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PubMed2000

Influence of morphine treatment in pregnant rats on the mineralocorticoid activity of the adrenals in their neonates.

Exposure of pregnant rats to morphine, from day 11 to day 18 of gestation, was previously reported to induce both an adrenal atrophy and hypoactivity of the glucocorticoid function in newborns at term, but did not affect, in vitro, the responsiveness of those glands to adrenocorticotrophin hormone (ACTH) concerning corticosterone release. Moreover, these effects were mediated by maternal hormones from the adrenal glands. In the present work, we investigated the effects of a prenatal morphine exposure on the mineralocorticoid activity of the adrenals in neonates. The first aim of the present study was to determine in these newborns 1) the adrenal and plasma aldosterone concentrations at birth time and during the early postnatal period 2) the plasma levels of Na+ and K+ at birth time, 3) the in vitro responsiveness of the newborn adrenals to angiotensin II (A(II)) and ACTH. The second aim of our study was to investigate the mineralocorticoid activity of the adrenals in newborns from adrenalectomized mothers treated with morphine during gestation. According to present data morphine given to intact mothers induced in newborns a severe adrenal atrophy but increased adrenal aldosterone content and plasma aldosterone level. However, prenatal morphine was unable to affect significantly Na+/K+ ratio in both mothers and newborns. In vitro, the adrenals of neonates from morphine-treated mothers were unresponsive to An and ACTH for promoting aldosterone release; in contrast, aldosterone secretion was significantly stimulated by high potassium levels (55 mEq). Maternal adrenalectomy performed one day before the beginning of morphine treatment prevented morphine-induced adrenal atrophy but was unable to affect significantly the adrenal mineralocorticoid function of the offspring. Such data suggest that a prenatal morphine exposure stimulated both aldosterone synthesis and release in neonates. However, this basal hyperfunction did not appear to be coupled with an enhanced adrenal responsivity to AII or ACTH. Prenatal morphine-induced hyperactivity of the mineralocorticoid function of the newborn adrenals, which drastically contrast with hypoactivity of the glucocorticoid one, was independent of adrenal factors from maternal origin.

Adrenal Glands↗
PubMed1995

The mechanism of lactitol (NS-4) in inducing adrenomedullary proliferative lesion in rats.

We used 13-week repeated oral administration of lactitol as part of a study to clarify the mechanism by which lactitol induces the proliferation of adrenomedullary chromaffin cells. There was a marked increase in urinary calcium (Ca) excretion even though the lactitol administration had no effect on the blood Ca level. A tendency for an increase in adrenal venous blood epinephrine (EPI) and norepinephrine (NE) concentrations was seen. Organ weight measurement of adrenal glands revealed a tendency for an increase in absolute weight and a significant increase in relative weight. Morphometric analysis of adrenomedullary chromaffin cells showed a tendency for an increased total cell volume and a decreased numerical density; but, there was no conspicuous change in the total cell number. Determinations of the anti-bromodeoxyuridine (BrdU) and antiproliferative cell nuclear antigen (PCNA) antibody-positive cell counts showed a tendency for an increased proliferation rate for adrenomedullary chromaffin cells. Electron microscopy showed a slight increase in the number of Golgi apparatuses in these cells. Because the marked increase in urinary Ca excretion was concomitant with morphological changes that suggested the hyperfunction of chromaffin cells in the adrenal medulla and a tendency for an increased cell proliferation rate, we assume that persistent hyperfunction of the adrenomedullary chromaffin cells, which was mediated by enhanced Ca absorption from the intestinal tract, may have induced proliferative lesion.

Adrenal Medulla↗
PubMed1997

Residual pituitary function after transsphenoidal hypophysectomy in dogs with pituitary-dependent hyperadrenocorticism.

Pituitary function was assessed before and after transsphenoidal hypophysectomy in 39 dogs with pituitary-dependent hyperadrenocorticism (PDH). Anterior pituitary function was investigated using combined administration of four hypophysiotropic releasing hormones (corticotropin-releasing hormone (CRH), GHRH, GnRH, and TRH) with measurements of ACTH, cortisol, GH, LH, prolactin (PRL), and TSH Pars intermedia function was assessed by measurements of basal plasma alpha-MSH concentrations and adrenocortical function by baseline urinary corticoid/creatinine ratios. At eight weeks after hypophysectomy basal plasma ACTH, cortisol, GH, LH, PRL, and TSH concentrations were significantly lower than before surgery. In seven dogs with elevated alpha-MSH concentrations, the values returned to the normal level after surgery. In the combined anterior pituitary function test there were no plasma GH, LH, PRL, and TSH responses to stimulation, whereas plasma ACTH and cortisol responses were small but significant. Remission of hyperadrenocorticism was obtained in 35 dogs and recurrences occurred in 3 of these within 16 months postoperatively. At 8 weeks after hypophysectomy, these 3 dogs were not discernible, with respect to residual pituitary and adrenocortical function, from the 32 dogs with persisting remission. Urinary corticoid/creatinine ratios in the latter group of dogs did not increase during 22 months after hypophysectomy. In contrast to the presurgical findings, at 8 weeks after hypophysectomy there were significant positive correlations between baseline urinary corticoid/creatinine ratios and basal levels and responses for ACTH, indicating return to normal function of the pituitary-adrenocortical axis. It is concluded that among the adenohypophyseal cells present in the sella turcica after hypophysectomy, the corticotropes have a distinct behavior. Much more so than the other cell types, the unaffected corticotropes tend to remain functional, or a repressed reserve fraction of corticotropes may become functional. This may be due to the removal of the hypothalamic influence of a postulated corticotropin-release inhibiting factor or a diminished inhibitory influence of a postulated paracrine factor. The corticotropes may maintain normocorticism, but may also lead to mild recurrence after relatively long periods of remission.

Adrenocortical Hyperfunction↗
PubMedDate not supplied

Adrenal incidentalomas: indications for surgery.

Adrenalectomy represents the gold standard treatment for hyperfunctioning adrenal incidentaloma. In cases of silent adrenal masses, on the other hand, the surgical removal of an adrenocortical cancer entails the sacrifice of a large number of safe benign masses, and in most cases surgery is therefore unjustified. The aim of this paper was to clarify the surgical indications for adrenal incidentaloma by reviewing our experience in comparison with the main reference literature. Over the period from 1995 to 2001 we managed 40 cases of incidentaloma. US and CT abdominal scans, adrenal scintigraphy and biochemical tests were performed on an outpatient basis. Seven pre-Cushing syndromes were removed. Ten incidentalomas measured 4 cm or more in diameter: 5 of these were operated on and in 5 cases surgery was not feasible or was refused. Only one malignant mass was detected (an angiosarcoma). Four postoperative minimal complications (18.7%) were observed. The follow-up (median: 48 months) was uneventful. The surgical approach was traditional in 11 cases and laparoscopic in 1 case. Surgery should be considered mandatory in cases of hyperfunctioning adrenal masses in the presence of suspect radiological evidence, in cases of discordant CT and scintigraphy findings and when the maximum diameter is 4 cm or more.

Adrenal Gland Neoplasms↗
PubMed2000

Discriminatory power of MRI for differentiation of adrenal non-adenomas vs adenomas evaluated by means of ROC analysis: can biopsy be obviated?

The purpose of our study was to evaluate the discriminatory power of MRI in high-field magnet (1.5 T) for differentiation of adrenal non-adenomas vs adenomas assessing the following parameters separately and in combination: mean diameter of adrenal mass; previously described and new ratios as well as index calculated from signal intensity (SI) on SE T2-weighted images, chemical shift imaging (CSI), and Gd-DTPA-enhanced dynamic studies. One hundred eight adrenal masses (36 non-hyperfunctioning adenomas, 27 pheochromocytomas, 23 aldosterone-secreting adenomas, 20 malignant masses and 2 cortisol-secreting adenomas) in 95 patients were evaluated with SE sequences, CSI and Gd-DTPA dynamic studies. Indices and ratios of SI for all examined MRI methods were calculated and examined retrospectively for significance of differences between the groups with calculation of sensitivity and specificity. Receiver operating characteristics (ROC) analysis of calculated parameters in combination was performed. The multifactorial analysis of all four parameters, including size of the tumor, T2(liver) index, CSI ratio reflecting lipid content in the tumor and Wo(max/last) ratio reflecting maximal washout of contrast agent from the tumor had 100 % sensitivity and 100 % specificity in characterization of adrenal non-adenoma. The best performance of combination of mean tumor diameter with single MRI SI parameter was achieved in combination with T2(liver) index for all adrenal masses (area under ROC 0.987) and CSI ratio for non-hyperfunctioning adrenal masses (area under ROC 0.991). Magnetic resonance imaging enables sensitive and specific diagnosis of adrenal non-adenoma.

Adenoma↗
PubMed1976

Gallbladder visualization in adrenal scanning: case report.

A patient suspected of having adrenal hyperfunction was scanned with 131I-iodocholesterol. Intense gallbladder activity persisted for several days and was eliminated by a fatty meal. The possibility of confusion with unilateral adrenal localization is discussed.

Adrenal Gland Diseases↗
PubMed1992

Endocrinopathies of hyperfunction: Cushing's syndrome and aldosteronism.

Increased function of the adrenal cortex is a normal response in times of physiologic and psychologic stress. Adrenal cortical secretions (e.g., glucocorticoids, aldosterone) orchestrate a multitude of internal processes aimed at maintaining homeostasis and psychologic integrity. Many patients admitted to a critical care unit will manifest some increase, even minor, in adrenal function. However, excessive secretions of these hormones can have a lethal effect of fluid and electrolyte balance, energy metabolism, and immune function. Cushing's syndrome denotes a disorder characterized by increased circulating levels of glucocorticoids (primarily cortisol). An easily recognizable disorder, it may arise from pathology of the adrenal cortex or the anterior pituitary glands, ectopic secretions from a nonendocrine tumor, or from excessive doses of exogenously administered glucocorticoids. Cushing's syndrome is rarely an admitting diagnosis to critical care but is a disorder that can seriously affect recovery from coexisting illnesses if not treated. Aldosteronism, although rare, will often be diagnosed after admission to a critical care unit for management of troublesome hypertension, hypokalemia, congestive heart failure, and various dysrhythmias. Suspicion of the diagnosis should always arise when these manifestations occur, particularly when hypokalemia is refractory to potassium supplementation. Without timely diagnosis and treatment, these patients will succumb to lethal dysrhythmias.

Adrenocortical Hyperfunction↗
PubMed1997

[The necessity for perioperative cortisol substitution. Spontaneous and stimulated ACTH and cortisol secretion during unilateral adrenalectomy for renal cell carcinoma].

UNLABELLED: Impaired adrenal function during perioperative stress carries the risk of acute cortisol (Cs) deficiency (Addisonian crisis), which may be critical without Cs supplementation. Thus, with evidence of dysfunction of the adrenal glands perioperative substitution is indicated. However, it is unclear whether unilateral adrenalectomy may attenuate the adrenocorticoid response. Glucocorticosteroids are potent agents with several well-known side effects. The purpose of the present study was to evaluate if routine Cs supplementation is justified and necessary in patients undergoing adrenalectomy during nephrectomy for renal-cell cancer. METHODS: Ten consecutive patients with renal-cell cancer (5 male, 5 female; age 58 +/- 10 years; ASA class I-II) who underwent adrenalectomy with radical nephrectomy were included in this study. None of them had received steroids for at least 5 years prior to the current surgery. Anaesthesia was induced with propofol, fentanyl, and vecuronium and maintained with isoflurane (PetIso: 0.8 +/- 0.3 vol.%) in nitrous oxide (66%) and oxygen. The patients did not receive any Cs treatment perioperatively. Monitoring included heart rate (beats/min), mean arterial pressure (mm Hg), central venous pressure (mm Hg), O2 saturation (%), and body temperature (degrees C, rectal). Plasma analyses included Cs (Cs radioimmunoassay IBL; normal 120-250 ng/ml), adrenocorticotropic hormone (ACTH) (ACTH-II IRMA; normal (10-50 pg/ml), glucose, and electrolytes determined as follows: preoperatively (8 a.m.); 1-6 h (60-min intervals) after surgery; pre-corticotropin-releasing hormone (CRH) (Corticobiss: 2 micrograms/kg i.v.) administration (1st postop. day at 8 a.m. and after 30, 60, 90, and 120 min. The study was completed with plasma analyses on postoperative days 2 and 3 (8 a.m.). RESULTS: None of the patients showed any clinical signs of plasma parameter of adrenal insufficiency due to the unilateral adrenalectomy. Serum levels (median: 25%/75% percentiles) of Cs (maximum [max.]:253 [217/288] ng/ml) and ACTH (max.:347 ([68/405] pg/ml) were elevated above the normal range postoperatively). After intravenous stimulation with CRH (1st postoperative day), Cs (max.:273 [248/310] ng/ml) and ACTH (max.: 107 ([75/275] pg/ml) were also increased above normal. During postoperative days 2 and 3 (8 a.m.) Cs and ACTH remained in the high-normal range. CONCLUSIONS: Data from this study indicate that unilateral adrenalectomy was associated with adequate spontaneous Cs secretion by the remaining adrenal gland. Moreover, stimulation with CRH demonstrated adequate reactivity of the pituitary-adrenal axis. None of the patients showed any signs of Cs deficiency by clinical or serum parameters. Therefore, we do not recommend routine Cs supplementation in patients undergoing adrenalectomy during tumor nephrectomy, nevertheless, Cs supplementation remains necessary for patients with primary hypothalamic-pituitary-adrenal dysfunction (Addison's disease) or hyperfunction (Cushing's disease).

Adrenalectomy↗
PubMed1990

Long-term results of adrenal autotransplantation in Cushing's disease.

Many advances have been made in the diagnosis and treatment of patients with Cushing's disease. Currently, partial or total transsphenoidal resection of the pituitary gland is the surgical approach favored by many authorities, with cure rates of 80% to 90% reported for selected patients. However, long-term follow-up is still needed for this treatment modality, and recurrence rates of up to 50% have been reported. In addition, less favorable results are seen with this approach when there is recurrence after initial transsphenoidal surgery or when there is not a single microadenoma responsible for the disease of the pituitary gland. We report a series of 14 patients who were treated with bilateral adrenalectomy and autotransplantation. Follow-up is 4 to 14 years. Four patients show iodocholesterol uptake at the site of autotransplantation. Of these, three patients have been weaned from cortisone for 12, 10, and 2 years. The fourth patient, after being weaned from all medications, underwent partial resection of a hyperfunctioning autotransplant and currently, 7 years after the original operation, is being weaned from cortisone. A fifth patient was weaned to a reduced dose of cortisone for 8.5 years, but the dose was recently increased. There is no evidence of Nelson's syndrome in any of these patients with clinical evidence of functioning autotransplants. We believe that bilateral adrenalectomy with autotransplantation still plays an important role in the long-term management of selected cases of Cushing's disease and that autotransplantation, when successful, results in few complications and allows long-term freedom from exogenous steroid medication.

Adolescent↗
PubMed1979

Adrenal scintigraphy with 131I-19-iodochlesterol in the diagnosis of Cushing's syndrome associated with adrenal tumor.

Seven patients with Cushing's syndrome secondary to adrenocortical tumors were studied using 131I-19-iodocholesterol. The diagnosis of all cases were verified histologically. In three cases with adenoma the uptake of the tracer was in the tumor only, while the two patients with adrenocortical carcinoma failed to show adrenal accumulation of the labelled compound. In two patients there was a hyperplasia-like scintigraphic pattern, while the stimulation and suppression biochemical tests suggested adrenal tumor. One of these cases was verified as a mixed form (adenoma plus hyperplasia), and the tumor bearing gland was significantly larger on the scan which helped the preoperative localization. In the other case, verified as bilateral multiple adrenocortical adenomas, the autonomus function of both adrenals was proved by dexamethasone suppression scanning. It seens reasonable to use the latter as an adunctive diagnostic procedure in patients where there is a discrepancy between the standart scintiscan and the biochemical indexes of adrenal hyperfunction.

19-Iodocholesterol↗
PubMed1992

Structure and dynamics of adrenal mitochondria following stimulation with corticotropin releasing hormone.

The mitochondria of rat adrenals were investigated qualitatively and quantitatively in different functional states of the adrenal cortex. Following stimulation of the animals with corticotropin releasing hormone (CRH), the corticosterone serum levels reached a maximum 1 hour after stimulation with CRH. The amount of inner mitochondrial membrane within the zona fasciculata increased showing a biphasic time course, with a first maximum 2 hours and a second maximum 8 hours after stimulation. In contrast, a significant rise of mitochondrial volume occurred only 24 hours after CRH stimulation. Therefore, the dense vesicularization of mitochondrial cristae may constitute an early process to enhance the steroidogenic capacity of these cells. Within cells of the transition zone between zona glomerulosa and zona fasciculata, we could depict a special type of mitochondria with characteristic crescent-like cristae only seen after stimulation with CRH. This type of mitochondria may represent an intermediate form between mitochondria of zona glomerulosa and zona fasciculata underlining the impressive transformational capacity of adrenocortical mitochondria. After hypophysectomy, zona fasciculata cells contained mitochondria with tubular inner membranes, representing a hypofunctional state. In contrast, the hypofunctional state after hypophysectomy and the hyperfunctional state after stimulation of the adrenal cortex via CRH injection did not appear to correlate with the morphology of mitochondria from the zona reticularis and adrenal medulla.

Adrenal Cortex↗
PubMed1990

Assessment of survey radiography and comparison with x-ray computed tomography for detection of hyperfunctioning adrenocortical tumors in dogs.

Results of abdominal survey radiography and x-ray computed tomography (CT) were compared in 13 dogs with hyperadrenocorticism histologically attributed to adrenocortical tumors. X-ray computed tomography enabled accurate localization of the tumor in all 13 dogs. Apart from 2 poorly demarcated irregular-shaped and mineralized carcinomas, there were no differences between adenoma (n = 3) and carcinoma (n = 10) on CT images. In 1 dog, invasion of the caudal vena cava by the tumor was suggested on CT images and was confirmed during surgery. Suspicion of adhesions between tumors of the right adrenal gland and the caudal vena cava on the basis of CT images was confirmed during surgery in only 2 of 6 dogs. Survey radiography allowed accurate localization of the tumor in 7 dogs (4 on the right side and 3 on the left). In 6 of these dogs, the tumor was visible as a well-demarcated soft tissue mass and, in the other dog, as a poorly demarcated mineralized mass. The smallest tumor visualized on survey radiographs had a diameter of 20 mm on CT images. Six tumors with diameter less than or equal to 20 mm were not visualized on survey radiographs. In 1 of these dogs, a mineralized nodule was found in the left adrenal region, without evidence of a mass. In a considerable number of cases, survey radiography can provide presurgical localization of adrenocortical tumors in dogs with hyperadrenocorticism; CT is redundant in these instances. In the absence of positive radiographic findings, CT is valuable for localization of adrenocortical tumors.

Adenoma↗
PubMed2003

Plasma and salivary 6beta-hydroxycortisol measurements for assessing adrenocortical activity in patients with adrenocortical adenomas.

The aim of this study was to examine and compare the potential usefulness of plasma and salivary 6beta-hydroxycortisol measurements for assessing adrenocortical activity in patients with adrenocortical adenomas. Plasma and salivary cortisol as well as 6beta-hydroxycortisol determinations were performed by radioimmunoassay after extraction with ethyl acetate followed by chromatographic separation using a modified paper chromatographic system. Samples were obtained from 36 control subjects and 37 patients with non-hyperfunctioning adrenocortical adenomas in the morning at 8 a.m. after a low-dose of dexamethasone and after stimulation with synthetic depot ACTH. Basal and post-dexamethasone hormone levels were also measured in plasma and salivary samples of 4 patients with Cushing's syndrome from adrenal adenomas. In the baseline state, patients with non-hyperfunctioning adrenocortical adenomas had significantly higher plasma and salivary 6beta-hydroxycortisol levels (mean+/-SE, 79.0+/-7 and 17.1+/-2.2 ng/dl, respectively) compared to those measured in controls (62.0+/-4 and 7.7+/-0.6 ng/dl, respectively), whereas baseline plasma and salivary cortisol levels (9.6+/-0.5 microg/dl and 342+/-39 ng/dl, respectively) were similar to those measured in the control group (9.9+/-0.4 microg/dl and 366+/-24 ng/dl, respectively). In all groups, the changes in plasma and salivary 6beta-hydroxycortisol concentrations after dexamethasone suppression and ACTH stimulation were similar to the changes in plasma and salivary cortisol levels, although the differing ratios of 6betaOHF to cortisol indicated potentially important variations in the induction of 6beta-hydroxylase activity between the three groups. In patients with Cushing's syndrome, baseline plasma and salivary 6beta-hydroxycortisol concentrations (754+/-444 and 104+/-88 ng/dl, respectively) were more markedly increased than plasma and salivary cortisol levels (24.8+/-6.7 microg/dl and 1100+/-184 ng/dl, respectively), and all remained non-suppressible after dexamethasone administration. These results suggests that plasma and salivary 6beta-hydroxycortisol determinations may precisely detect not only overt increases of cortisol secretion in patients with Cushing's syndrome but also mild glucocorticoid overproduction presumably present in patients with non-hyperfunctioning adrenocortical tumors.

Adrenal Cortex Neoplasms↗
PubMed1981

Spironolactone bodies in aldosteronomas and in the attached adrenals. Enzyme histochemical study of 19 cases of primary aldosteronism and a case of aldosteronism due to bilateral diffuse hyperplasia of the zona glomerulosa.

The formation of spironolactone (S) bodies, eosinophilic laminated cytoplasmic inclusions, is induced in the aldosterone-producing cells of the human adrenal cortex after the administration of spironolactone. The aim of this study was to define the enzyme histochemical characteristics of S bodies, S-body-containing cells, and the apparently hyperplastic zona glomerulosa (zG) of adrenal tissues attached to aldosteronomas. S bodies were found in 14 of 19 aldosteronomas, in 10 of 19 adrenal tissues attached to aldosteronomas, and in the adrenal tissues in a patient with aldosteronism due to bilateral diffuse zG hyperplasia. The S bodies themselves exhibited most intense 3 beta-hydroxysteroid dehydrogenase (3 beta HSD) activity but did not exhibit glucose-6-phosphate dehydrogenase (G6PD), NADP-dependent isocitrate dehydrogenase (NADP-ICDH), or succinate dehydrogenase (SDH) activity, confirming histochemically the origin of S bodies in the smooth endoplasmic reticulum. In two adenomas, S bodies were found to be surrounded by reaction products of acid hydrolase but were not found in the other adenomas and the remaining adrenal tissues. S-body-containing cells, irrespective of being neoplastic or not, showed enhanced 3 beta HSD, G6PD, and NADP-ICDH activity and weak SDH activity (Type I pattern of enzyme activity). Though zG was hyperplastic in most of the adrenal tissues attached to the adenomas, zG cells that did not contain S bodies showed the opposite pattern (Type II pattern) of enzyme activity (ie, weak 3 beta HSD, G6PD, and NADP-ICDH activity and intense SDH activity), in contrast to those in the adrenal tissues in a patient with aldosteronism due to bilateral diffuse zG hyperplasia (which showed the Type I pattern). The results are consistent with the view that hyperplastic zG cells, except S-body-containing cells, in the case of aldosteronoma are not hyperfunctioning. The latter cells may have enhanced but possibly abortive steroidogenic activity.

Adenoma↗
PubMed1988

Hypercortisolism and the resistance to dexamethasone suppression during gestation.

Maternal adrenocortical function was studied by measuring plasma cortisol and urinary free cortisol during gestation. Changes in suppressibility of pituitary-adrenocortical function were determined by dexamethasone administration. Urinary free cortisol as well as plasma cortisol increased during the course of gestation. The suppressibility by dexamethasone became less effective as pregnancy advanced. These results suggest that pregnant women have pituitary-adrenocortical hyperfunction and tissue refractoriness to glucocorticoid which increases during the course of gestation.

Adrenal Cortex↗
PubMed1986

Primary pigmented nodular adrenocortical disease: diagnosis and management.

A rare cause of Cushing's syndrome appearing to originate in the adrenal glands is primary pigmented nodular adrenocortical disease (PPNAD). It may be variably associated with a complex of other pathologic characteristics that include cardiac myxomas, cutaneous myxomas, and lentigines, mammary myxoid lesions, testicular tumors, pituitary adenomas, and neuroectodermal tumors. We have reviewed the clinical, biochemical, radiographic, operative, and pathologic details of seven patients with PPNAD who have been evaluated at Mayo Clinic. Biochemical testing supported autonomous adrenal hyperfunction. This was based on the failure of cortisol suppression by high-dose dexamethasone, failure of pituitary stimulation by metyrapone administration, either normal or suppressed basal adrenocorticotropic hormone (ACTH) levels, normal radiographic studies of the sella turcica, and normal abdominal and thoracic computerized tomography in search of an ectopic ACTH-producing tumor. Six patients underwent bilateral total adrenalectomy without postoperative complications. Nelson's syndrome has not occurred, but acute adrenocortical insufficiency may have contributed to the death of one patient. To recognize the possible diagnosis of PPNAD preoperatively is important to proceed directly with adrenalectomy, avoiding unnecessary pituitary surgery. Moreover, if PPNAD is part of the broader pathologic complex, the possible presence of cardiac myxomas that may be multiple, recurrent, in atypical locations, and familial must be borne in mind.

Adolescent↗