Validation of pathological grading systems for predicting metastatic potential in pheochromocytoma and paraganglioma
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PURPOSE: The Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) was proposed for predicting the metastatic potential of pheochromocytoma and paraganglioma to overcome the limitations of the Pheochromocytoma of the Adrenal Scaled Score (PASS). However, to date, no study validating the GAPP has been conducted, and previous studies did not include mutations in the succinate dehydrogenase type B (SDHB) gene in the score calculation. In this retrospective cohort study, we validated the prediction ability of GAPP and assessed whether it would be improved by inclusion of the loss of SDHB immunohistochemical staining. METHODS: We divided the tumors into non-metastatic and metastatic groups based on the presence of synchronous or metachronous metastases. The GAPP score and PASS at the initial operation were measured. Moreover, we combined some GAPP parameters with the immunohistochemical staining of SDHB to obtain a modified GAPP (M-GAPP) score. RESULTS: Metastasis occurred in 15/72 (20.8%) patients, with a mean follow-up of 43.5 months. Loss of SDHB staining was more frequent (P = 0.044) in the metastatic group. The GAPP score (P = 0.006), PASS (P = 0.003), and M-GAPP score (P<0.001) were all higher in the metastatic group. Twelve of 40 (30.0%) moderately or poorly differentiated tumors, as defined by the GAPP score, and 12/34 (35.3%) tumors with a PASS ≥4 were metastatic. Conversely, 10/19 (52.6%) tumors with an M-GAPP score ≥3 were metastatic. The area under the curve of the M-GAPP score (0.822) was significantly higher than that of the GAPP (0.728) (P = 0.012), but similar to that of the PASS (0.753) (P = 0.411). The GAPP (P = 0.032) and M-GAPP scores (P = 0.040), but not PASS (P = 0.200), negatively correlated with metastasis-free survival. CONCLUSION: The GAPP was validated, and M-GAPP, a combination of some GAPP parameters and loss of SDHB staining, might be useful for the prediction of the metastatic potential of pheochromocytoma and paraganglioma.
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GAPP scoring was independently validated by Koh et al. and is considered a useful risk stratification tool for the prediction of metastatic potential.
“GAPP scoring was independently validated by Koh et al. and is considered a useful risk stratification tool for the prediction of metastatic potential (122).”
From Adrenal incidentaloma (2020) · cites this paper as [122]
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References 19 · Cited by 114
- Authors
- Koh, J.-M., Ahn, S. H., Kim, H., Kim, B.-J., Sung, T.-Y., Kim, Y. H., Hong, S. J., Song, D. E., & Lee, S. H.
- Affiliations
- Asan Medical Center (KR)
- Inha University (KR)
- Gyeongsang National University (KR)
- Corresponding author
- Song, Dong Eun
- Lee, Seung Hun
- ORCID
- Venue
- PLoS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2017-11-08
- Volume
- 12
- Issue
- 11
- Pages
- e0187398
- ISSN
- 1932-6203 (ISSN-L)
- PMC
- PMC5678867
- OpenAlex
- W2767206373
- Cited by
- 114
- References
- 19
- Citations by year
- 2026: 13 · 2025: 14 · 2024: 18 · 2023: 15 · 2022: 12 · 2021: 11 · 2020: 9 · 2019: 14 · 2018: 8
- FWCI
- 8.68
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- PLoS ONE (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Figshare (submittedVersion, cc-by)
- Funders
- Asan Institute for Life Sciences, Asan Medical Center (2016-0840)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
- Health Sciences › Medicine › Neurology › Neuroblastoma Research and Treatments
- Keywords
- pheochromocytoma, paraganglioma, metastatic potential, tumor grade, PASS score, SDHB immunohistochemistry, SDHB mutation, metastasis prediction, metastasis-free survival, tumor differentiation, adrenal tumors, synchronous and metachronous metastases
- MeSH terms
- Adrenal Gland Neoplasms, Adult, Female, Humans, Immunohistochemistry, Male, Middle Aged, Neoplasm Metastasis, Paraganglioma, Pheochromocytoma, ROC Curve, Succinate Dehydrogenase, Reproducibility of Results, Survival Analysis
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