire survey during the decade from 1963 in Japan, 68 cases of idiopathic aldosteronism were collected in order to investigate the actual treatment for this disorder performed in Japan. Idiopathic aldosteronism was usually treated by unilateral or subtotal adrenalectomy. Unlike resection of adenoma, adrenals surgery were not so effective and only 50% of cases obtained a favourable result. Surgical treatment was effective in 60.5% of cases of nodular hyperplasia while effective only in 26.3% of cases of diffuse hypreplasia. Opinions from answers to the questionaires favoured total bilateral adrenalectomy or subtotal adrenalectomy either with or without spironolactone administration.

A Survey of 162 Cases Treated by Adrenal Surgery

Adrenal surgery was performed on 162 cases of various diseases, including 67 primary aldosteronism (PA), 22 adrenal Cushing's syndrome (ACS), 19 hypophyseal Cushing's syndrome (HCS) and 32 pheochromocytoma (Pheo), at Tohoku University School of Medicine from December 1956 to March 1976. The diagnosis, treatments and prognosis of these cases were surveyed. The diagnostic accuracy of pneumoretroperitoneum with tomography was 63% in PA, 95% in ACS and 93% in Pheo, while that of adrenal scintigraphy was 86% in PA and 100% in ACS. Scintigraphy and aldosterone assay in venous blood samples the most reliable for the localization of PA. The unilateral approach was the most suitable procedure for the removal of adrenal tumors in PA, ACS and single Pheo within a few hundred grams. The anterior approach was preferable for single Pheo over a few hundred grams and bilateral or multiple Pheo. As regards the treatment for HCS, good results were obtained by a combination therapy of bilateral subtotal adrenalectomy, hypophyseal radiation and reserpine administration. Two cases of Pheo were inoperable. There were 3 deaths immediately after the removal of Pheo. We later investigated the blood pressure in successfully treated cases. Hypertension was noticed in 18% of PA and 10% of ACS. No hypertensive case was found in Pheo.

Application of lead-hematoxylin and methyl blue staining for the identification of ACTH and ICSH cells in the adenohypophysis of the scincid lizard, Mabuya carinata (Schneider)

On the basis of their morphology, tinctorial affinity and distribution in the pars distalis, two types of acidophilic (A1 and A2) and four types of mucoid (B1–B4) cells were distinguished in the pars distalis of the scincid lizard, Mabuya carinata (Schneider). The lead-hematoxylin (PbH)-positive cells included two distinct cell types (B3 and B4), only one of which (B3) was stained by methyl blue (MB). Following castration B2 and B3 cells exhibited significant hyperactivity which possibly indicated their involvement in gonadotropin secretion. As in certain other reptiles, the activity of B3 cells can be correlated with that of the testicular interstitial cells and the epididymis suggesting that B3 and B2 cells were possibly the source of interstitial cell-stimulating hormone (ICSH) and follicle-stimulating hormone (FSH), respectively. B4 cells exhibited conspicuous hypertrophy, hyperplasia, and degranulation after subtotal adrenalectomy or after metopirone administration indicating that this cell type was responsible for corticotropin (ACTH) secretion. The possible significance of the response of the other cell types to the experimental treatments undertaken in the present study is discussed.

Follow-up studies of 73 patients with adrenal surgery (author's transl)

From 1963 to 1977, 101 patients underwent adrenal surgery at our clinic. Of these, 73 patients for whom time more than 2 years had elapsed after surgery were followed-up.Twenty-six patients with primary aldosteronism caused by adrenal adenoma had unilateral adrenalectomy. One was lost to follow-up. One died of unknown cause 2 years after operation. The remaining 24 patients are living and engaging in ordinary activity. Blood pressure has almost been normalized in all patients. Symptoms and signs referrable to primary aldosteronism have disappeared in all patients.Out of 14 patients with pheochromocyuoma, 12 had a single adrenal tumor removed by adrenalectomy and contralateral tumor extirpation, and one with multiple pheochromocytoma had unilateral adrenalectomy followed by tumor extirpation. One died of diabetes mellitus 2 years after operation. Hypertension and symptoms have disappeared in 11 patients, but they have recurred in 2 patients.Adrenal cortical adenoma was the cause of Cushing's syndrome in 16 patients who had unilateral adrenalectomy. One patient died one week after operation. Hypertension and symptoms have disappeared except in one patient. Two-thirds of the patients failed to discontinue steroid replacement therapOut of 14 patients with Cushing's syndrome by adrenal cortical hyperplasia, 11 underwent bilateral adrenalectomy. Eight of these have no hypertension or symptoms on corticosteroid replacement and are engaging in ordinary activity, but one patient has persistent hypertension. Nelson's syndrome has developed in the remaining 2 patients. One patient had subtotal adrenalectomy and has no hypertension or symptoms. Two patients had unilateral adrenalectomy combined with pituitary irradiation. One died of pneumonia 8 years and 5 months after operation. The other one is free from any symptoms and signs.One patient with Cushing's syndrome due to adrenal carcinoma had laparotomy and died of liver metastasis.One patient with adrenogenital syndrome due to adrenal adenoma had adrenalectomy. Hypertension and hursutism has subsided gradually and has regular menstruation.

Hypothalmic-Pituitary-Adrenal function of untreated longterm survivors following subtotal adrenalectomy for Cushing's disease

Hypothalamic-Pituitary-Adrenal function was studied in 5 longterm survivors (11 to 24 years) who achieved independence of steroid replacement after subtotal adrenalectomy for Cushing's disease. Adrenal function seemed adequate when plasma cortisol values were measured, but the response of the adrenal gland to insulin-induced hypoglycaemia and to tetracosactrin stimulation was subnormal in all 5. It is concluded that the function of the hypothalamic-pituitary-adrenal axis never fully recovers and that adrenal crisis, at times of stress, remains a hazard indefinitely in these patients.

Idiopathic edema: Pathogenesis, clinical features, and treatment

Previously published findings are reviewed and new data are presented relating to the clinical features, pathophysiology, and treatment of the idiopathic edemas, a group of disorders diagnosed by exclusion of the known causes of edema. The disorders occur almost exclusively in women, show diurnal (postural), not “cyclic” fluctuations in severity, often cause discomfort and occasionally pain, and may be aggravated by prolonged orthostasis, hot environments, menses, and some drugs. Among the pathophysiologic factors known to be important in edema formation, in general: (1) Hypoproteinemia is more commonly the result than the cause of idiopathic edema; (2) elevated capillary hydrostatic pressure is seldom the primary cause, except perhaps in rare cases of occult congestive heart failure; (3) changes in tissue pressure may limit the progression of, but do not initiate, the edema; (4) changes in capillary wall permeability and (5) changes in capillary diffusion area, perhaps because of excessive dilatation of precapillary sphincters appear to be of importance; (6) abnormalities of lymphatic flow are not demonstrable; and (7) the upright posture is an important contributor to the excessive transudation in over 80% of the patients studied (“orthostatic edema”) but not in the remaining minority of patients (“nonorthostatic edema”). By balance studies with constant diets and by an abbreviated “posture test”, it has been shown that (A) the majority of patients with “orthostatic edema” have excessive orthostatic sodium (Na) retention that results from an excessive orthostatic fall in glomerular filtration rate, frequently associated with excessive renal tubular reabsorption of the subnormal filtered Na load because of orthostatic hyperaldosteronism, and (B) about 30%–40% of patients with orthostatic edema have “orthostatic water retention”, demonstrable by an inability to excrete more than 55% of a 20 ml/kg water load during 4 hr in the upright posture. In most of these patients but not in most other types of edema, orthostatic excretion of the water load is restored to normal by ethanol (a known inhibitor of vasopressin release). Improvement in orthostatic renal excretion by external compression of the legs and persistence of excessive orthostatic changes in leg volume despite restricted Na intake support microscopic evidence that a capillary “leak” may be the primary cause of most orthostatic edemas. Reduced dopamine excretion has been reported and linked to the causation of some types of idiopathic edema. Obesity is often associated with edema that differs from other idiopathic edemas in that it usually disappears with caloric restriction and weight loss. In occasional patients, undue sensitivity to heat may play a role as important as that of posture. Treatment of idiopathic edema includes avoidance of excessive salt intake, reduction of the duration of standing and sitting, and administration of conventional diuretics, preferably at 7 or 8 p.m., followed by recumbency for several hours before sleep. Hyperaldosteronism has responded to subtotal adrenalectomy (which is not recommended) and constitutes a strong indication for the use of spironolactone, which facilitates recumbent excretion of Na retained in the upright posture. Sympathomimetic amines (ephedrine, phenylephrine, and preferably dextroamphetamine) are the only agents that will usually significantly reduce the excessive weight gain from morning to evening (the hallmark of orthostatic edema), perhaps by preventing excessive capillary pooling and transudation. They have been used safely, effectively, and without loss of their efficacy, for up to 20 yr in several patients. Elastic stockings and garments are occasionally useful and regular exercise, especially swimming, may be beneficial.

Diagnostic and therapeutic problems in Cushing's syndrome. Series of 7 cases.

A series of 7 patients (all females) with Cushing's syndrome are presented. The syndrome was due to an adenoma of the cortex in 4 cases, to a carcinoma in 1 and to bilateral adrenal hyperplasia in another. In the 7th patient the cortical hyperplasia was due to a bronchogenic carcinoma (ectopic production of ACTH). Surgery was performed in all patients except 1. In 5 patients (adenoma, carcinoma) the diseased adrenal was removed. In 1 patient a left total and a right subtotal adrenalectomy were performed. Out of those 7 patients 4 survived and 3 died. 1 died a few hours after surgery, another 3 months after operation and the last one was never operated on.

Nelson's Syndrome

Comments and Correction1 May 1977Nelson's SyndromeJ. L. GABRILOVE, M.D., G. L. NICOLIS, M.D., PH.D.J. L. GABRILOVE, M.D.Search for more papers by this author, G. L. NICOLIS, M.D., PH.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-86-5-665_1 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the editor: From their study of Nelson's syndrome Moore and colleagues (1) reported nine patients with the disorder out of 120 patients treated for Cushing's syndrome. Of these nine only two had received prior pituitary irradiation; one having received 3000 rad, the other 5000. They cite a case from Salassa's and associates' series of pituitary tumors in patients with Cushing's syndrome who was reported without comment to have developed a pituitary tumor subsequent to pituitary irradiation with 2400 rad and bilateral subtotal adrenalectomy. Most radiotherapists would consider that dosages of 2400 to 3000 rad to the pituitary at the...

Selection of patients and operative approach in primary aldosteronism.

A system for discriminating between adrenal adenoma and hyperplasia based on the levels of aldosterone production, plasma renin concentration, severity of electrolyte disturbances, plasma aldosterone patterns during recumbency and after assuming erect posture, and 131I-19-iodocholesterol scan has been developed. Indicated for operation are patients with adenomas whose elevated blood pressure cannot be continuously controlled with usual doses of medication and patients with documented deterioration of target organ function. Adrenalectomy has been performed 83 times in 81 patients with a diagnosis of primary hyperaldosteronism. Results of excision of adrenal adenomas have been excellent with significant lowering of blood pressure in all cases and cure of hypertension in over 60%. Results of total or subtotal adrenalectomy for hyperplasia have been poor with almost all patients still requiring medication for hypertension. Adenomas have always been unilateral, and usually can be localized so that unilateral exploration is curative. Therefore, we have tried to distinguish preoperatively between adenoma and hyperplasia. Anterior transperitoneal adrenalectomy has been effective with few complications, and no postoperative hypercortisolism after unilateral adrenalectomy for adenoma. The unilateral extraperitoneal approach gives shorter morbidity and potentially fewer serious complications.

Treatment of Cushing's disease with reserpine and pituitary irradiation.

Eighteen patients with Cushing's disease were treated with reserpine and pituitary irradiation. Complete remission was obtained in 9 out of 18 patients after reserpine treatment of 1-2 mg per day for a mean period of 20.4 months, and pituitary irradiation with a mean of 5,865 rads. In another 9 patients, reserpine 0.8-2 mg per day for a mean period of 22.5 months, and pituitary irradiation with a mean of 6,650 rads, were employed. Of these 9 patients, an additional subtotal adrenalectomy was carried out in 6 patients who are now in complete remission. Because of severe psychic symptoms resulted from the original disease in 2 of the remaining 3 patients, subtotal adrenalectomy was performed first and pituitary irradiation and reserpine treatment followed. Remission was eventually obtained in these 2 cases. One patient refused the operation, and thus had little clinical remission. All of the 17 cases in remission were followed up for periods of 6 months to 10 yr. During this time, only one case which had responded to reserpine and pituitary irradiation relapsed, but regained remission following resumption of therapy. Another died of cerebral glioblastoma 4 yr after remission of the disease. It was noteworthy that endocrinologic data including: plasma levels of ACTH and 11-OHCS, suppressibility by dexamethasone, responses of plasma GH to arginine and to insulin loads, and diurnal rhythm of plasma 11-OHCS were nearly normal in a considerable number of the cases in remission. Effectiveness of the combined therapy with reserpine and pituitary irradiation for treating Cushing's disease may support a working hypothesis that reserpine acts through some as yet unknown mechanism to correct a presumed central nervous disorder, while suitable pituitary irradiation probably corrects the pituitary dysfunction directly.

Surgical management of pheochromocytoma in children

A review is presented of seven children under the age of 18 yr who underwent surgical excision of pheochromocytomas. Twelve partial or total adrenalectomies were performed. Because of the high occurrence of bilateral tumors (70%), it is recommended that a biopsy be considered on the contralateral gland even though it appears normal at operation. Total adrenalectomy is recommended whenever a pheochromocytoma is present in the adrenal gland of a child since recurrence of the tumor in the residual gland after a subtotal resection is likely; the two recurrent tumors in this series occurred after subtotal adrenalectomy. Identical female twins had associated medullary thyroid carcinoma and pheochromocytoma (Sipple's syndrome). Two male siblings each had bilateral pheochromocytomas. Only one of the 12 tumors removed in this series was invasive. Aortography and selective venous catheterization were the most helpful diagnostic studies in establishing the diagnosis of pheochromocytoma. A review is presented of seven children under the age of 18 yr who underwent surgical excision of pheochromocytomas. Twelve partial or total adrenalectomies were performed. Because of the high occurrence of bilateral tumors (70%), it is recommended that a biopsy be considered on the contralateral gland even though it appears normal at operation. Total adrenalectomy is recommended whenever a pheochromocytoma is present in the adrenal gland of a child since recurrence of the tumor in the residual gland after a subtotal resection is likely; the two recurrent tumors in this series occurred after subtotal adrenalectomy. Identical female twins had associated medullary thyroid carcinoma and pheochromocytoma (Sipple's syndrome). Two male siblings each had bilateral pheochromocytomas. Only one of the 12 tumors removed in this series was invasive. Aortography and selective venous catheterization were the most helpful diagnostic studies in establishing the diagnosis of pheochromocytoma.

Isolation of aldosterone urinary metabolites, glucuronides and sulfate

A simple method is described for the simultaneous isolation of aldosterone-18-glucuronide and tetrahydroladosterone-glucuronide from the urine of normal subjects following intravenous administration of purified 3H-aldosterone. Using the two isolated conjugates as recovery indicators, the urinary excretion rates of the two compounds determined in normal subjects were within the ranges established by previous investigations. A method is also established for the isolation and estimation of urinary aldosterone sulfate. The mean urinary excretion rate of this compound was 2.09 ± 0.6μg/24 h in normals and 2.83 ± 0.45μg/24 h in patients with benign essential hypertension. In one instance of confirmed primary aldosteronism investigated, the pre-operative urinary aldosterone sulfate was 13.76μg/24 h and the same compound was undetectable in the urine of the patient after sub-total adrenalectomy.

Role of renin and aldosterone in hypertension due to a renin-secreting tumor

A renin-secreting renal tumor is a rare cause of curable hypertension characterized clinically by hypertension, hypokalemia, hyperaldosteronism and increased plasma renin activity (PRA). Prior subtotal adrenalectomy in such a patient allowed examination of the role of hyperreninemia in the absence of excessive aldosterone secretion. Hypertension was severe, but serum potassium and aldosterone production were normal. PRA was markedly increased under all conditions studied, with a persistent increase noted with changes in posture. Renal vein renin studies were critical in localizing the renal tumor, and unilateral nephrectomy resulted in a prompt fall in PRA to subnormal levels and dramatic cure of the hypertension. Renin content of the renal tumor was more than 500 times higher than in the adjacent kidney, and histologic studies confirmed that the tumor was of juxtaglomerular cell origin. This case demonstrates that severe hypertension can occur in this syndrome despite subtotal adrenalectomy and further illustrates an association of renin overproduction with a specific type of hypertension.

Recurrent Cushing's Disease Following "Total" Adrenalectomy

Although Cushing's disease is thought to be primarily a hypothalamic or pituitary disorder or both, therapy has traditionally been directed at the adrenal glands. Treatment by subtotal adrenalectomy has been largely abandoned because of the high rate of recurrence, approximately 30%.¹In view of the currently accepted mechanism of this disorder, any remaining adrenal tissue either left purposely or inadvertently would be exposed to continued stimulation by adrenocorticotropic hormone (ACTH). As a result, total bilateral adrenalectomy is the usual therapy at the present time, but because of technical surgical problems, or the presence of ectopic adrenal tissue, this procedure does not guarantee total remission. As far as we have been able to ascertain, there have been only four cases reported of recurrence after a presumed total adrenalectomy.^2,3 We have recently observed four patients with recurrence or persistence of hypercortisolism following seemingly total adrenalectomy. <h3>Methods</h3> Following recognition of the

Remission or recurrent hyperaldosteronism resulting from subtotal adrenalectomy of adenomatous hyperplastic adrenal glands.

This case report concerns a woman who developed the syndrome of primary aldosteronism and underwent unilateral adrenalectomy with a return of blood pressure, serum potassium and aldosterone secretion rate to normal. Several months later there was a recurrence of symptoms and signs, and plasma renin activity was suppressed. Subtotal removal of the remaining adrenal gland cured the hypertension and the laboratory tests became normal. Histologic examination of both adrenal glands showed multiple micronodular hyperplasia and two tumorous lesions (adenomatous hyperplasia) in the right adrenal cortex and one in the left.