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【Objective】 To explore a new treatment of primary bilateral macronodular adrenal hyperplasia (PBMAH) and its efficacy. 【Methods】 Clinical data of 20 PBMAH patients treated in our hospital during Mar.2010 and Apr.2021 were retrospectively analyzed. All patients underwent laparoscopic subcutaneous displacement of vascularized adrenal. The clinical symptoms, plasma free cortisol, adrenocorticotrophic hormone (ACTH), and 24 h urinary free cortisol were regularly monitored after surgery. 【Results】 Of all 20 patients, 19 were followed up for 18 to 120 months (median 60 months). Three months after surgery, reexamination showed 1 patient had decreased plasma free cortisol and increased ACTH, but had no symptoms of low corticosteroids. After another 3 months, the plasma free cortisol and ACTH returned to normal. After 4 to 48 months, the parameters recovered in all patients and the clinical symptoms disappeared. 【Conclusion】 Laparoscopic vascularized adrenal displacement is a new and effective method for the treatment of PBMAH. It can alleviate the Cushing syndrome with no obvious adverse reactions.
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Objective To investigate the clinical characteristics in patients with bilateral macronodular adrenal hyperplasia ( BMAH). Methods We enrolled 357 patients with adrenal tumors including 42 BMAH, 167 cortisol-producing adrenal adenoma (CPA), and 148 non-functional adenomas (NFA). The clinical manifestations, hormonal explorations and complications were compared among groups. Results (1) Age at diagnosis of BMAH group was significantly higher than that of CPA group [(53.7±8.3 vs 41.1±13.1)years], female proportion of BMAH group was lower that that of CPA group(54.8%vs 88.0%, P＜0.01). Both were similar to NFA group. (2) Compared to NFA group, metabolic abnormalities were severer in BMAH and CPA groups. Prevalence of hypertension, diabetes mellitus, and hypokalemia were the highest in BMAH group. ( 3) BMAH patients had lower midnight serum cortisol, 24 hour urine free cortisol, and adrenocorticotrophic hormone levels than CPA patients (P＝0.008, 0.010, and P＝0.001, respectively). The suppression rate of serum cortisol after low-dose dexamethasone suppression test was significantly higher in BMAH group ( P＝ 0. 005). Conclusion BMAH patients were at higher age at diagnosis. Although hypercortisolism in BMAH patients is relatively slight, metabolic abnormalities are more severe. Besides functional diagnosis, physicians should pay more attention to the assessment of complications related to hypercortisolism.
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Objective To assess the long-term outcome of unilateral adrenaletomy in patients with adrenocorticotropic hormone independent macronodular adrenal hyperplasia (AIMAH).Methods The data from 82 cases of AIMAH were reviewed and summarized including clinical manifestations, endocrinological data, imaging findings and postoperative follow-up.Fouty-nine males and thirty-three females with a mean age of fifty years composed our series.Among the 82 cases, 41 demonstrated Cushing syndrome (CS), 74 presented with hypertension, 38 manifested diabetes mellitus, 35 complicated of osteoporosis and 11 of them with bone fracture, 39 complained of edema.Laboratory tests showed low ACTH plasma levels (＜ 2.2 pmol/L) in 62 of 79 cases.High level of 24-hour urinay free cortisol excretion(＞ 284.2nmol/L) was found in 67 of 79 cases.Elevated serum cortisol with loss of the circadian rhythm was presented in 55 of 60 cases.Failed to suppress cortisol secretion was observed in 61 of 63 conducted with low-dose dexamethasone suppression tests and in 47 of 53 implemented with high-dose dexamethasone suppression tests.Bilateral massively enlarged multinodular adrenal glands were found in all cases with CT or MR imaging.Unilateral adrenalectomy was performed in the larger side of adrenal gland in all 82 cases.All adrenalectomies were carried out including 47 in right sides and 35 in left sides, with 77 by retroperitoneoscopic approach and 5 by open retroperitoneal approach.Results Histopathological examination confirmed nodular hyperplasia of the adrenal cortex for all specimens.After a mean duration of 48.5 months, 80 of 82 patients were available for follow-up.Most clinical symptoms resolved within 6 months after operation.Cushingoid features disappeared in 58.5％ (24/41)of patients who initially presented with typical signs of Cushing Syndrome.Weight loss was seen in 56.3％ (45/80).Improvement of hypertension and diabetes was observed in 63.5％ (47/74) and 76.3％ (29/38) respectively.All the eyelids swelling and edema of the limbs subsided within 3 months.In long-term follow-up (more than 6 months), 82.9％ (63/76) had clinical and biochemical recurrence within a mean time of 14.6 months and 46.0％ (29/63) received contralateral adrenal surgery.Two patients died, one died from heart attack and another one died from pulmonary infection, 2 years and 7 years after unilateral adrenalectomy, respectively.Conclusions Unilateral adrenalectomy just has transient efficiency for AIMAH.Most patients will experience clinical and biochemical recurrence for a long period,and have to receive another operation for the contralateral hyperplastic adrenal gland.
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Objective To discuss the treatment experience of adrenocorticotropic hormone independent macronodular adrenal hyperplasia (AIMAH).Methods The clinical data of 24 cases of AIMAH from August 1972 to December 2016 were retrospectively analyzed.The cases included 10 males and 14 females with a mean age of 43 (range 26 to 59) years.16 patients presented with typical Cushing syndrome (CS) and 8 patients had weight gain, hypertension or diabetes mellitus without any sign of CS.The circadian rhythm of serum cortisol was abnormal.Low and high dose dexamethasone suppression tests failed to suppress cortisol secretion.The preoperative CT scan showed bilateral enlargement of the adrenal glands with multiple macmnodules.14 patients underwent open surgery, including 5 cases of unilateral adrenalectomy, 6 cases of adrenalectomy combined with contralateral subtotal adrenalectomy and 3 cases of bilateral adrenalectomy simuhaneously.6 patients underwent conventional laparoscopic adrenalectomy,including 2 cases of unilateral adrenalectomy, 2 cases of adrenalectomy combined with contralateral subtotal adrenalectomy and 2 cases of bilateral adrenalectomy.4 patients underwent robot-assisted laparoscopic bilateral adrenalectomy.Results The postoperative pathological examination showed that all patients had bilateral adrenal macronodular or adenomatoid hyperplasia.During the mean follow-up of 68 months (range 3 to 192 months), the clinical symptoms of CS disappeared after surgery in all cases.7 patients who received unilateral adrenalectomy had urinary free cortisol and serum cortisol within normal ranges and no further enlargement of the contralateral gland was noticed.Among the 3 patients who received bilateral adrenalectomy simultaneously, 1 case died of adrenal crisis on the seventh day post-operation.The remaining 2 cases presented with adrenal insufficiency but returned to normal after glucocorticoid replacement therapy.Nelson's syndrome was not observed in other patients.Conclusions The use of bilateral adrenalectomy to treat AIMAH may involve risk.Unilateral adrenalectomy is recommended for an alternative treatment for AIMAH.Controlateral adrenalectomy with lifelong corticosteroid replacement or contralateral subtotal adrenalectomy may be performed if the symptoms have not improved or recurred after unilateral adrenalectomy.
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Objective To study the surgical treatment of adrenocorticotropic hormone independent macronodular adrenal hyperplasia (AIMAH).Methods The clinical data of 12 AIMAH patients were analyzed retrospectively.There were 3 males and 9 females, with an average age of 55 years (range, 39-67 years).10 cases had typical clinical features of Cushing syndrome.Endogenous hypercortisolism was confirmed on the basis of loss of circadian rhythm of serum cortisol, high late-night serum cortisol level and inadequate cortisol suppression after overnight low-dose dexamethasone suppression test.ACTH independence was established on the basis of suppressed serum ACTH levels and inadequate cortisol suppression after overnight high-dose dexamethasone suppression test.CT scan showed bilateral enlargement of the adrenal glands with multiple macronodules.Steroid supplement was given after operation.Results Surgical intervention was performed in all the patients.Seven patients underwent bilateral adrenalectomy,and unilateral adrenalectomy was performed in 5 patients, one of whom had the history of contralateral adrenalectomy.Pathological examination confirmed multinodular hyperplastic adrenal enlargement.The average duration of postoperative follow-up was 50 months (range, 1-105 months).One patient undergoing bilateral adrenalectomy died from respiratory failure and pulmonary infection one month after operation.Remission of Cushing syndrome symptoms was obtained after surgery in other 11 patients.For these 11 cases,the serum cortisol declined into normal ranges, but suppressed serum ACTH levels and inadequate cortisol suppression after overnight low-dose dexamethasone suppression test were still present.Two patients received contralateral adrenalectomy due to recurrent overt Cushing syndrome after one year and 5 years, respectively.Conclusions Unilateral adrenalectomy might be an effective and safe treatment modality for AIMAH, but subclinical hypercortisolism would be present postoperatively.Contralateral adrenalectomy may be performed in case of the recurrence of overt Cushing syndrome.
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Objective To investigate the therapeutic outcomes of unilateral adrenalectomy for Cushing syndrome in patients with adrenocorticotropic hormone (ACTH) independent bilateral macronodular adrenal hyperplasia.Methods 22 patients diagnosed with Cushing syndrome caused by ACTH independent bilateral macronodular adrenal hyperplasia from January 2005 to December 2015 were retrospectively concluded.There are 17 male patients and 5 female patients with the median age of 46.5 years.All patients were presented with Cushing syndrome such as moon face, buffalo hump and other typical symptoms of Cushing syndrome.The laboratory tests showed disappearance of cortisol rhythm, elevated 24-hour urinary free cortisol, suppressed or normal ACTH and unsuppressed low dose dexamethasone suppressing test.CT scan showed bilateral macronodular adrenal hyperplasia in all patients.Results All the patients received unilateral adrenalectomy.Pathological results showed adrenal cortex nodular hyperplasia.16 patients had reexamination in the endocrine department.The median follow-up time was 26 (17-118)months for these 16 patients.In the follow-up between 3 to 9 months after surgery, laboratory test showed serum and urinary cortisol level returned to normal range and Cushing syndrome also disappeared in these 16 patients.CT scan showed no recurrence.And no adrenal insufficiency occurred.One patient had recurrence one year after surgery and two patients turned better after surgery but had recurrence at 3 years and 10 years after surgery.Two patients received contralateral adrenalectomy and cortisol hormone replacement therapy after surgery.Another patient with recurrence refused second surgery.No recurrence was observed in other patients.Four patients were followed up by telephone, and all recovered well after surgery.18 patients had hypertension before surgery and their blood pressure significantly decreased after surgery.Among them 13 cases blood pressure returned to normal range.5 patients had elevated blood glucose before surgery, and 3 patients' blood glucose returned to normal range.Conclusions Unilateral adrenalectomy for Cushing syndrome in patients with ACTH independent bilateral macronodular adrenal hyperplasia is safe and effective.
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Adenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. It is characterized by multinodular enlargement of the adrenal glands and hypercortisolism. Although bilateral adrenalectomy is the standard therapy, unilateral adrenalectomy is an effective alternative. Here we present a case of a 71-year-old female referred to the Endocrinology Department for further evaluation of bilateral adrenal macronodular hyperplasia. Based on dynamic hormone tests and imaging studies, she was diagnosed with AIMAH. Due to persistent hypercortisolism, she underwent completion contralateral surgery after unilateral adrenalectomy. This case demonstrates that unilateral adrenalectomy should be considered in a patient presenting with obvious symptoms of hypercotisolism and relatively asymmetric adrenal enlargement.
Subject(s)
Aged , Female , Humans , Adrenal Glands , Adrenalectomy , Cushing Syndrome , Endocrinology , HyperplasiaABSTRACT
OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Ablation Techniques/methods , Adrenocortical Hyperfunction/surgery , Ethanol/therapeutic use , Tomography, X-Ray Computed/methods , Adrenal Cortex Neoplasms/surgery , Adrenal Gland Neoplasms/surgery , Adrenalectomy/methods , Adrenocortical Adenoma/surgery , Aldosterone/biosynthesis , Cushing Syndrome/surgery , Hyperplasia/surgery , Pheochromocytoma/surgery , Reproducibility of Results , Retrospective Studies , Treatment OutcomeABSTRACT
La hormona luteinizante (LH) y la gonadotropina coriónica humana (hCG) puede inducir esteroidogénesis, hiperplasia y tumorigénesis adrenal a través del estímulo sobre el receptor constitutivo de la LH (R-LHCG) en la corteza adrenal. Los mecanismos fisiopatológicos del síndrome de Cushing adrenal dependiente de LH (SCa-LH) no se han establecido plenamente, pero es reconocida la relación ontogénica adrenal-gonadal con mutua participación de diversos genes, factores de transcripción y enzimas esteroidogénicas como posible causa. El SCa-LH fue descrito en mujeres durante la gestación por el estímulo de hCG y en la posmenopausia ante el aumento de LH, así como en hurones luego de la gonadectomía quirúrgica.
Luteinising hormone (LH) and human chorionic gonadotropin (hCG) can induce steroidogenesis, hyperplasia, and adrenal tumorigenesis through the stimulus of the constitutive LH receptor (R-LHCG) within the adrenal cortex. The pathophysiological mechanisms of luteinising hormone-dependent Cushing's syndrome are not completely understood, but the ontogenic relationship between the adrenal cortex and the gonads, with mutual participation of different genes, transcription factors and steroidogenic enzymes cited as a possible cause, is well-recognised. SCa-LH has been described in pregnant women, as a result of hCG stimulus, and in post-menopausal women, due to increased levels of LH, as well as in ferrets after gonadectomy.
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Objective To investigate the effect of has-miR-663 on proliferation and apoptosis of human adrenal cortical cell line SW-13.Methods Based on the first stage study,in adrenocorticotropinindependent macronodular adrenal hyperplasia (AIMAH),hsa-miR-663 was up-regulated.The synthetic hsa-miR-663 mimics and inhibitors were transfected the SW-13 cell line.Real-time quantitative PCR (RT-PCR) tested the effect of the transfection.According to the results of RT-PCR,cell line was divided into five groups,including blank group,mimics NC group,inhibitor NC group,mimics group,inhibitor group.Then proliferating of the cell was tested by MTT and the apoptosis by Annexin V-FITC/PI.Result By RT-PCR,mimics group showed that has-miR-663 was over-expressed,and inhibitor group were knocked down.MTTshowed that,compared with NC group and Blank group,SW-13 cell line proliferated faster in mimics group and slower in inhibitor group.Annexin V-FITC/PI showed that apoptosis of SW-13 cell line was not different in all the five groups.Conclusion Up-regulating has-miR-663 could accelerate proliferation of adrenal cortical cells.The microRNA might play some role in the pathogenesis of AIMAH.
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Objective To investigate the different expressed microRNA in adrenocorticotropinindependent macronodular adrenal hyperplasia (AIMAH).Methods For the screening different expressed microRNA in AIMAH,5 AIMAH fresh tissues and 5 normal adrenal fresh tissues were collected.Total RNA was extracted by using Trizol reagents,then was purified and tested by denaturing agarose gel electrophoresis and NanoDrop 2000.Then the purified RNA was labeled by using Hy3TM fluorescent label and hybridized with miRCURY Array.MicroRNA array was scanned and analyzed by GenePix 4000B microarray scanner and GenePix proV6.0.The ratio acquired by divided AIMAH fluorescent correction value by normal adrenal fluorescent correction value.If the ratio was greater than 3.0,the microRNA was defined as up-regulating,or smaller than 0.3,as down-regulating.For the validation 40 AIMAH samples and 10 normal adrenal samples were used.The miRCURY array result was validated by real-time quantitative PCR.All the data was managed by SPSS 17.0.When P ＜ 0.05,the difference was significant in Student's t test.Results There were 12 different expressed microRNA between normal adrenal gland and AIMAH,including 7 up-regulating and 5 down-regulating microRNAs.Up-regulating microRNAs included hsa-miR-663,hsa-miR-498,hsa-miR-638,hsa-miR-501-5p,hsa-miR-585,hsa-miR-557 and hsa-miR-144.Down-regulating microRNA included hsa-miR-744,hsa-miR-143,hsa-miR-26a,hsa-miR-22,hsa-miR-29a.Four different expressed microRNAs were identified in validation test,including 3 up-regulating and 1 down-regulating microRNA.Upregulating microRNA included has-miR-663,has-miR-498,and has-miR-557.Down-regulating microRNA was has-miR-744.Conclusion Compared with normal adrenal gland,there might be some different expressed microRNAs in AIMAH.
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No abstract available.
Subject(s)
Humans , Male , Middle Aged , Adrenalectomy , Arginine Vasopressin , Catecholamines/physiology , Cushing Syndrome/diagnosis , Hydrocortisone/metabolism , Vasopressins/physiologyABSTRACT
Objective To evaluate the clinical characteristics of patients with adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH).Methods A total of 10 AIMAH cases were enrolled in this retrospective study.The clinical and laboratory findings of all patients were collected and analyzed.Results All patients manifested some clinical features and biochemical evidence of Cushing's syndrome.The plasma adrenocorticotropic hormone (ACTH) level was undetectable in all the patients and their serum cortisol secretion rhythm was abnormal.Low and high-dose dexamethasone suppression tests failed to suppress the cortisol secretion.The bilateral macronodular adrenal enlargement was shown by CT/magnetic resonance imaging.The supine-upright posture test was positive in four patients.Three patients were performed bilateral adrenalectomy,five were unilateral adrenalectomy and the remaining two patients were taken propranolol.All the patients had followed up for 10 to 89 months.Contralateral adrenalectomy was performed in two patients with recurrent symptoms after unilateral adrenalectomy and two patients given propranolol were underwent bilateral adrenalectomy when their symptoms had not been improved or recurred.Conclusion AIMAH is a relatively rare subtype of Cushing's syndrome with unique clinical and laboratory findings.Propranolol is a good choice if the supine-upright posture test is positive.Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH.Bilateral adrenalectomy could be performed if the symptoms have not been improved or recurred after unilateral adrenalectomy.
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Objective To discuss the clinical feasibility and significance of retroperitoneal laparoscopic adrenalectomy for bilateral macronodular adrenal hyperplasia.Methods 9 patients with bilateral macronodular adrenal hyperplasia received retroperitoneal laparoscopic operations.5 cases had the typical clinical manif(e)stetions of Cushing's syndrome and 4 patients presented hypertension or diabetes mellitus without any sign of Cushing's syndrome.Body mass index was 18.8-31.2,with 25.0 as the average.Serum cortisol was 544-1746 nmol/L,with 984 nmol/L as the average.Urinary free cortisol (UFC) was 347-1989 nmol/24h with 1129 nmol/24h as the average.CT scan showed bilateral enlargement of the adrenal glands with multiple macronodules.The size of the adrenal gland was 6 cm × 5 cm × 4 cm to 10 cm × 6 cm× 3 cm,with 8 cm× 5 cm × 4 cm as the average.Results One patient underwent retroperitoneal laparoscopic bilateral adrenalectomy with replantation of adrenal gland tissue in scrotum,1patient underwent right lateral adrenalectomy combined with left subtotal adrenalectomy,4 patients underwent right lateral adrenalectomy ( 1case was converted to open surgery due to hemorrhage),and 3 patients underwent left lateral adrenalectomy.The average operation duration was 116 min,ranging from 60 to 165 min.The estimated average intraoperative blood loss was 110 ml,ranging from 20 to 600 ml.No severe complications like injury in great vessels or organs happened during the operation.Pathological diagnosis showed nodular or adenomatoid hyperplasia of adrenal cortex.The average postoperative follow-up was 58 months,ranging from 12 to 84 months.Clinical symptoms of Cushing's syndrome disappeared after surgery in the 5 cases with typical clinical features of Cushing's syndrome.Body weight and blood glucose recovered to normal level for those 4 cases presenting body weight increasement,hypertension or diabetes mellitus.2 cases with right lateral adrenalectomy received left adrenalectomy 33 and 41months after the first operation due to recurrence of Cushing's syndrome.Conclusions Retroperitoneal laparoscopic adrenalectomy for bilateral macronodular adrenal hyperplasia is technically feasible in experienced hands. Unilateral adrenalectomy can relieve most of the Cushing's symptoms.Bilateral adrenalectomy or unilateral adrenalectomy combined with contralateral subtotal adrenalectomy can be performed if the symptoms are not improved or recurred after unilateral adrenalectomy.
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Objective To improve the diagnosis and treatment ot adrenocorticotropin-independent macornodular adrenal hyperplasia (AIMAH).Methods The clinical data of 17 cases with AIMAH from 2000 to 2011 were analyzed retrospectively,including 3 subclinical AIMAH,10 clinical AIMAH and 4 highrisk AIMAH patient,with common radiological characteristic of bilaterally enlarged adrenal glands with multiple nodules like ginger.The 3 cases of subclinical AIMAH patients presented with decreased serum ACTH,normal or slightly elevated plasma cortisol and urinary free cortisol level,no suppression following 1 mg overnight dexamethasone suppression test and absence of clinical signs of Cushing syndrome (CS).While clinical AIMAH and high-risk AIMAH presented with clinical signs of CS,elevated plasma cortisol and urinary free cortisol level,suppressed serum ACTH,loss of normal circadian rhythm in cortisol secretion and no suppression following the low-dose and high-dose overnight dexamethasone suppression test.Among the 4 cases of high-risk AIMAH,2 cases presented with osteoporosis,2 cases with hepatic dysfunction,3 cases with cardiopulmonary dysfunction,and 4 cases with severe hypertension.Three cases of subclinical AIMAH were treated with symptomatic treatment,10 cases of clinical AIMAH patients with surgical operation,4 cases of high-risk AIMAH patients with ketoconazole and surgical operation.Results Three subclinical AIMAH patients received symptomatic treatment and discharged from hospital with normal blood pressure and blood glucose.During the period of follow-up from 3 months to 3 years,endocrine results were normal.Seven clinical AIM AH patients underwent unilateral adrenal tumor resection plus ipsilateral partial adrenalectomy or total adrenalectomy.CS disappeared completely after 6 to 9 months.Two clinical AIMAH patients underwent simultaneous bilateral adrenalectomy.One case died of adrenal crisis after operation,and the other case presented with adrenal insufficiency but returned to normal after glucocorticoid replacement therapy,no Nelson's syndrome happened during the follow-up for 5 years.One clinical AIMAH patient undertook unilateral adrenalectomy twice by interval,followed by routine corticosteroid replacement therapy.Followed up for 10 years,no Nelson's syndrome happened.Four high-risk AIMAH patients received ketoconazole and then underwent right total adrenalectomy.Cortisol levels returned to normal after 1 to 2 months and during the follow-up for 1 to 3 years,the laboratory examinations maintained normal.Conclusions Different treatment methods should be adapted to different subtypes of AIMAH.For subclinical AIMAH,the principal treatment is symptomatic,and close follow-up with regular adrenal imaging and endocrine examination is required.Surgical operation should be performed when clinical symptoms of AIMAH appear.Medical management is essential for high-risk AIMAH to inhibit the production of cortisol at first.Once these patients could stand the stimulation caused by operation,the adrenal glands should be resected as soon as possible.The unilateral adrenalectomy is an effective treatment for clinical AIMAH.
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Objective To improve the diagnosis and treatment of ACTH-independent macronodular adrenal hyperplasia(AIMAH).Methods A 51-year-old female patient with Cushing's syndrome caused by AIMAH was reported.Elevated early morning plasma cortisol levels,increased 24 h urinary free cortisol excretion,and loss of the normal circadian rhythm in cortisol secretion were presented.There was no suppression of cortisol secretion by administration of low-and high-dose overnight dexamethasone suppression test.Cardio-pulmonary function was very bad with the highest blood pressure reaching 300/120 mm Hg( 1 mm Hg=0.133 kPa).Initially,she was treated with mitotane(60 mg/d),but was not effective.After taking ketoconazole (800 mg/d)for 5 days,cardio-pulmonary function was not effectively improved with blood pressure only descending to 180/120 mm Hg.Orthopnoea appeared and Spo2fell once to 75％.The patient had to undergo right total adrenalectomy immediately.ResultsThe mass resected was 10 cm× 10 cm in size and weighted 67.5 g.Histological examination of the removed adrenal revealed nonpigmented macronodular cortical hyperplasia.The patient continued to take ketoconazole (400-800 mg/d)from the 6th day of the operation without steroid replacement therapy in that period.With normal cortisol levels( plasma cortisol at 8:00 was 18.65 μg/dl,24 h urinary free cortisol was 78.75 μmol),she left hospital after the general condition had been improved.During the following updated 10 months follow up,the indexes of her laboratory examination were maintained normal.ConclusionIndividualized therapy should be adopted for the patient with AIMAH.The medication is useful to suppress the adrenal gland cortisol production for those with progression of symptoms,very high blood pressure,hypokalemia and hypoproteinemia.Once the cardio-pulmonary function improves,the target organ should be resected as soon as possible.The supplement of cortisol is not appropriate during the perioperative period.The unilateral adrenalectomy is an effective treatment for AIMAH.
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ACTH-independent macronodular adrenal hyperplasia (AIMAH) is an uncommon cause of Cushing's syndrome (CS). The pathophysiology of this disorder is heterogeneous in its molecular origin and also in its clinical presentation. AIMAH can present mainly as an incidental radiological finding with sub-clinical CS or rarely with overt CS. In a few familial cases reported with AIMAH, specific aberrant G-protein coupled receptors were expressed in the adrenals of all affected members, but sporadic cases are more common. The aberrant adrenal function of G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis. Unilateral or bilateral adrenalectomy has been the most frequently used treatment for this adrenal disorder; alternatively, the identification of aberrant receptors using in vivo protocol of investigation can offer specific pharmacological approach to control abnormal steroidogenesis and possibly prevent AIMAH progression.
Subject(s)
Adrenalectomy , Cell Proliferation , Cushing Syndrome , GTP-Binding Proteins , HyperplasiaABSTRACT
Objective To explore the diagnosis and treatment of adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH). Methods The clinical symptoms, endocrinological and radiological characteristics, treatment modalities, and postoperative clinical course of 12 AIMAH patients were retrospectively reviewed. There were 5 men and 7 women with an average age of 48 years old. Results All cases had some clinical features and biochemical evidence of Cushing's syndrome. The circadian rhythm of serum cortisol was abnormal. Low- and high-dose dexamethasone suppression tests failed to suppress cortisol secretion. CT or MRI scan showed bilateral enlargement of the adrenal glands with multiple macronodules. Bilateral adrenalectomy was performed in three patients and unilateral adrenalectomy combined with contralateral subtotal adrenalectomy was performed in one patient. Seven patients underwent unilateral adrenalectomy of the larger gland. The removed adrenal glands weighted within 8-96 g range and pathologic diagnoses were nodular or adenomatoid hyperplasia of adrenal cortex. The duration of postoperative follow-up was 14 to 124 months. Clinical symptoms of Cushing's syndrome disappeared after surgery in all cases and there was no Nelson's syndrome after bilateral adrenalectomy. Urinary free cortisol and serum cortisol were within normal ranges. No further enlargement of the contralateral gland has been noticed in patients who received unilateral adrenalectomy during the follow-up.Conclusions AIMAH had unique endocrinological and pathological features. Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH. Bilateral adrenalectomy or unilateral adrenalectomy combined with contralateral subtotal adrenalectomy may be performed if the symptoms had not been improved or recurred after unilateral adrenalectomy.
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ObjectiveTo investigate the diagnosis and treatment of adrenocorticotropin-independent macronodular adrenal hyperplasia (AIMAH). MethodsThe clinical data of 14 cases of AIMAH from August 1972 to July 2010 were retrospectively analyzed.The cases included 5 males and 9 females with a mean age of 45 (range 26 to 58 ) years.Ten patients demonstrated typical Cushing's syndrome (CS) and 4 patients presented with weight gain,hypertension or diabetes mellitus without any signs of CS.The circadian rhythm of serum cortisol was abnormal.Low and high dose dexamethasone suppression tests failed to suppress cortisol secretion.CT scan showed bilateral enlargement of the adrenal glands with multiple macronodules.All patients underwent open surgery,including 5 cases of unilateral adrenalectomy,6 cases of adrenalectomy combined with contralateral subtotal adrenalectomy and 3 cases of bilateral adrenalectomy.ResultsIt was established by pathological examination that all patients had bilateral adrenal macronodular or adenomatoid hyperplasia.During the mean follow-up of 69 months (range 12 to 120 months),the clinical symptoms of CS disappeared after surgery in all cases.The 5 patients who received unilateral adrenalectomy had urinary free cortisol and serum cortisol within normal ranges and no further enlargement of the contralateral gland was noticed.Among the 3 patients who received bilateral adrenalectomy,1 case died of adrenal crisis on day seven post-operation.The remaining 2 cases presented with adrenal insufficiency but returned to normal after glucocorticoid replacement therapy.Nelson's syndrome was not observed in the other patients.ConclusionsAIMAH has unique endocrinological and pathological features,presenting as an independent etiology of CS.Unilateral adrenalectomy appears to be an effective and safe alternative treatment for AIMAH and long-term remission can be achieved.Bilateral adrenalectomy or unilateral adrenalectomy combined with contralateral subtotal adrenalectomy may be performed if the symptoms have not improved or recurred after unilateral adrenalectomy.
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ACTH Independent cushing syndrome, involving both adrenal glands, represents a minority of endogenous hyperadrenocorticisms and is caused by infrequent diseases. ACTH independent macronodular adrenal hyperplasia (AIMAH) is one of them and is caused by aberrant cortical adrenal receptors expression that are able to stimulate cortisol secretion. We report a 62 years old male, presenting with hypertension, weight gain, urinary lithiasis and osteopenia. The diagnosis of Cushing's syndrome was made with a morning cortisol level after 1 mg nocturnal dexamethasone of 8.2 ug/dL. Urinary free 24 hour cortisol levels were normal, circadian rhythm of cortisol was maintained and ACTH was <5 pg/mL. Abdominal magnetic resonance imaging showed bilateral nodular adrenal enlargement. The functional study, looking for abnormal receptors, showed a potent cortisol secretory response after stimulation with vasopressin.