Bilateral Laparoscopic Adrenalectomy Research Articles

P-61 From Cushing to Nelson Syndrome: Diagnostic challenges and Surgical Dilemma

Abstract Introduction Diagnosis of the origin of ACTH-dependent Cushing's syndrome (CS) can be difficult in some cases, especially when MRI of the pituitary gland does not reveal an adenoma. Inferior petrosal (IPSS) or cavernous sinus sampling (CSS) with CRH stimulation helps confirm the central origin in most cases. CSS also helps lateralize the tumor within the pituitary gland with a sensitivity of 80%. Because transsphenoidal pituitary surgery is successful in the majority of MRI-negative cases, bilateral adrenalectomy is performed less frequently. Even with advanced radiologic studies, the origin may not be detected. Adrenalectomy may be an option in these cases. However, loss of feedback inhibition of the hypothalamic-pituitary-adrenal axis; can lead to aggressive transformation of an existing ACTH-secreting pituitary adenoma into Nelson's syndrome within 1-5 years. Clinical Case A 46-year-old female patient complaining of hot flashes and unable to lose weight was evaluated for CS and referred to the Department of Neurosurgery for a pituitary adenoma (Table 1). Since the adenoma was <6mm CSS with CRH stimulation was performed. The results did not support central origin (Table 2). Ectopic ACTH secretion was considered, but without finding the origin of the tumor on radiological examination, and treatment with ketoconazole and metyrapone was started for CS. However the patient then discontinued outpatient follow-up. The patient presented to our clinic 6 years later with a new pituitary MRI showing tumor growth (Fig.1b). She complained of fatigue and obesity. She was depressed and anxious. The skin on her face, hands and hump was dark and patchy (Fig.1c). During these years, she consulted several endocrinologists. MRI was performed annually. Brain MRI had showed a fronto-basal extraaxial mass lesion. The patient underwent microsurgical resection and pathology revealed a microcystic meningioma (WHO grade I). 3 months later bilateral nodular hyperplasia of adrenal gland was detected; she underwent bilateral laparoscopic adrenalectomy (no neoplasia). ACTH gradually increased up to 418pg/ml; the diagnosis of Nelson's syndrome was established. The patient underwent endoscopic transsphenoidal adenomectomy. The ACTH level was found to be 12pg/ml on the 2nd postoperative day. Pathological examination revealed a ACTH secreting pituitary adenoma (Ki-67:7%). CS patients suffer from emotional lability due to the effects of elevated cortisol and to the physical changes of their body. Proximal muscle weakness and obesity causes fatigue symptoms. The diagnosis is often delayed until complications occur. Differential diagnosis could be difficult and challenging. Patient's emotional lability could adversely affect treatment/follow-up compliance. The presented case had meningioma resection which could be delayed until remission from CD and bilateral adrenalectomy which could be avoided by endoscopic pituitary surgery. This case is a good example of the need for pituitary centers of excellence.Figure.Fig.1.a. MRI scan of the pituitary gland at first admission shows (a) a lesion extending posteriorly inferior to the right half of the pituitary gland, hyperintense at T2-weighted, iso-hypointense at T1-w, with heterogeneous contrast enhancement consistent with pituitary microadenoma (4mm*2mm) Fig.1.b. Progression in size of the adenoma (6mm*4mm) Fig.1.c. Darkening of the skin due to hypersecretion of ACTH. Table 1.Cavernous sinus sampling with corticotropin-releasing hormone stimulation Table 2.Laboratory tests in different times

Bilateral Cortical-sparing Adrenalectomy for the Treatment of Bilateral Aldosterone-producing Adenomas

Abstract Aldosterone-producing adenoma (APA) is 1 of the major subtypes of primary aldosteronism (PA). Although most APA occurs unilaterally, bilateral APAs have rarely been documented. Because of its rarity, optimal management of patients with bilateral APAs has not been established. Here, we report a case of bilateral APAs that was successfully treated with simultaneous bilateral cortical-sparing surgery. A 44-year-old Japanese woman was referred to us for the evaluation of PA. She had typical clinical characteristics of PA, including hypertension, hypokalemia, and high plasma aldosterone concentration with suppressed renin. She was diagnosed as having PA based on the results of confirmatory testing. Computed tomography revealed bilateral adrenal nodules with diameters of 17 and 10 mm on the right and left adrenal gland, respectively. Adrenal venous sampling indicated excess aldosterone production from bilateral adrenal lesions. She underwent simultaneous bilateral laparoscopic partial adrenalectomy that normalized her blood pressure and serum potassium levels. Aldosterone synthase immunohistochemistry on the resected adrenal tumor tissue confirmed the diagnosis of bilateral APAs. Long-term postsurgical follow-up data suggest cure of the disease without the need for glucocorticoid replacement therapy. Bilateral cortical-sparing adrenalectomy appears to be a viable treatment option at least for selected patients with bilateral APAs.

Laparoscopic transabdominal anterior bilateral adrenalectomy (La-TABA): an alternative approach for severe Cushing's syndrome.

Severe uncontrolled Cushing's syndrome (CS) is an acute life-threatening condition. As it is often not responsive to medical therapy, emergency bilateral adrenalectomy (BA) may present the only therapeutic option. Moreover, multiple bone fractures, representing one of the clinical consequences of hypercortisolism, may not permit both prone and lateral patient's positioning during surgery, thus making minimally invasive approaches nonfeasible. We report our preliminary experience with an alternative approach in this selected patients' category. Among 613 patients who underwent endoscopic adrenalectomy at our centre (34-5.6% BA) between January 1997 and October 2021, 3 patients were scheduled for laparoscopic transabdominal anterior bilateral adrenalectomy (La-TABA). Surgery was performed with patient in supine position, with fastened arms and legs to allow Trendelenburg and anti-Trendelenburg movements and lateral rotations. The procedure consisted in a synchronous bilateral adrenalectomy. A modern multi-articulated operative table provided rotations on the right and left side to perform left and right adrenalectomy, respectively. One male and two female patients with a mean age of 34 ± 3.1years underwent La-TABA. Mean operative time was 200 ± 138.3min. No perioperative complications were registered. Mean postoperative hospital stay was 8 ± 4.4days. In very severe ACTH-dependent CS, BA should be performed as soon as possible. La-TABA may represent the most appropriate therapeutic option, as this patients' category is not able to tolerate prone and lateral positioning required during the conventional surgery. Despite the higher technical complexity of the procedure compared with the other endoscopic approaches to adrenalectomy, it retains the benefits of minimally invasive surgery in terms of postoperative complications and recovery.

FRI223 Ectopic ACTH Syndrome

Abstract Disclosure: S. Mallesara Sudhakar: None. E.S. Story: None. Introduction: Ectopic ACTH Syndrome (EAS) is caused by hypercortisolism from extra-hypophysial tumors. EAS accounts for 10-15 % of Cushing’s Syndrome. Case 1: A 36 year old male with recently diagnosed stage IV well differentiated grade 2 neuroendocrine tumor (NET) of the pancreas with liver metastases developed new hypertension, hyperglycemia, hypokalemia, metabolic alkalosis, fatigue and muscle weakness. There were no obvious signs of Cushing’s Syndrome. ACTH was 679 pg/ml, cortisol 120 ug/ml. MRI pituitary was normal. He required 4 antihypertensives, insulin, and metyrapone was initiated for hypercortisolism. Due to difficulty obtaining medications during the COVID pandemic, intolerance to therapies and lack of improvement in clinical symptoms, decision was made to perform laparoscopic bilateral adrenalectomy. He was started on hydrocortisone and fludrocortisone. Pathology revealed diffuse adrenal hyperplasia. Post-surgery he improved dramatically and was able to discontinue antihypertensives and insulin. Chemotherapy for the underlying tumor was discontinued due to cytopenias. His cancer progressed and he succumbed to disease 10 months from initial diagnosis. Case 2: A 60 year old female presented with severe hypokalemia, metabolic alkalosis, hypercalcemia, cytopenias, HTN, weight loss, muscle weakness, nausea and mental status changes. CT abdomen showed bilateral adrenal hyperplasia. ACTH was 489 pg/ml, cortisol 81 ug/ml. MRI pituitary was unremarkable. PET-CT additionally showed hypermetabolic mediastinal lymph nodes and right upper lobe nodular opacity, and widespread osseous lesions. She was started on ketoconazole and antihypertensives. Bilateral adrenalectomy was considered but could not be performed due to rapid clinical deterioration. Etomidate was initiated. Fine needle aspiration of a mediastinal lymph node showed small cell carcinoma. Patient became too frail to receive systemic chemotherapy and succumbed within 2 weeks of hospitalization. Discussion: Most Ectopic ACTH Syndrome cases are caused by NET of the lung, pancreas and thymus. In children, Ewing’s sarcoma has been reported. Small cell carcinoma of lung, malignant pancreatic NETs, and medullary carcinoma of thyroid often present with metastasis and carry an ominous prognosis. Not all patients have clinical features of classic Cushing's syndrome. The outcome of EAS correlates with tumor type. Optimum treatment is surgical resection of the tumor, often not possible with metastatic disease. Hypercortisolism may be controlled with adrenal enzyme inhibitors and receptor blockers. Bilateral surgical adrenalectomy may be needed when symptoms are not controlled by medical management. Early and rapid control of Cushing’s Syndrome is important to facilitate antineoplastic treatment of the underlying tumor. Presentation: Friday, June 16, 2023

FRI267 Carney Complex From A Single Familycarney Complex From A Single Family Followed Up Over 20 Years With Different Clinical Pattern Evolution

Abstract Disclosure: A.W. Kuhn: None. D.S. de Melo: None. H.L. Charchar: None. B.M. Mariani: None. M.Y. Nishi: None. F.L. Ledesma: None. F.Y. Tanno: None. V. Srougi: None. J.L. Chambo: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. M.C. Fragoso: None. Background: Carney complex (CNC) is a hereditary syndrome with autosomal dominant inheritance, composed of multiple neoplasms, such as benign and malignant, endocrine and nonendocrine tumors (cardiac, cutaneous and neural). The primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome is the most common endocrine neoplasia in CNC. The most frequent genetic cause is related with the presence of germline pathogenic allelic variants on PRKAR1A. Overall CNC penetrance among carriers of the PRKAR1A allelic variant is approximately 97.5%, however, families with long-term follow up is rarely described in literature. Clinical Case: A 17-year-old male, was referred to our Hospital in 2003 due to weight gain (≈10 Kg), BMI 27.27 Kg/m2, acne, occurrence of abdomen purplish striae (>1cm), facial plethora and arrest of pubertal development for last 2 years. He has had pigmented facial lesions since 9 years old. Laboratory revealed suppressed ACTH <18.0 pg/mL, failure to suppress cortisol levels after low dose dexamethasone test (DST-1mg): cortisol 29 µg/dL (normal range: <1.8 µg/dL), and total urinary cortisol (TUC), 2809 µg/24 h (normal range: 30-300 µg/24h). Abdominal CT scan showed normal right adrenal gland and a micronodule (0.8 cm) in the left adrenal gland. Bone densitometry showed an important loss of density. Owing to ACTH-independent Cushing's syndrome and adrenal with micronodule, the most plausible diagnostic hypothesis was PPNAD associated to CNC. He underwent bilateral laparoscopic adrenalectomy and the histological data confirmed PPNAD on both adrenal glands. He developed primary adrenal insufficiency and he maintains continuous replacement with gluco and mineralocorticoids. The genetic analysis identified a heterozygous germline nonsense pathogenic variant on PRKAR1A (p.Tyr21X) in exon 2. His relatives, such as father, two younger sisters, one paternal aunt and a female cousin carried the same nonsense variant. All of them also presented facial lentiginous but without Cushing features. In the first investigation, all his relatives showed possible autonomous cortisol secretion. In follow-up of almost 20 years, the index case and his family members did not develop other manifestations of CNC. Only his father developed metabolic syndrome, but all relatives maintained subclinical hypercortisolism due to possible autonomous cortisol secretion without any clinical repercussions. Clinical Lessons: Carney complex is a rare disease and the follow-up of familial cases over the years can show different clinical pattern despite the same germline pathogenic variant. Probably somatic molecular mechanisms as modulators could trigger specifically clinical manifestations. Presentation: Friday, June 16, 2023

Laparoscopic Bilateral Adrenalectomy with the Transabdominal Lateral Approach

Background: Laparoscopic adrenalectomy (LA) is a widely accepted method for most adrenal lesions. However, bilateral LA is performed less often than unilateral adrenalectomy. The most common indication for bilateral LA is adrenocorticotropin hormone (ACTH)-dependent Cushing’s syndrome, including persistent Cushing’s disease following unsuccessful transsphenoidal surgery and ectopic ACTH syndrome. Objectives: This retrospective study was conducted to assess the indications, safety, efficacy, and outcomes for bilateral LA with the transabdominal lateral approach. Methods: This retrospective study was conducted between January 2004 and February 2022. During the study period, transperitoneal LA was performed on 279 patients, among whom, 258 cases were unilateral LA. Therefore, our analysis included 21 consecutive patients who underwent laparoscopic bilateral LA with the transabdominal lateral approach. The surgery indication, tumor side and weight, operation time, conversion to open surgery, need for an additional trocar, complications, hospital stay, and follow-up information were analyzed. Results: Indications of bilateral LA were refractory Cushing’s disease (n=14), occult ectopic primary bilateral macronodular adrenal hyperplasia (n=5), ACTH secretion (n=1), and bilateral pheochromocytoma (n=1). The mean operative time was 207.8±21.3 min, including repositioning time. Intraoperative and postoperative complications were seen in 3 (14%) and 4 (19%) patients, respectively. No conversion to open surgery was observed. Median hospital stay was 7 (range, 5-10) days and median follow-up was 81 (range, 55-94.5) months. Three patients died at 62, 64, and 88 months after adrenalectomy due to heart failure, renal failure, and myocardial infarction, respectively. No adrenal insufficiency or signs of recurrent hypercortisolism was observed. Conclusion: Our results demonstrated that laparoscopic bilateral LA was safe and effective, allowing acceptable morbidity and hospital stay. The most common surgical indication was ACTH-dependent Cushing's syndrome, followed by ACTH-independent Cushing's syndrome. The lateral transperitoneal approach obtains an excellent anatomical view. In our series, operative time and conversion to open surgery rate were in line with the literature.

Laparoscopic transperitoneal adrenalectomy for adrenal tumours of 6 cm or greater: A single-centre experience.

The present study aimed to evaluate the safety and efficacy of transperitoneal laparoscopic adrenalectomy (LA) for large adrenal tumours by comparing the outcomes of tumours larger than 6 cm with those smaller than 6 cm and also to identify the risk factors associated with prolonged operative time in transperitoneal LA. One hundred and sixty-three patients underwent LA at our clinic from January 2014 to December 2020. Bilateral LA was performed in 20 of these 163 patients. A total of 143 patients were included in this study. Data were analysed retrospectively from the patients' medical records collected. Large tumour (LT) group consists of 33 patients and the small tumour (ST) group consists of 110 patients. There was no statistically significant difference between the groups regarding conversion to open surgery and complications. A multiple regression analysis was conducted to identify the independent predictors of prolonged operation time. The tumour size ≥8 cm (odds ratio [OR], 19.132; 95% confidence interval [CI], 3.881-94.303; P < 0.001) and diagnosis of pheochromocytoma (OR, 2.762; 95% CI, (1.123-6.789, P = 0.026) were the significant predictors of prolonged operation time. Our study shows that LA can be considered the treatment of choice for small and large adrenal tumours. The tumour size ≥8 cm and diagnosis of pheochromocytoma are the independent risk factors for the prolonged operative time in transperitoneal LA.

Simultaneous bilateral laparoscopic cortical-sparing adrenalectomy for bilateral pheochromocytomas in multiple endocrine neoplasia type 2.

This study aimed to assess the feasibility of synchronous bilateral laparoscopic or open cortical-sparing adrenalectomy (SB-LCSA or SB-OCSA) for bilateral pheochromocytomas (bPHEOs) in multiple endocrine neoplasia type 2 (MEN2). Altogether, 31 patients (54.8% were women) were diagnosed with MEN2-related bPHEOs, and 29 of them underwent varying specific adrenalectomies. We systematically analyzed and evaluated their clinical profiles, mutation types, tumor histopathological features, and follow-up records. All 31 patients with bPHEOs presented with RET-C634 (90.3%) and RET-M918T (9.7%) mutations, and the median age at initial presentation was 38 years (range, 23-78). bPHEOs were synchronous in 27 patients and metachronous in 4 (12.9%) patients. In total, 29 patients underwent initial cortical-sparing adrenalectomy (CSA) including 23 (79.3%) undergoing synchronous bilateral CSA (18 SB-LCSA and 5 SB-OCSA) and 6 (20.7%) undergoing metachronous CSA. SB-LCSA and synchronous surgery were associated with less bleeding volume and shorter length of hospital stay than SB-OCSA and metachronous surgery (all P's < 0.05). Corticosteroid replacement treatment was necessary for 14 patients (45.2%) after bilateral CSA. During a median follow-up period of 7 years (range, 1.8-23), three of these patients (10.3%) had a recurrent disease that required reoperation. SB-LCSA is feasible for treating synchronous bPHEOs and should be recommended as a prioritized surgical approach.

Hereditary Bilateral Synchronous Pheochromocytomas in a Tibetan Pedigree With a Germline Adenomatous Polyposis Coli (APC) Mutation

Background: Germline pathogenic mutations associated with pheochromocytoma (PHEO) are present in most patients with hereditary bilateral PHEOs. Adenomatous polyposis coli (APC) gene has previously been identified as a tumor suppressor gene in familial adenomatous polyposis and colorectal cancers, but not PHEO. Methods: A father and a son from Tibet were clinically diagnosed with bilateral synchronous PHEOs. The son underwent staged retroperitoneal laparoscopic bilateral total adrenalectomy, and the father underwent retroperitoneal laparoscopic left adrenalectomy. A pedigree investigation was performed with a follow-up of 4 years. Whole-exome sequencing was performed to reveal the susceptibility genes in the pedigree. Results: The adrenal masses in the father and son were pathologically diagnosed as hereditary bilateral synchronous PHEOs. A pedigree investigation of 26 family members spanning 3 generations was performed. Multiple endocrine gland–related tumors, including PHEO, pancreatic neuroendocrine tumor, and pituitary adenoma, were diagnosed in the family. DNA sequencing identified a novel heterozygous germline missense mutation (c.896C&gt;G) of the APC gene in the proband. The same heterozygous germline mutation of the APC gene was also present in the pedigree. Conclusions: We reported a pedigree from Tibet with hereditary bilateral synchronous PHEOs, which revealed a novel heterozygous germline missense mutation of the APC gene that may predispose to PHEO.

Cushing's disease: role of bilateral adrenalectomy.

Laparoscopic bilateral adrenalectomy (BAD) is one of the treatments of Cushing's Disease (CD), but its indications and outcome is debated. The literature on BAD was reviewed as part of a work performed for the Cushing's disease guideline. The surgical morbidity of BAD is reported between 10 and 18% and no mortality has been reported in the largest series. Because of the endocrine sacrifice it will be mostly performed after a multidisciplinary team discussion in selected cases of refractory CD (mostly after failure of pituitary surgery and/or medical treatment). It is also frequently discussed in female patients desiring pregnancy. Corticotroph tumor progression occurs in 40% of the patients but is in most patients manageable when detected early by a careful long term monitoring with pituitary MRI and ACTH assays after BAD. BAD is a safe and effective treatment of CD used in specific situations and requiring long term monitoring.

Synchronous bilateral laparoscopic adrenalectomy: Surgical technique and perioperative results of a 13-years’ experience

Introduction and objectivesLaparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes. Patients and methodsA total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay. ResultsMean operative time was 189.3 ± 48.9 min. Mean estimated blood loss was 163.0 ± 201.3 mL. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1−30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma. ConclusionSynchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required.

Adrenalectomía laparoscópica bilateral sincrónica: técnica quirúrgica y resultados perioperatorios en 13 años de experiencia

Introduction and objectivesLaparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes. Patients and methodsA total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay. ResultsMean operative time was 189.3±48.9min. Mean estimated blood loss was 163.0±201.3ml. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1-30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma. ConclusionSynchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required.

A novel nonsense mutation in ARMC5 causes primary bilateral macronodular adrenocortical hyperplasia

BackgroundPrimary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare form of adrenal Cushing’s syndrome. The slowly progressing expansion of bilateral adrenal tissues usually persists for dozens of years, leading to delayed onset with severe conditions due to chronic mild hypercortisolism. About 20–50% cases were found to be caused by inactivating mutation of armadillo repeat-containing protein 5 (ARMC5) gene.Case presentationA 51-year-old man was admitted for severe diabetes mellitus, resistant hypertension, centripedal obesity and edema. PBMAH was diagnosed after determination of adrenocorticotropic hormone and cortisol levels, dexamethasone suppression tests and abdominal contrast-enhanced CT scanning. The metabolic disorders of the patient remarkably improved after sequentially bilateral laparoscopic adrenalectomy combined with hormone replacement. Sanger sequencing showed germline nonsense mutation of ARMC5 c.967C>T (p.Gln323Ter). The second somatic missense mutation of ARMC5 was detected in one out of two resected nodules, reflecting the second-hit model of tumorigenesis. Routine genetic testing in his apparently healthy offspring showed one of two daughters and one son harbored the germline mutation.ConclusionsIn conclusion, our case report highlight the importance of genetic testing in the molecular diagnosis of PBMAH. Genetic screening in related family members will find out asymptomatic variant carriers to guide life-long follow-up.

Laparoscopic Bilateral Adrenalectomy Should Be an Early Consideration in Ectopic Adrenocorticopic Hormone Secretion

Introduction: Ectopic ACTH secretion (EAS) causes myriad metabolic derangements which may be effectively managed with laparoscopic bilateral adrenalectomy (LBA) in fit-for-surgery candidates. Clinical Case: A 67-year old woman with a remote 12-pack year smoking history presented with unintentional weight loss, cough, fever and chest pain. Imaging revealed a right-sided lung mass with liver lesions. Liver biopsy confirmed a well-differentiated neuroendocrine tumour (NET). Attempt at palliative lung resection was aborted due to disease extent. She was treated with multiple courses of chemotherapy but her disease progressed. Two years later, she presented with hypokalemia of 2.8 mmol/L (3.5 - 5.0 mmol/L) and uncontrolled hypertension. She was treated with spironolactone and oral potassium. She was notably hyperglycaemic with clinical features of cortisol excess. A random cortisol of 2742 nmol/L (<500 nmol/L), corresponding ACTH of 201 ng/L (5 - 50 ng/L) and lack of diurnal variation in cortisol day curve were in keeping with EAS. Metyrapone and spironolactone normalised blood pressure and biochemical derangement. With her initially high cortisol levels and its associated immunocompromised state, she developed pneumocystis jirovecii pneumonia (PCP), and was briefly admitted to the intensive care unit. In hospital, monitoring her response to EAS management necessitated multiple blood investigations, which proved burdensome for the patient. To effect definitive management of EAS and reduce invasive monitoring, she wished to have LBA and underwent an uneventful procedure. She was discharged on fludrocortisone and hydrocortisone along with lanreotide for primary disease management. Metyrapone and spironolactone were stopped. At initial outpatient follow up, she had normal blood pressure and electrolytes. By her report, her quality of life had improved tremendously, given the reduced need for multiple clinic visits and blood investigations. Unfortunately, her primary disease progressed with gradual deterioration of physical function. She died six months after LBA but, as per her desire, spent her final months with loved ones. Clinical Lesson: In patients with EAS who have unresectable primary disease but otherwise good performance status, LBA is a viable alternative to medical management and should be discussed with patients early after diagnosis.

V10-02 ROBOTIC BILATERAL PARTIAL ADRENALECTOMY FOR PHEOCHROMOCYTOMA

You have accessJournal of UrologyAdrenal Surgery & Kidney Cancer II (Advanced Kidney Cancer & Vascular Reconstruction) (V10)1 Apr 2020V10-02 ROBOTIC BILATERAL PARTIAL ADRENALECTOMY FOR PHEOCHROMOCYTOMA Jeremy Archer*, Jonathan Pavlinec, Shavano Stedman, Tejal Patel, Sara Falzarano, and Li-Ming Su Jeremy Archer*Jeremy Archer* More articles by this author , Jonathan PavlinecJonathan Pavlinec More articles by this author , Shavano StedmanShavano Stedman More articles by this author , Tejal PatelTejal Patel More articles by this author , Sara FalzaranoSara Falzarano More articles by this author , and Li-Ming SuLi-Ming Su More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000000935.02AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Patients with multiple endocrine neoplasia type 2 (MEN2) and von Hippel-Lindau disease (VHL) are risk of bilateral adrenal pheochromocytomas. Total adrenalectomy was historically the standard of care for patients with hereditary syndromes, but this leads to lifelong steroid supplementation. Adrenal-sparing surgery has been described in the treatment of adrenal incidentaloma, aldosteronoma, isolated metastasis, and the hereditary pheochromocytomas of MEN2 and VHL. Synchronous bilateral laparoscopic partial adrenalectomy with intraoperative ultrasonography has been described for pheochromocytomas as safe and technically feasible, but the majority of bilateral lesions have been treated by staged operations. We report our experience of synchronous bilateral robotic partial adrenalectomy for multifocal bilateral adrenal pheochromocytomas. METHODS: A 36-year-old female with marfanoid habitus and biopsy-proven medullary thyroid cancer was found to have bilateral adrenal masses on staging workup. Laboratory results showed elevated serum metanephrines, and she was started on alpha blockade with phenoxybenzamine. Imaging revealed a 3 cm right adrenal mass with a separate area of 1 cm irregularity and a 2 cm left adrenal lesion. Bilateral robotic partial adrenalectomies were successfully performed using a 4-arm technique with the daVinci Xi robotic platform (Intuitive, Sunnyvale CA) and a 12mm assistant trocar at the umbilicus. The patient was positioned in full-flank positioning for each side with docking from the same side of the room. The midline subxiphoid robotic 8mm and the 12mm assistant trocars were used for both portions of the operation. RESULTS: Enucleation of the tumors was performed with a total operative time of 330 minutes and estimated blood loss of 50mL. Pathology revealed three pheochromocytomas, and the patient was started post-operatively on a steroid taper. She was referred for genetic counseling given suspicion for MEN2B syndrome. CONCLUSIONS: Bilateral adrenal-sparing surgery for pheochromocytoma is safe and feasible and may be achieved using a robotic 4-arm technique with linear trocar configuration with repositioning. This approach in patients with genetic predisposition to synchronous adrenal lesions may preclude or delay the need for lifelong steroid supplementation. Source of Funding: N/A © 2020 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 203Issue Supplement 4April 2020Page: e931-e931 Advertisement Copyright & Permissions© 2020 by American Urological Association Education and Research, Inc.MetricsAuthor Information Jeremy Archer* More articles by this author Jonathan Pavlinec More articles by this author Shavano Stedman More articles by this author Tejal Patel More articles by this author Sara Falzarano More articles by this author Li-Ming Su More articles by this author Expand All Advertisement PDF downloadLoading ...

Laparoscopic partial adrenalectomy using near-infrared imaging: the initial experience

Introduction To present our initial clinical experience with laparoscopic partial adrenalectomy using indocyanine green dye with near-infrared fluorescence imaging. Material and methods A total of eight patients underwent transperitoneal laparoscopic partial adrenalectomy using indocyanine green dye with near-infrared fluorescence imaging in our clinic. After 5 mg intravenous indocyanine green dye administration, we resected the mass under the guidance of near-infrared fluorescence imaging and white light visualization in an effort to completely excise the mass while sparing uninvolved adrenal tissue. Results Seven patients underwent unilateral and one patient underwent bilateral laparoscopic partial adrenalectomy. The median tumor size was 43 mm. The surgery was successfully performed with negative margins in all patients. The tumors were hypofluorescent relative to normal adrenal tissue with indocyanine green dye with near-infrared fluorescence imaging in patients with Cushing’s syndrome, aldosteronoma, and adrenal cyst. However, pheochromocytoma and angiomyolipoma were noted to be isoflourorescent and hyperfluorescent relative to normal adrenal parenchyma, respectively. Conclusions Laparoscopic partial adrenalectomy using intraoperative indocyanine green dye with near-infrared fluorescence imaging seems to be safe and feasible. This technology may ultimately be helpful in resecting lesions with more precise surgical margins by identifying the vascular structure during laparoscopic partial adrenalectomy. Abbreviations: LTA: Laparoscopic total adrenalectomy; LPA:Laparoscopic partial adrenalectomy; ICG: Indocynanine green; NIRF: Near-infrared fluorescence; HPA: Hypothalamic-pituitary-adrenal

Pheochromocytoma arising from an ectopic adrenal tissue in multiple endocrine neoplasia type 2A.

SummaryA 21-year-old woman was referred to our hospital to treat bilateral pheochromocytomas (PCCs) after a diagnosis of multiple endocrine neoplasia type 2A (MEN2A). We performed bilateral laparoscopic adrenalectomy. One year after the operation, urinary fractionated metanephrines in 24-h urine increased. MRI showed a 30 mm tumor on the interaortocaval region and 123I-MIBG concentrated in this area. We excised the tumor and performed para-aortic lymphadenectomy. Histopathologic examination confirmed a PCC arising from ectopic adrenal tissue. Urinary fractionated metanephrines in 24-h urine declined to basal levels immediately after the operation. We detected no recurrence of paraganglioma or PCC for 5 years after the treatment.Learning points:Most ectopic adrenal tissue is associated with no symptoms and contains only the adrenal cortex.Adrenocortical tumors sometimes arise from ectopic adrenal tissues similarly to in the normal adrenal gland.PCC arising from ectopic adrenal tissue occurs infrequently.MEN2-related PCC is accompanied by adrenal medullary hyperplasia, which might be part of tumorigenesis.

Hypercortisolism and primary aldosteronism caused by bilateral adrenocortical adenomas: a case report

BackgroundCo-existing Cushing’s syndrome and primary aldosteronism caused by bilateral adrenocortical adenomas, secreting cortisol and aldosterone, respectively, have rarely been reported. Precise diagnosis and management of this disorder constitute a challenge to clinicians due to its atypical clinical manifestations and laboratory findings.Case presentationWe here report a Chinese male patient with co-existing Cushing’s syndrome and primary aldosteronism caused by bilateral adrenocortical adenomas, who complained of intermittent muscle weakness for over 3 years. Computed tomography scans revealed bilateral adrenal masses. Undetectable ACTH and unsuppressed cortisol levels by dexamethasone suggested ACTH-independent Cushing’s syndrome. Elevated aldosterone to renin ratio and unsuppressed plasma aldosterone concentration after saline infusion test suggested primary aldosteronism. Adrenal venous sampling adjusted by plasma epinephrine revealed hypersecretion of cortisol from the left adrenal mass and of aldosterone from the right one. A sequential bilateral laparoscopic adrenalectomy was performed. The cortisol level was normalized after partial left adrenalectomy and the aldosterone level was normalized after subsequent partial right adrenalectomy. Histopathological evaluation of the resected surgical specimens, including immunohistochemical staining for steroidogenic enzymes, revealed a left cortisol-producing adenoma and a right aldosterone-producing adenoma. The patient’s symptoms and laboratory findings resolved after sequential adrenalectomy without any pharmacological treatment.ConclusionsAdrenal venous sampling is essential in diagnosing bilateral functional adrenocortical adenomas prior to surgery. Proper interpretation of the laboratory findings is particularly important in these patients. Immunohistochemistry may be a valuable tool to identify aldosterone/cortisol-producing lesions and to validate the clinical diagnosis.

Cushing syndrome: uncovering Carney complex due to novel PRKAR1A mutation.

SummaryCarney complex (CNC) is a rare multiple neoplasia syndrome characterized by spotty pigmentation of the skin and mucosa in association with various non-endocrine and endocrine tumors, including primary pigmented nodular adrenocortical disease (PPNAD). A 20-year-old woman was referred for suspected Cushing syndrome. She had signs of cortisol excess as well as skin lentigines on physical examination. Biochemical investigation was suggestive of corticotropin (ACTH)-independent Cushing syndrome. Unenhanced computed tomography scan of the abdomen did not reveal an obvious adrenal mass. She subsequently underwent bilateral laparoscopic adrenalectomy, and histopathology was consistent with PPNAD. Genetic testing revealed a novel frameshift pathogenic variant c.488delC/p.Thr163MetfsX2 (ClinVar Variation ID: 424516) in the PRKAR1A gene, consistent with clinical suspicion for CNC. Evaluation for other clinical features of the complex was unrevealing. We present a case of PPNAD-associated Cushing syndrome leading to the diagnosis of CNC due to a novel PRKAR1A pathogenic variant.Learning points:PPNAD should be considered in the differential for ACTH-independent Cushing syndrome, especially when adrenal imaging appears normal.The diagnosis of PPNAD should prompt screening for CNC.CNC is a rare multiple neoplasia syndrome caused by inactivating pathogenic variants in the PRKAR1A gene.Timely diagnosis of CNC and careful surveillance can help prevent potentially fatal complications of the disease.

Laparoscopic bilateral anterior transperitoneal adrenalectomy: 24 years experience.

The aim of this study is to evaluate the feasibility, safety, advantages and surgical outcomes of laparoscopic bilateral adrenalectomy (LBA) by an anterior transperitoneal approach. From 1994 to 2018, 552 patients underwent laparoscopic adrenalectomy, unilateral in 531 and bilateral in 21 patients (9 females and 12 males). All patients who underwent LBA were approached via a transperitoneal anterior route and form our study population. Indications included: Cushing's disease (n = 11), pheochromocytoma (n = 6), Conn's disease (n = 3) and adrenal cysts (n = 1). Mean operative time was 195 ± 86.2min (range 55-360min). Conversion was necessary in one case for bleeding. Three patients underwent concurrent laparoscopic cholecystectomy with laparoscopic common bile duct exploration and ductal stone extraction in one. Three postoperative complications occurred in one patient each: subhepatic fluid collection, intestinal ileus and pleural effusion. Mean hospital stay was 6.1 ± 4.7 days (range 2-18 days). In our experience, transperitoneal anterior LBA was feasible and safe. Based on our results, we believe that this approach leads to prompt recognition of anatomical landmarks with early division of the main adrenal vein prior to any gland manipulation, with a low risk of bleeding and without the need to change patient position. Unlike the lateral approach, there is no need to mobilize the spleno-pancreatic complex on the left or the liver on the right. The ability to perform associated intraperitoneal procedures, if required, is an added benefit.