Dataset Information

Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy.

ABSTRACT:

Importance

Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management.

Objective

To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence.

Design, setting, and participants

This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019.

Exposures

Total or cortical-sparing adrenalectomy.

Main outcomes and measures

Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality.

Results

Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutations were detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survival was associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma.

Conclusions and relevance

Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma.

SUBMITTER: Neumann HPH

PROVIDER: S-EPMC6692838 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Publications

Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy.

Neumann Hartmut P H HPH Tsoy Uliana U Bancos Irina I Amodru Vincent V Walz Martin K MK Tirosh Amit A Kaur Ravinder Jeet RJ McKenzie Travis T Qi Xiaoping X Bandgar Tushar T Petrov Roman R Yukina Marina Y MY Roslyakova Anna A van der Horst-Schrivers Anouk N A ANA Berends Annika M A AMA Hoff Ana O AO Castroneves Luciana Audi LA Ferrara Alfonso Massimiliano AM Rizzati Silvia S Mian Caterina C Dvorakova Sarka S Hasse-Lazar Kornelia K Kvachenyuk Andrey A Peczkowska Mariola M Loli Paola P Erenler Feyza F Krauss Tobias T Almeida Madson Q MQ Liu Longfei L Zhu Feizhou F Recasens Mònica M Wohllk Nelson N Corssmit Eleonora P M EPM Shafigullina Zulfiya Z Calissendorff Jan J Grozinsky-Glasberg Simona S Kunavisarut Tada T Schalin-Jäntti Camilla C Castinetti Frederic F Vlcek Petr P Beltsevich Dmitry D Egorov Viacheslav I VI Schiavi Francesca F Links Thera P TP Lechan Ronald M RM Bausch Birke B Young William F WF Eng Charis C

JAMA network open 20190802 8

<h4>Importance</h4>Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management.<h4>Objective</h4>To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence.<h4>Design, setting, and participan ...[more]

PMID: 31397861

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Project description:BackgroundIn patients with bilateral pheochromocytoma, partial adrenalectomy offers the chance to preserve adrenal function and avoid the need for lifelong steroid supplementation. However, the risk of tumour recurrence raises questions about this procedure. The aim of our study was to compare partial and total adrenalectomy in bilateral pheochromocytoma through a systematic review with meta-analysis.MethodsA systematic search was carried out using databases (MEDLINE, EMBASE, Scopus, Web of Science, CENTRAL) and registers of clinical trials (ClinicalTrials.gov, European Trials Register, WHO International Trials Registry Platform). This meta-analysis included studies up to July 2022 without language restrictions. A random effects model meta-analysis was performed to assess the risk of tumor recurrence, steroid dependence and morbidity in these patients.ResultsTwenty-five studies were included in the analysis involving 1444 patients. The relative risk (RR) of loss of adrenal hormone function during follow-up and the need for steroid therapy was 0.32 in patients after partial adrenalectomy: RR 0.32, 95% Confidence Interval (CI): 0.26-0.38, P < 0.00001, I2 = 21%. Patients undergoing partial adrenalectomy had a lower odds ratio (OR) for developing acute adrenal crisis: OR 0.3, 95% CI: 0.1-0.91, P=0.03, I2 = 0%. Partial adrenalectomy was associated with a higher risk of recurrence than total adrenalectomy: OR 3.72, 95% CI: 1.54-8.96, P=0.003, I2 = 28%.ConclusionPartial adrenalectomy for bilateral pheochromocytoma is a treatment that offers a chance of preserving adrenal hormonal function, but is associated with a higher risk of local tumor recurrence. There was no difference for the risk of metastasis and in overall mortality among the group with bilateral pheochromocytomas undergoing total or partial adrenalectomy. This study is in line with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and AMSTAR (A Measurement Tool to Assess Systematic Reviews) Guidelines (10, 11).Systematic review registrationhttps://osf.io/zx3se.

Project description:BackgroundSkin pigmentation after adrenalectomy occurs due to an increase in adrenocorticotropic hormone (ACTH) following adrenal insufficiency. ACTH-induced pigmentation usually appears as generalized hyperpigmentation and is known to appear after bilateral adrenalectomy. We report a case of unusual transient hyperpigmentation that developed immediately after unilateral adrenalectomy for pheochromocytoma and spontaneously resolved without corticosteroid supplementation.Case descriptionA 29-year-old woman was admitted to the hospital due to sudden-onset chest pain and headache. A 2.8-cm left adrenal mass with heterogeneous enhancement was incidentally found in chest computed tomography during the evaluation. Multiple old infarctions were observed in brain magnetic resonance imaging (MRI), and left ventricular thrombi were found by echocardiography. Biochemical evidence confirmed the diagnosis of pheochromocytoma, while serum ACTH and cortisol levels were within normal ranges. The patient underwent laparoscopic left adrenalectomy via a posterior retroperitoneal approach and recovered without immediate postoperative complications. On day 3 after surgery, a crescent-shaped café-au-lait skin pigmentation occurred on both the subcostal and the lumbar areas of the abdomen. Serial serum cortisol slightly decreased during the immediate postoperative period and recovered on day 3. Serum ACTH was elevated. Under close observation without corticosteroid supplementation, the pigmentation faded on day 8 after surgery. On day 15, the pigmentation clearly disappeared and serum ACTH decreased to within the normal range. A month later, ACTH and all adrenal hormones were within normal range.ConclusionsWe hypothesized that skin pigmentation appeared due to an imbalance of the hypothalamic-pituitary-adrenal (HPA) axis after resection of one adrenal gland. Skin pigmentation may be the first and early manifestation of adrenal insufficiency in patients who undergo unilateral adrenalectomy due to a non-Cushing's tumor. Therefore, a careful physical examination may allow early detection of adrenal insufficiency and optimal treatment planning.

Project description:BackgroundPartial adrenalectomy has recently been advocated to preserve unaffected adrenal tissue during resection of pheochromocytoma.ObjectiveTo describe a robot-assisted laparoscopic partial adrenalectomy (RALPA) technique and to report on early functional and oncologic outcomes.Design, setting, and participantsFrom 2007 to 2010, 15 RALPA were performed on 12 consecutive patients with pheochromocytoma. Follow-up data of >1 yr are available on 11 procedures. Median follow-up for the entire cohort was 17.3 mo (range: 6-45).Surgical procedurePositioning and port placement is designed for adequate reach and visualization of the upper retroperitoneum. The plane between the adrenal cortex and pheochromocytoma pseudocapsule is identified visually and with laparoscopic ultrasound. The tumor is dissected away from normal adrenal cortex, preserving normal adrenal tissue.MeasurementsPreoperative, perioperative, pathologic, and functional outcomes data were analyzed.Results and limitationsFourteen of 15 cases were completed robotically. Among 15 procedures, 4 were performed on a solitary adrenal gland. Four cases required resection of multiple tumors (up to six) with two performed in a solitary gland. The mean age of the patients was 30 yr, and the mean body mass index was 27. The mean operative time was 163 min, the median estimated blood loss was 161 ml, and the median tumor size was 2.7 cm (range: 1.3-5.5). There was one conversion to an open procedure in a patient requiring reoperation on a solitary adrenal gland. One patient who underwent RALPA on a solitary adrenal gland required postoperative steroid supplementation at last follow-up. At a median follow-up of 17.3 mo (range: 6-45), there were no recurrences or metastatic events. Study limitations include small sample size and short follow-up.ConclusionsRALPA for the treatment of pheochromocytoma is feasible and safe and provides encouraging functional and oncologic outcomes, even in patients with a solitary adrenal lesion or multiple ipsilateral lesions.

Dataset Information

Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy.

Importance

Objective

Design, setting, and participants

Exposures

Main outcomes and measures

Results

Conclusions and relevance

Publications

Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy.

Similar Datasets

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