Lesion-based indicators predict long-term outcomes of pheochromocytoma and paraganglioma– SIZEPASS

Frontiers in Endocrinology

Endocrinology

Gabriele Galatá¹  Nadia Talat¹  Helena Hanschell¹  Klaus Martin Schulte²  Simon Aylwin³  Alfredo Blanes⁴  Salvador Diaz-Cano⁵ 

[1] Department of Endocrine Surgery, Division of Surgery, King’s College Hospital Foundation Trust, London, United Kingdom;Department of Endocrine Surgery, Division of Surgery, King’s College Hospital Foundation Trust, London, United Kingdom;Department of Surgery, School of Medicine and Psychology, College of Health and Medicine, Australian National University, Canberra, ACT, Australia;Department of Endocrinology, Division of Medicine, King’s College Hospital Foundation Trust, London, United Kingdom;Department of Pathology, University Hospital of Malaga, Malaga, Spain;Reader in Cellular and Molecular Pathology (Division of Cancer Studies), King’s Health Partners, London, United Kingdom;

关键词: adrenal;    extra-adrenal;    paraganglioma;    pheochromocytoma;    PPGL;    adrenal medulla;    prediction PASS score;    biomarker;   

DOI  :  10.3389/fendo.2023.1235243

 received in 2023-06-06, accepted in 2023-07-12,  发布年份 2023

来源: Frontiers

【 摘 要 】

AimWe seek a simple and reliable tool to predict malignant behavior of pheochromocytoma and paraganglioma (PPGL).MethodsThis single-center prospective cohort study assessed size of primary PPGLs on preoperative cross-sectional imaging and prospectively scored specimens using the Pheochromocytoma of the Adrenal Gland Scaled Score (PASS). Multiplication of PASS points with maximum lesion diameter (in mm) yielded the SIZEPASS criterion. Local recurrence, metastasis or death from disease were surrogates defining malignancy.Results76 consecutive PPGL patients, whereof 58 with pheochromocytoma and 51 female, were diagnosed at a mean age of 52.0 ± 15.2 years. 11 lesions (14.5%) exhibited malignant features at a median follow-up (FU) of 49 months (range 4-172 mo). Median FU of the remaining cohort was 139 months (range 120-226 mo). SIZEPASS classified malignancy with an area under the curve (AUC) of 0.97 (95%CI 0.93-1.01; p<0.0001). Across PPGL, SIZEPASS >1000 outperformed all known predictors of malignancy, with sensitivity 91%, specificity 94%, and accuracy 93%, and an odds ratio of 72 fold (95%CI 9-571; P<0.001). It retained an accuracy >90% in cohorts defined by location (adrenal, extra-adrenal) or mutation status.ConclusionsThe SIZEPASS>1000 criterion is a lesion-based, clinically available, simple and effective tool to predict malignant behavior of PPGLs independently of age, sex, location or mutation status.

【 授权许可】

Unknown   
Copyright © 2023 Hanschell, Diaz-Cano, Blanes, Talat, Galatá, Aylwin and Schulte