The Effects of Sampling Lateralization on Bilateral Inferior Petrosal Sinus Sampling for Pediatric Cushing's Disease-A Single Endocrinology Centre Experience and Review of the Literature.

Elzbieta Moszczyńska,Marcin Roszkowski,Elzbieta Marczak,Patrycja Zagata-Lesnicka,Krzysztof Kądziołka,Mieczysław Szalecki

doi:10.3389/fendo.2021.650967

Elzbieta Moszczyńska, Marcin Roszkowski + Show 4 more

Open Access

https://doi.org/10.3389/fendo.2021.650967

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Abstract

BackgroundThis study aims to analyze the diagnostic accuracy of bilateral inferior petrosal sinus sampling (BIPSS), the gold standard test for the differential diagnosis of ACTH-dependent Cushing’s syndrome (CS) in a group of pediatric patients with Cushing’s disease (CD).MethodsThis is a retrospective analysis which include 12 patients with hypercortisolemia and inconclusive pituitary MRI, who underwent bilateral inferior petrosal sinus sampling (BIPSS) and transsphenoidal surgery (TSS) from 2004 to 2020 in the Children’s Memorial Health Institute (CMHI) Warsaw, Poland. Pituitary origin of ACTH secretion was considered if baseline central to peripheral (C/P) ACTH level ratio was ≥ 2 or C/P ratio was ≥ 3 after human corticotropin-releasing hormone (hCRH) stimulation. The diagnosis was histologically confirmed in almost all cases after TSS.ResultsThe diagnostic accuracy of BIPSS reached 75% at baseline and 83.3% after CRH stimulation. The compatibility of localization of a microadenoma by BIPSS with the surgical location was 66.7%.ConclusionsOwing to its high diagnostic effectiveness, BIPSS remains the best test to differentiate CD from EAS. The indications for the procedure should be carefully considered, because EAS in the pediatric population, unlike in adults, is extremely rare. Moreover BIPSS has only limited value for indicating tumor localization.

Highlights

Cushing’s disease (CD) is caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma and is the most common cause of Cushing’s syndrome in children over 7 years of age [1,2,3]
We describe a series of 12 children treated in one specialist center, all of whom underwent the bilateral inferior petrosal sinus sampling (BIPSS) procedure
We evaluated 12 children who were admitted to the Children’s Memorial Health Institute (CMHI) for clinical evaluation and underwent BIPSS due to the presence of Cushing’s syndrome

Summary

Introduction

Cushing’s disease (CD) is caused by an ACTH-secreting pituitary adenoma and is the most common cause of Cushing’s syndrome in children over 7 years of age [1,2,3]. Corticotropin-releasing hormone (CRH) stimulation, and high dose dexamethasone suppression test (HDDST) can be used as second line test to reinforce the diagnosis of CD. Bilateral inferior petrosal sinus sampling (BIPSS) with CRH stimulation, is the most reliable method since it offers the highest approachable sensitivity and specificity in confirming pituitary ACTH production exceeding 95% [4, 5]. This study aims to analyze the diagnostic accuracy of bilateral inferior petrosal sinus sampling (BIPSS), the gold standard test for the differential diagnosis of ACTH-dependent Cushing’s syndrome (CS) in a group of pediatric patients with Cushing’s disease (CD)

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