Adrenal-preserving laparoscopic surgery in selected patients with bilateral adrenal tumors

According to some authors, a higher incidence of subclinical hypercortisolemia is found among patients with bilateral benign adrenal tumors than in those with unilateral tumors. It is still unknown whether all patients with bilateral adrenal tumors and subclinical hypercortisolemia should undergo surgery, and, if so, which tumor should be removed first. The aim of the study was to investigate whether unilateral adrenalectomy can lead to resolution of hypercortisolemia in patients with bilateral adrenal tumors and to improvement of their clinical status. The study group consisted of 25 patients with bilateral benign adrenal tumors and subclinical hypercortisolemia. In 24 patients, unilateral adrenalectomy was performed. The adrenal gland was selected for removal on the basis of scintigraphy and/or tumor diameter. Cortisol concentrations were measured before the surgery and at 1 and 6 months after the surgery at 8:00 AM, 10:00 PM, and after dexamethasone suppression. The morning blood levels of adrenocorticotropic hormone, dehydroepiandrosterone, 17‑hydroxyprogesterone, glycated hemoglobin, and lipid profile were determined. In all surgical patients, hypercortisolemia resolved after the surgery. However, only in 14 patients (58%), the clinical improvement was evident (improved control of diabetes and hypertension, body mass loss). Although subclinical hypercortisolemia resolved after surgery in all patients with bilateral adrenal tumors, only patients with poorly controlled diabetes and hypertension and a rapid increase in body mass benefited from the surgery.

Bilateral adrenal neuroblastoma (NB) is rare and is mainly stage 4S. Its incidence, presenting features, management, and outcome have not been fully defined yet. We searched the Italian NB Registry (RINB) for stage 4S NB infants with bilateral adrenal primary tumor to compare them with stage 4S NB with unilateral tumor. Between 1979 and 2016, the RINB enrolled 3731 NB patients aged 0-18 years including 317 infants (8.5%) diagnosed with stage 4S NB. Eleven/317 (3.5%) had a bilateral adrenal primary tumor (Group 1) and 190/317 (59.9%) had a unilateral tumor (Group 2). Group 1 infants were significantly younger (51 vs. 89 days) but were comparable with Group 2 for any other presenting features. In the absence of specific protocols, upfront treatment was based on symptoms, size of adrenal tumors, and biology, and consisted of observation in 5 cases, radiotherapy in one, chemotherapy in 2, and surgery in 3. Five/11 developed progression and 2 of them, both with MYCN amplification, died. The 5-year EFS rates of Group 1 and 2 were 54.5% vs. 73.3% (P=.14) and 5-year OSs were 81.8% and 89.4%, respectively (P=.44). Our data support the hypothesis that 4S NB infants with bilateral adrenal tumors can have favorable outcome with personalized therapeutic approach. The three patients with MYCN amplified tumor benefited from upfront aggressive chemotherapy, in accordance with current protocols. Because of the rarity of this intriguing form of neuroblastoma, collaborative prospective studies are warranted, especially in view of gaining a better insight on its biological and genetic features.

Objective To improve the recognization of bilateral adrenal tumors from different tissues and to discuss the treatment.Methods One case of bilateral adrenal tumors from different tissues:pheochromocytoma in one side and adrenocortical adenoma in the other side was reported and the data of syptoms,physical features,auxillary examination,diagnosis and treatment were retrospectively reviewed.Results The patient was diagnosed as adrenocorticotropic hormone (ACTH) -independent Cushing's syndrome in other hospital and received laparoscopic adrenalectomy for right adrenal tumor.During the operation the blood pressure fluctuated seriously and was even more than 200 mm Hg.The pathological report showed the mass was pheochromocytoma.After the operation,the patient's clinical manifcstation was not changed.Half a year later the lab test showed blood ACTH was still less than 5 pg/ml,24 h urinary-free cortisol (UFC) was 571.32 μg,and 24 h urinary catecholamines (U CA) was:noradrenalin (NE) 22.80 μg,epinephrine (E) 2.55 μg,dopamine (DA) 92.92 μg.CT detected a mass in left adrenal gland.The patient received laparoscopic adrenalectomy for left adrenal tumor after being transferred to Peking Union Medical College Hospital.The tumor was proved as adrenocortical adenoma by postoperational pathology.One week after the operation,the 24 h UFC was 56.2 μg.Conclusions Bilateral adrenal tumors from different tissues are very rare in clinic.Adrenalectomy for tumors from both sides and remaining the normal adrenal glands are recommended. Key words: Bilateral adrenal tumors ; Pheochromocytona; Cushing's syndrome ; Histology

BackgroundCoexistence of a catecholamine-secreting tumor and an adrenal cortical tumor is quite rare which makes both diagnosis and management challenging. The purpose of this article is to describe the presence of this condition, share a stepwise approach for preoperative evaluation, and review the related literature.Case presentationA 44-year-old male patient had a history of hypertension and aggravating hypokalemia for years. Abdominal computed tomography incidentally found concomitant bilateral adrenal and left para-aortic tumors. Comprehensive adrenal hormone tests revealed a high aldosterone renin ratio and mildly elevated 24-h urine vanillylmandelic acid and norepinephrine levels. Subsequently, a metaiodobenzylguanidine scan showed uptake over the left para-aortic tumor, and NP-59 adrenal scintigraphy showed uptake over the left adrenal tumor. Further confirmatory tests, including captopril suppression, irbesartan suppression, and saline infusion, all confirmed the diagnosis of hyperaldosteronism. Adrenal venous sampling following 2 months of preparation with an alpha blocker demonstrated a left aldosterone-producing adrenal adenoma. Combining hormonal analysis, imaging studies, and adrenal venous sampling, the patient was diagnosed with left adrenal aldosteronoma, right adrenal nonfunctional tumor, and left para-aortic paraganglioma (PGL). Accordingly, laparoscopic left adrenalectomy and left PGL excision were performed smoothly under alpha blocker maintenance. The pathology report confirmed left adrenal cortical adenoma and left para-aortic PGL. Postoperatively, the blood pressure, biochemical tests, and adrenal hormone assays returned to normal, and related symptoms disappeared and were relatively stable during the follow-up period of two years.ConclusionsThis is the first case of left para-aortic PGL coexisting with an ipsilateral aldosterone-producing adenoma presenting as a left para-aortic tumor associated with bilateral adrenal tumors. Awareness of the rarity of this coexistence can avoid unexpected disasters during the process of evaluation and management.

Introduction The field of Cushing syndrome is a various area; there are still subjects incompletely clarified as the subclinical pattern as well as cortisol producing bilateral adrenal tumours. Case presentation The paper presents a 67-year old male case previously known with metabolic complications. He had an abdominal ultrasound done for unspecific complains and multiple gallbladder stones together with a right adrenal tumour were found. Later the computer tomography revealed bilateral adrenal tumours of almost 1.5 centimetres diameters (right larger than left) and a morning plasma cortisol level of 2.58 micrograms/ decilitre after low dose of dexametasone suppression test confirming the subclinical Cushing syndrome. After 6 months the endocrine and imagery profile was similar but the gallbladder patter aggravated so surgery was performed (together with right adrenalectomy) by a laparoscopic procedure. The blood pressure profile improved after surgery. Conclusion Subclinical Cushing syndrome diagnosis is challenging especially if metabolic complications or bilateral adrenal tumours are presented. Based on our observations in this case unilateral adrenalectomy improved the metabolic pattern

Cushing’s syndrome accounts for approximately 20–30% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushing’s syndrome due to bilateral adrenal tumors is extremely rare. Adrenal oncocytomas are another rare cause of endogenous hypercortisolism: about 13 cases are described in the literature. Oncocytomas are rare epithelial neoplasms, characterized by abnormally excessive accumulation of defective mitochondria in the cytoplasm of cells, and make up 1.8% of all adrenal neoplasms. We describe a 58-year old patient with Cushing’s syndrome and bilateral adrenal tumors. Multispiral computed tomography of the adrenals showed signs suspicious of lipid-poor atypical adenomas or malignant tumors. Surgical treatment was the method of choice, and the larger tumor was excised first. Due to the absence of remission of endogenous hypercortisolism the excision of the second tumor was performed. Morphological and immunohistochemical examination confirmed the diagnosis of bilateral oncocytic adrenocortical tumors with uncertain malignant potential. Cases of bilateral hormone-producing adrenal oncocytomas have not been described in the literature.

Bilateral adrenal tumors are very uncommon in clinical practice and all originate from the same histology. We presented here a case report and literature review of bilateral adrenal tumors from different histology: pheochromocytoma in one side and adrenocortical adenoma in the other side. The patient was a 37 years old female suffered from Cushing's syndrome form 3 years. One year ago she was diagnosed as ACTH-independent Cushing's syndrome and received Laparoscopic adrenalectomy for right adrenal tumor which diagnosed as a pheochromocytoma by the pathological reports. After the operation, patient's clinical manifestation was not change, then half-year later the lab test showed no improvement in the blood biochemical parameters. Finally, CT detected a mass in left adrenal gland. Thereafter, this patient received Laparoscopic adrenalectomy for left adrenal tumor. The tumor was diagnosed as adrenocortical adenoma by the pathologists. One week after operation, the blood biochemical parameters became normal. In conclusion, bilateral adrenal tumors from different histology are very rare, adrenalectomy for both side tumors and preserving the normal adrenal glands is necessary.

We report three cases of Cushing's syndrome (CS) with bilateral adrenal tumors. When bilateral adrenal tumors are encountered, a differential diagnosis is difficult to make, especially in the case of functioning bilateral adrenocortical adenoma. Adrenal scintigraphy has become a standard technique to determine the laterality of excessive hormone secretion; however, this examination results in bilateral adrenal activity in the functioning bilateral adrenocortical adenoma. Our three patients were diagnosed with adrenocorticotropic hormone (ACTH)-independent CS based on biochemical testing, and an abdominal computed tomography (CT) scan detected bilateral adrenal tumors. Adrenal scintigraphy showed bilateral adrenal activity in all cases. However, adrenal venous sampling (AVS) demonstrated three different hormone-excess patterns (case 1: bilateral cortisol-excess secretions; case 2: unilateral cortisol-excess secretion and bilateral aldosterone-excess secretions; and case 3: bilateral cortisol-excess secretions and bilateral aldosterone-excess secretions). Based on these findings, we could select optimal treatment for each case. Therefore, AVS is useful to obtain a definitive diagnosis and adequate therapy for CS with bilateral adrenal tumors.

Effect of surgical versus conservative management on cardiovascular outcomes in patients with bilateral adrenal tumours and cortisol excess: an international, retrospective cohort study.

Objective To determine signalment, clinical signs, concurrent diseases, response to surgical treatment, and long-term outcome of ferrets with bilateral adrenal tumors or adrenal hyperplasia. Design Retrospective study. Animals 56 ferrets with bilateral adrenal tumors or adrenal hyperplasia confirmed histologically following subtotal bilateral adrenalectomy. Procedure Medical records of all ferrets with bilateral adrenal tumors or hyperplasia examined between 1994 and 1997 were reviewed. Ferrets underwent a subtotal bilateral adrenalectomy or a unilateral adrenalectomy initially, followed by a unilateral subtotal adrenalectomy when tumors or hyperplasia later developed on the contralateral adrenal gland. A long-term follow-up of a minimum of 18 months after final adrenal gland surgery was obtained by examination of medical records and follow-up telephone conversations. Results Clinical signs of hyperadrenocorticism included bilaterally symmetric alopecia, return to male sexual behavior in castrated male ferrets, or swollen vulva in spayed female ferrets. Surgical treatment of bilateral adrenal disease by subtotal bilateral adrenalectomy (or unilateral adrenalectomy followed by contralateral unilateral subtotal adrenalectomy) was effective with a mortality rate of < 2%. Only 3 (5%) ferrets required glucocorticoid or mineralocorticoid replacement following subtotal bilateral adrenalectomy. Recurrence after bilateral adrenalectomy was 15% with a mean long-term follow-up period of 30 months. Conclusions and Clinical Relevance Bilaterally symmetric alopecia, return to male sexual behavior in castrated male ferrets, or swollen vulva in spayed female ferrets are indicative of adrenal tumors or adrenal hyperplasia in ferrets. Surgical treatment of bilateral adrenal disease by subtotal bilateral adrenalectomy is effective, with a low rate of complications and postoperative recurrence rate. (J Am Vet Med Assoc 1999;215:820–823)

Adrenalectomy for adrenal tumor was performed on 25 patients at Kushiro municipal general hospital between 1982 and 1993. There were 17 women and 8 men aged 34 to 66 (mean 52.7) years. Clinical diagnoses were pheochromocytoma (6), Cushing's syndrome (8), primary aldosteronism (4), incidentaloma (7). Pathological diagnoses were pheochromocytoma (6), cortical adenoma (17), hyperplasia with hypercortisolism (1), schwannoma (1). Angiography and venous sampling were unuseful for clinical identification of adrenal tumor. After removal of the tumor, glucose-intolerance disappeared in 4 of 6 cases with pheochromocytoma and one of 7 cases with incidentaloma, mental aberration was improved in 2 of 8 cases with Cushing's syndrome, and hypertension was improved in 4 of 7 cases with incidentaloma. Since clinical symptoms (hypertension and glucose-intolerance) were improved postoperatively in 4 of 7 cases with incidentaloma, adrenalectomy is recommended for incidentaloma. Transabdominal approach was suitable for pheochromocytoma and bilateral adrenal tumor, but postoperative recovery was slow. In our experience, pleural injury was found in 40% of translumbar approach. Four laparoscopic operations were performed recently, this procedure gave most fast postoperative recovery to compare with other approach.

A 65 year-old man visited the outpatient clinic of internal medicine complaining of fever and upper abdominal pain. Bilateral adrenal tumors were detected by CT scan and ultrasonography without any hormonal abnormalities. The diagnosis was bilateral primary non-functioning adrenal tumors because there was no tumor except adrenals with the detailed examinations. Then we are informed about the patient and he was transferred for the surgery. Cortisol was administrated because adrenal insufficiency was observed before surgery. Right adrenalectomy was performed after the recovery of patient status with cortisol. The pathological diagnosis was malignant lymphoma (diffuse large, B cell type). The patient was getting worse and dead 15 days after the surgery.

Subclinical Cushing's syndrome (SCS) is characterized by subtle autonomous cortisol secretion from adrenal tumors without specific signs and symptoms of hypercortisolism. Patients with SCS have a high prevalence of "lifestyle-related diseases," such as hypertension, diabetes mellitus, dyslipidemia, and osteoporosis. Long-term follow-up of SCS patients is reportedly indispensable for establishing indications for surgical treatment of SCS. We performed a follow-up survey of 27 patients with SCS (median: 5.3 years) and compared those who had undergone surgical treatment (n=15) with those who had not (n=12). The mean diameter of tumors was 31 mm; 16 (59%) patients had unilateral lesions and 11 (41%) carried bilateral ones. In 67% and 60% of the treatment group, respectively, hypertension and diabetes mellitus improved. We also noticed that eight of 11 (73%) SCS patients with bilateral adrenal tumors had extra-adrenal malignancies in various tissues. Interestingly, among nine SCS patients who had malignancies, eight showed bilateral adrenal uptake in ¹³¹I-aldosterol scintigraphy. The results imply that surgical treatment can reduce cardiovascular risks in SCS patients. Screening for malignancy may be necessary in patients with bilateral adrenal tumors suspected of autonomous hypersecretion of cortisol from both sides.

We present an educational case of bilateral adrenal tumor. A 52year-old woman underwent abdominal ultrasonography for screening purposes, which detected bilateral adrenal tumor. On non-contrast-enhanced CT, the left adrenal tumor had a lowdensity area indicating a fatty component (a). Contrast-enhanced CT revealed central necrosis in the right adrenal tumor (b). T2weighted MR imaging showed that the right tumor had high intensity (c). The left tumor appeared with lower intensity on inphase MR imaging (d) compared to out-of-phase MR imaging (e), indicating a fatty component. I-metaiodobenzylguanidine (MIBG) highly accumulated in the right tumor with preserved physiological uptake in the left tumor (f). The left tumor showed high uptake of I-adosterol with no accumulation in the right tumor (g). These results of scintigraphy, consistent with CT and MR findings, suggested that the left tumor was an adrenocortical adenoma and that the right tumor was a pheochromocytoma [1]. Laboratory tests revealed elevated urine metanephrines but normal corticosteroids. The patient underwent laparoscopic right total adrenalectomy. The right tumor was pathologically diagnosed as a pheochromocytoma with no malignancy. The left tumor was not resected. Surgery is the primary treatment for pheochromocytoma [2]. In contrast, operation is not recommended for hormonally inactive adrenocortical adenoma less than 4 cm in diameter [3]. This patient had bilateral adrenal tumors, which confused the diagnosis and treatment planning because pheochromocytoma occasionally involves bilateral adrenal glands, especially in multiple endocrine neoplasia [4]. A thorough series of noninvasive imaging techniques consisting of CT, MRI, and dual adrenal scintigraphy avoided unnecessary and invasive bilateral tumorectomy and thus prevented adrenal dysfunction.

Objective To investigate the operative indication, therapeutic effects and feasibility of laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma. Methods The clinical data of 9 cases of bilateral pheochromocytoma from Jan. 2011 to Dec. 2016 were retrospectively analyzed. The cases included 7 males and 2 females with a mean age of 40 years old, ranging from 22 to 58. CT showed all the 9 cases had bilateral adrenal tumors. Results All patients accepted laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma. All survived and had been followed up for 4 months to 6 years (median 3.2 years) . All cases kept normal adrenal function and avoid long-term corticosteroid dependence. Conclusions Bilateral pheochromocytoma is rare and difficult to treat. Adrenal function protection under laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma is safe and effective for patients. This opertation can both completely resect bilateral adrenal tumors and avoid long-term corticosteroid dependence. Key words: Pheochromocytoma; Bilateral; Laparoscopic; Cortical-sparing