Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems

Abstract Purpose The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP) are scoring systems to predict metastatic potential in pheochromocytomas (PCC) and paragangliomas (PGLs). The goal of this study is to assess PA...

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Published in:The journal of clinical endocrinology and metabolism 2020-12, Vol.105 (12), p.1-e4670
Main Authors: Wachtel, Heather, Hutchens, Troy, Baraban, Ezra, Schwartz, Lauren E, Montone, Kathleen, Baloch, Zubair, LiVolsi, Virginia, Krumeich, Lauren, Fraker, Douglas L, Nathanson, Katherine L, Cohen, Debbie L, Fishbein, Lauren
Format: Article
Language:English
Subjects:
Adrenal Gland Neoplasms - diagnosis
Adrenal Gland Neoplasms - mortality
Adrenal Gland Neoplasms - pathology
Adrenal glands
Adult
Biomarkers, Tumor - analysis
Clinical s
Cohort Studies
Diagnosis
Female
Follow-Up Studies
Humans
Male
Medical examination
Metastases
Metastasis
Middle Aged
Neoplasm Grading
Neoplasm Metastasis
Neuroendocrine tumors
Paraganglioma
Paraganglioma - diagnosis
Paraganglioma - mortality
Paraganglioma - pathology
Pennsylvania - epidemiology
Pheochromocytoma
Pheochromocytoma - diagnosis
Pheochromocytoma - mortality
Pheochromocytoma - pathology
Physiological aspects
Prognosis
Research Design - standards
Retrospective Studies
Survival
Survival Analysis
Tumor markers
Tumors
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Summary:Abstract Purpose The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP) are scoring systems to predict metastatic potential in pheochromocytomas (PCC) and paragangliomas (PGLs). The goal of this study is to assess PASS and GAPP as metastatic predictors and to correlate with survival outcomes. Methods The cohort included PCC/PGL with ≥5 years of follow-up or known metastases. Surgical pathology slides were rereviewed. PASS and GAPP scores were assigned. Univariable and multivariable logistic regression, Kaplan–Meier survival analysis, and Cox proportional hazards were performed to assess recurrence-free survival (RFS) and disease-specific survival (DSS). Results From 143 subjects, 106 tumors were PCC and 37 were PGL. Metastases developed in 24%. The median PASS score was 6.5 (interquartile range [IQR]: 4.0-8.0) and median GAPP score was 3.0 (IQR: 2.0-4.0). Interrater reliability was low–moderate for PASS (intraclass correlation coefficient [ICC]: 0.6082) and good for GAPP (ICC 0.7921). Older age (OR: 0.969, P = .0170) was associated with longer RFS. SDHB germline pathogenic variant (OR: 8.205, P = .0049), extra-adrenal tumor (OR: 6.357, P 
ISSN:0021-972X
1945-7197
DOI:10.1210/clinem/dgaa608