Simultaneous Bilateral Adrenalectomy Research Articles

Bilateral pheochromocytoma: Clinical characteristics, treatment and longitudinal follow‐up

Comprehensive data about patients with bilateral pheochromocytoma are limited. We aimed to describe the clinical presentation, genetic analysis, treatment and outcomes of patients with bilateral pheochromocytoma. A retrospective study at a tertiary care centre. All patients with bilateral pheochromocytoma evaluated at Mayo Clinic in Rochester, Minnesota between January 1951 and December 2015. Tumour size, genetic testing, plasma/urine metanephrines and catecholamines. A total of 94 patients (51% women) were diagnosed with bilateral pheochromocytoma at a median age at first presentation of 31years (range, 4-70). Bilateral disease was noted in 8.0% of pheochromocytoma patient overall and 37.5% of patients 18years of younger. Most patients presented with synchronous tumours (80%). Median time to metachronous tumours was 4.5years (range, 1-38). Genetic disease was identified in 75 (80%) patients, including MEN 2A (42.6%), VHL (19.1%), MEN 2B (9.6%) and NF1 (8.5%). Excess catecholamines were present in 97% of patients. Patients with synchronous pheochromocytoma commonly underwent simultaneous bilateral adrenalectomy (99%), and 18 (24%) had cortical-sparing surgery. Multicentric tumours were reported in 23 of 77 (30%) patients with available data. Recurrent disease was found in 9.6% of patients, and 8.5% developed metastatic disease. Median follow-up was 8.5years. At the study conclusion, 4 patients had died due to pheochromocytoma or adrenalectomy. Bilateral pheochromocytoma occurred in 7.0% of adults with pheochromocytoma and 37.5% of paediatric patients. Genetic disease was identified in 80% of patients, predominantly MEN2A. Multicentric tumours were common, but most were still cured following adrenalectomy.

The clinical patterns and RET proto-oncogene identification of pheochromocytoma in 13 multiple endocrine neoplasia type 2A pedigrees

To explore the clinical patterns and clinical significance for RET screening in adrenal pheochromocytoma (PHEO) associated with multiple endocrine neoplasia type 2A (MEN2A). The clinical data of 32 PHEO patients with MEN2A from 13 unrelated MEN2A pedigrees from August 1989 to January 2013 were analyzed. The comprehensive medical data included systemic examinations and germline RET gene screening. Among 68 patients belonging to 13 MEN2A families, 32 (47.1%) presented with PHEO. There were 19 males and 13 females with a mean age of (41 ± 12) years. And the mean maximum diameter of PHEO was (4.6 ± 2.2) cm. The diagnosis of PHEO was made after medullary thyroid carcinoma (n = 12, 37.5%), simultaneously (n = 12, 37.5%), initially (n = 7, 21.9%) and death during appendectomy for PHEO-induced hypertensive crisis (n = 1, 3.1%). The diagnosis of PHEO was made before (n = 22) or after (n = 10) clinical screening. The former had 12 symptomatic cases while the latter only 1 case (12/22 vs 1/10, P = 0.024).Except for 5 asymtomatic fatal cases during non-PHEO operations, bilateral PHEO was found in 17 cases including 3 unilaterally treated cases developing another PHEO in contralateral adrenal with a lag period of 5, 10 and 17 years. There were 7 symptomatic patients in bilateral cases versus 6 in unilateral cases (7/17 vs 6/10, P = 0.440). Twenty-five patients underwent PHEO surgery: laparascopic approach in 14 cases (8 with bilateral simultaneous adrenalectomy) and open approach in 11 (2 with bilateral simultaneous adrenalectomy). And 10 patients undergoing bilateral adrenal-sparing operations or adrenalectomy required hormonal replacement therapy. During a mean observation period of 72 (1-282) months, no local recurrence, distant metastasis or Addisonian crisis were noted in 25 cases (contralateral relapse in 3 cases). Among them, 2 cases developed adrenocortical insufficiency unresponsive to an adjustment of hormonal doses.RET screening showed 4 recurrent missense substitutions in 32 MEN2A-PHEO patients: p. C634Y exon 11 (n = 27, 84.4%), p. C634R exon 11 (n = 3, 9.4%), p. C634F exon 11 (n = 1, 3.1%) and p. C618R exon 10 (n = 1, 3.1%). The mutations of RET proto-oncognene of PHEO in MEN2A are frequently located at codon 634. A combination of pedigree examination and RET gene screening may facilitate an early diagnosis and early treatment of asymptomatic PHEO patients in MEN2A.Laparoscopic cortical-sparing adrenalectomy for preserving adrenocortical function is a preferred surgical approach.

Laparoscopic Bilateral Transperitoneal Adrenalectomy for Cushing Syndrome

Laparoscopic adrenalectomy is well established for treatment of adrenal lesions. However, bilateral adrenalectomy for Cushing syndrome is a challenging and time-consuming operation. We report our experience of laparoscopic bilateral adrenalectomy for this disease in 19 patients. From September 2009 to August 2012, we have operated 19 patients with Cushing syndrome and performed bilateral laparoscopic adrenalectomy using the transperitoneal approach; synchronous in 15 patients and staged in 4 patients. In 15 patients, the surgery was carried out sequentially on both the sides in lateral position with intraoperative change in position. Complete adrenalectomy including periadrenal fat was carried out on both the sides. Nineteen patients were referred from Department of Endocrinology for bilateral adrenalectomy for adrenocorticotropin hormone (ACTH)-dependent and ACTH-independent Cushing syndrome. The indications for surgery were Cushing disease in 15 patients, occult/ectopic source of ACTH in 2 patients, and primary adrenal hyperplasia in 2 patients. Fifteen patients underwent bilateral adrenalectomy during the same operation. Four patients underwent staged procedures. All procedures were completed laparoscopically with no conversions. The mean operating time for simultaneous bilateral adrenalectomy was 210 minutes (range, 150 to 240 min). This included the repositioning and reprepping time. There were no major intraoperative complications. The average blood loss was 100 mL (range, 50 to 200 mL). None of the patients required blood transfusions in the postoperative period. The postoperative complications included minor port-site infection in 2 patients. One severely debilitated patient died on the 14th postoperative day because of hospital-acquired pneumonia. The remaining 18 patients have done well in terms of impact on the disease. Laparoscopic bilateral adrenalectomy for Cushing syndrome is feasible and safe. It confers all the advantages of minimally invasive approach such as less postoperative pain, shorter hospitalization, lesser wound complications, and faster recovery. The advantages of the laparoscopic approach have led to an earlier referral for bilateral adrenalectomy by endocrinologist in patients with failed pituitary surgery.

Laparoscopic simultaneous bilateral adrenalectomy for testosterone‐secreting bilateral adrenal tumors

Laparoscopic adrenalectomy is widely accepted as a safe and minimally-invasive procedure. Although it is a standard procedure for the surgical treatment of adrenal tumors, its simultaneous use with bilateral adrenalectomy is relatively rare. A 21-year-old woman was referred to Hamamatsu University School of Medicine University Hospital complaining of a deepening voice, hirsutism and secondary amenorrhea. Abdominal computed tomography showed bilateral adrenal tumors, and hormonal examinations showed that the tumors secreted excessive testosterone, resulting in virilizing symptoms. Laparoscopic simultaneous bilateral adrenalectomy was carried out. Postoperatively, serum testosterone levels immediately recovered to within the normal range. Menstruation began the month after the operation, and the hirsutism gradually regressed. This is the third reported case of bilateral virilizing adrenal tumors, and the first to be successfully treated with laparoscopic simultaneous bilateral adrenalectomy.

Safety and curative effect analysis of bilateral simultaneous adrenalectomy for the treatment of the bilateral adrenal pheochromocytoma associated with multiple endocrine neoplasia type 2A

Objective To study the clinical features of the bilateral adrenal pheochromocytomas (PHEO) in patients associated with multiple endocrine neoplasia type 2A (MEN2A) and to evaluate the safety and feasibility of applying simultaneous bilateral adrenalectomy (SBA).Methods From November 2003 to April 2011,clinical data of 8 patients from 4 unrelated MEN2A families treated with SBA were analyzed.There were 6 males and 2 females.The mean age was 44 years (range,35 to 56 years).4 patients were asymptomatic and 4 had clinical features related to PHEO.The diagnosis of bilateral PHEO was made after the diagnosis of MTC in 4 cases,previous hypertension in 1 case,and family investigation found 3 MTC and all were found with hyperparathyroidism.Plasma catecholamines were substantially elevated in 4 patients.Ultrasound and computerized tomography (CT) examination showed bilateral adrenal masses,of which 6 were bilateral and multiple tumors,2 were single tumor in one side and multiple tumors in the other side.On contrast CT,the tumors displayed heterogeneous contrast enhancement and low density area in the center of the tumors observed in 3 cases;renal,splenic vessels displacement and deformation were observed;local lymph node enlargement was not found.The mean maximum diameter of PHEO was 4.4 cm (range,2.7 to 7.0 cm).RET screening showed a heterozygous nucleotide substitution on exon 11,c.G1901A (p.C634Y) in all patients.After adequate adrenergic blockade,the SBA was performed by a single surgeon,PHEO were extirpated by laparoscopic radical adrenalectomy in 2 patie.nts,while in other 6 patients,PHEO were removed by partial adrenalectomy or adrenal tumorectomy with at least one normal appearing adrenal tissue preserved (4 by laparoscopic approach and 2 by open approach).Results The procedures were successful carried out on 8 cases with bilateral PHEO.There were totally 43 pieces of tumor obtained.The average time of operation was 181 min (range,150 to 240 min).Mean blood loss was 171ml (range,100 to 300 ml).Blood pressure fluctuations happened in 2 cases during operation (maximum:230/110 vs minimum:70/40 mm Hg).Histopathology showed PHEO in all patients.During the follow-up of 35 months (range,3 to 90),local and/or distant metastatic recurrence was not found in all cases,while two treated by total bilateral adrenalectomy and one by adrenal-sparing surgery required corticosteroid replacement.Conclusions SBA for bilateral PHEO in MEN2A is technically safe and feasible.Transperitoneal laparoscopic partial adrenalectomy for the treatment of bilateral PHEO can be considered as preferential choice. Key words: Multiple endocrine neoplasia type 2A; Pheochromocytoma; Bilateral simultaneous adrenalectomy; RET gene; Laparoscopy

Laparoscopic simultaneous bilateral adrenalectomy: Assessment of feasibility and potential indications

To report a single-center experience with laparoscopic simultaneous bilateral adrenalectomy (LSBA) and to evaluate its safety, surgical outcomes, and potential indications of the procedure. A total of 21 patients underwent LSBA between 2000 and 2010 at our institution. Four patients had bilateral Cushing's syndrome (CS), two had bilateral pheochromocytoma, and one had a bilateral metastatic tumor. Eleven patients had unilateral or bilateral aldosterone-producing adenoma (APA), associated with CS or subclinical CS. Three patients had unilateral APA with contralateral non-functioning adenoma. Partial adrenalectomy was performed first by using with four ports. After the excision of one gland, the contralateral gland was removed after repositioning of the patient. LSBA was completed in all 21 patients without major complications. Mean operative time was 329.7 min and the estimated blood loss was 94.1 mL. Mean tumor size was 21.8 mm. Of the 16 patients receiving an adrenal-sparing procedure, nine of 11 discontinued glucocorticoid replacement after 2 years. The remaining five patients receiving bilateral total adrenalectomy required 0.5-0.75 mg of dexamethasone permanently. No open conversions, no deaths or no adrenal insufficiencies were encountered. LSBA represents a safe and viable treatment option for selected patients with bilateral adrenal disease.

Postoperative Outcomes of Familial Adrenal Pheochromocytoma1

ABSTRACT There are no current guidelines for the management of familial pheochromocytoma (FP). We tried to determine the optimal management of patients with FP. Among 191 patients with pheochromocytoma who underwent surgical resection between 1979 and 2010, there were 18 FP (13 different kindreds; 11 females/7 males; mean age at initial operation: 38.7 years). The 18 FP cases comprised 10 with MEN2A, 2 with MEN2B, 4 with von Hippel-Lindau disease, and 2 with FP only, and all pheochromocytomas were of adrenal origin. The number of probands and family members was 9 and 9 respectively. Mean tumor size was 6.4 cm in diameter. Simultaneous bilateral adrenalectomy was performed in 6 patients, and unilateral adrenalectomy was performed as the initial surgery in 12 patients. A metachronous contralateral adrenalectomy was performed in 3 patients, 90, 236 and 312 months after the primary operation, respectively. None of the patients received partial adrenalectomy. Among another 9 patients with unilateral adrenalectomy, contralateral pheochromocytomas were suspected in 4 cases at the initial operation. However, none of these contralateral lesions developed severe symptoms or tumor enlargement during a median follow-up of 116 months. In the remaining 5 patients, pheochromocytoma did not develop in the contralateral adrenals over a median follow-up of 80.5 months. Bilateral lesions of adrenal pheochromocytoma in familial cases occurred in 78% of cases (14/18); 9 patients (including 4 with contralateral pheochromocytoma) did not require a contralateral adrenalectomy during a median follow-up of 119 months. No patients have suffered from Addisonian crisis. The ipsilateral adrenalectomy and follow-up of contralateral small pheochromocytoma is one of the management options to preserve adrenocortical function in FP patients.

Simultaneous Bilateral Laparoscopic Adrenalectomy for Adrenocorticotropic Hormone-Independent Macronodular Adrenal Hyerplasia: Report of a Case

Cushing's syndrome caused by adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is an extremely rare disease, which shows bilateral macronodular adrenal hypertrophy and autonomous cortisol production. We herein report a case of AIMAH treated successfully by minimally invasive simultaneous bilateral laparoscopic adrenalectomy. A 73-year-old woman with hypertension, diabetes mellitus, and osteoporosis was referred to our hospital because of an incidentally found huge bilateral adrenal mass. An abdominal computed tomography scan showed large bilateral adrenal glands with multiple nodules. A diagnosis of AIMAH was made and a simultaneous bilateral laparoscopic adrenalectomy was thus performed. The total operation time was 310 min and blood loss was 70 g. Both glands were hypertrophic (right 5 x 3 cm, 48.5 g and left 4 x 2 cm, 39.2 g) and consisted of multiple golden yellow macronodules. The postoperative course was uneventful. A simultaneous bilateral adrenalectomy for AIMAH performed by an experienced surgical team is therefore considered to be a safe and minimally invasive procedure.

Open versus laparoscopic simultaneous bilateral adrenalectomy

Objectives To compare our experience with synchronous bilateral adrenalectomy using laparoscopic and open techniques. Laparoscopic adrenalectomy has become the reference standard for management of benign unilateral adrenal pathologic findings. Methods This was a nonrandomized retrospective chart review of 12 known patients who underwent simultaneous bilateral adrenalectomy, comparing five laparoscopic and seven open procedures. One urologic surgeon performed all laparoscopic cases, and one general surgeon performed all open procedures. Results All patients had Cushing’s disease or syndrome. The average patient age was 47.4 years (range 24 to 71) and 42.4 years (range 19 to 70), with an average body mass index of 38.2 kg/m 2 and 36.0 kg/m 2 for the laparoscopic and open groups, respectively. The operating time was on average 60 minutes longer for the laparoscopic group. No open conversions were necessary. The median blood loss (100 versus 500 mL, P <0.01) and hematocrit drop (8.5% versus 12.6%, P = 0.05) were lower for the laparoscopic group. The transfusion rates and hospital stay trended lower in the laparoscopic group (20% versus 57% and 3 versus 8.5 days, respectively). Specimen weights for both the right and left glands trended larger for the laparoscopic group. The complication rates were similar between groups at 60% for the laparoscopic versus 71% for the open groups. Conclusions Simultaneous laparoscopic bilateral adrenalectomy is safe and effective. Compared with the open approach, it resulted in decreased blood loss, lower transfusion rate, and a trend toward a shorter hospital stay, although the operating time was longer. The laparoscopic approach should be the treatment of choice for bilateral adrenalectomy.

MECHANICAL EVENTS OF IN VIVO RABBIT AND CAT SPLEEN.

Splenic contractions were recorded in 23 rabbits and 6 cats under urethan and pentobarbital anesthesia. Rabbit splenic contractions consist of two distinct phases, a higher-frequency contractile phase (CPI) occurring every 10–45 sec and a slower-frequency contractile phase (CPII) occurring every 5–20 min. Bilateral adrenalectomy eliminates CPII, and splenic nerve block eliminates CPI. Cat splenic contractions consist of only the higher-frequency CPI phase. Bilateral adrenalectomy or splenic nerve block in the cat causes only a reduction in the contractile frequency. Simultaneous bilateral adrenalectomy and splenic nerve block eliminates splenic contractions in both rabbits and cats.