Total versus partial adrenalectomy in bilateral pheochromocytoma – a systematic review and meta-analysis

Abstract

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.

Diverse presentations of Cushing's syndrome during pregnancy – A case series

“…The report concluded that the sample was moderately differentiated with background nodular hyperplasia of the adrenal cortex. In the context of ACTH‐secreting phaeochromocytomas (which are rare), 18,26,27 current evidence supports surgical resection of the involved and contralateral adrenal glands if there is bilateral disease 18,26–28 …”

“…In the context of ACTH-secreting phaeochromocytomas (which are rare), 18,26,27 current evidence supports surgical resection of the involved and contralateral adrenal glands if there is bilateral disease. 18,[26][27][28] Pregnancy in patients with CS poses a diagnostic and therapeutic challenge. Due to the limited literature, management is often individualised and dependent upon maternal and fetal factors.…”

Personalisierte Therapie des Phäochromozytoms

“…Eine Metaanalyse aus 25 Studien mit insgesamt 1444 Patientinnen und Patienten kommt zu einem ähnlichen Ergebnis: Nach Erhalt der Nebennierenrinde im Rahmen einer bilateralen Entfernung von Phäochromozytomen besteht zwar prinzipiell ein erhöhtes Rezidivrisiko, jedoch ist das Risiko einer Metastasierung und die Gesamtsterblichkeit im Vergleich zur vollständigen bilateralen Adrenalektomie vergleichbar. Dagegen überwiegt der Vorteil eines Funktionserhaltes bei Belassen der Nebennierenrinde deutlich [ 39 ]. Eine aktuelle internationale Leitlinie zu Patientinnen und Patienten mit SDH-B -Mutation rät in dieser Patientengruppe allerdings dringend von einer organerhaltenden Operation ab, da hier das Malignitätsrisiko mit teilweise über 50 % doch beträchtlich ist.…”

Bilateral pheochromocytoma in patients with von Hippel–Lindau syndrome: clinical and molecular genetic features

“…В ходе анализа было отмечено, что риск развития рецидива основного заболевания после частичной адреналэктомии был значительно выше, чем после тотальной адреналэктомии (OР 3,72, 95% ДИ 1,54-8,96, р = 0,003). Тем не менее статистически значимой разницы в риске развития метастазов и в показателях общей выживаемости в группе пациентов с двусторонними ФХ, перенесших тотальную или частичную адреналэктомию, не выявлено [21].…”