Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma
Open accessAbstract
Phaeochromocytomas (PHEO) and paragangliomas are rare catecholamine-producing tumours. Although 10-30% of these tumours metastasise, histopathological criteria to discriminate malignant from benign tumours have not been established; therefore, reliable histopathological markers predicting metastasis are urgently required. A total of 163 tumours, including 40 metastatic tumours, collected by the Phaeochromocytoma Study Group in Japan (PHEO-J) were analysed using a system called grading system for adrenal phaeochromocytoma and paraganglioma (GAPP). The tumours were scored based on GAPP criteria as follows: histological pattern, cellularity, comedo-type necrosis, capsular/vascular invasion, Ki67 labelling index and catecholamine type. All tumours were scored from 0 to 10 points and were graded as one of the three types: well-differentiated (WD, 0-2 points), moderately differentiated (MD, 3-6 points) and poorly differentiated (PD, 7-10 points). GAPP scores of the non-metastatic and metastatic groups were 2.08±0.17 and 5.33±0.43 (mean±s.e.m., P<0.001) respectively. There was a significant negative correlation between the GAPP score and the interval until metastasis (r=-0.438, P<0.01). The mean number of years until metastasis after the initial operation was 5.5±2.6 years. The study included 111 WD, 35 MD and 17 PD types. The five-year survival of these groups was 100, 66.8 and 22.4% respectively. In addition, negative immunoreactivity for succinate dehydrogenase gene subunit B (SDHB) was observed in 13 (8%) MD or PD tumours and ten of the 13 (77%) had metastases. Our data indicate that a combination of GAPP classification and SDHB immunohistochemistry might be useful for the prediction of metastasis in these tumours.
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A GAPP score of 0 to 2 is considered low risk, 3 to 6 intermediate risk, and 7 to 10 high risk.
“A GAPP score of 0 to 2 is considered low risk, 3 to 6 intermediate risk, and 7 to 10 high risk.”
From Adrenal incidentaloma (2020) · cites this paper as [121]
Kimura et al. developed the GAPP scoring system for prediction of metastases, consisting of a smaller number of pathological parameters with weighted scoring.
“Subsequently, Kimura et al. (121) developed a less involved scoring system for prediction of metastases, Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) consisting of a smaller number of pathological parameters with weighted scoring (1 or 2 points) including histological pattern; cellularity; comedo necrosis; capsular/vascular invasion; Ki67 proliferative index; and catecholamine phe”
From Adrenal incidentaloma (2020) · cites this paper as [121]
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- Authors
- Kimura, N., Takayanagi, R., Takizawa, N., Itagaki, E., Katabami, T., Kakoi, N., Rakugi, H., Ikeda, Y., Tanabe, A., Nigawara, T., Ito, S., Kimura, I., Naruse, M., & _, _.
- Affiliations
- National Hospital Organization (JP)
- Kyushu University (JP)
- Kansai Medical University (JP)
- Kyorin University (JP)
- St. Marianna University School of Medicine (JP)
- Miyagi Prefectural Hospital Organization (JP)
- The University of Osaka (JP)
- Nihon University Itabashi Hospital (JP)
- Tokyo Women's Medical University (JP)
- Hirosaki University (JP)
- Tohoku University (JP)
- Ministry of Health Labour and Welfare (JP)
- Kyoto Medical Center (JP)
- ORCID
- Venue
- Endocrine Related Cancer
- Publisher
- Bioscientifica
- Published
- 2014-02-12
- Volume
- 21
- Issue
- 3
- Pages
- 405-414
- ISSN
- 1351-0088 (ISSN-L)
- 1479-6821
- OpenAlex
- W2153367841
- Cited by
- 393
- References
- 25
- Citations by year
- 2026: 29 · 2025: 45 · 2024: 42 · 2023: 41 · 2022: 51 · 2021: 39 · 2020: 24 · 2019: 27 · 2018: 27 · 2017: 27 · 2016: 21 · 2015: 10 · 2014: 6
- FWCI
- 21.14
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Endocrine Related Cancer (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Shimane University
- Tokyo Medical and Dental University
- Jikei University School of Medicine
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
- Keywords
- pheochromocytoma, paraganglioma, metastasis prediction, tumor grade, SDHB immunohistochemistry, Ki-67 labelling index, comedo necrosis, capsular invasion, survival
- MeSH terms
- Adrenal Gland Neoplasms, Adult, Aged, Aged, 80 and over, Catecholamines, Female, Follow-Up Studies, Humans, Immunoenzyme Techniques, Male, Middle Aged, Neoplasm Metastasis, Paraganglioma, Pheochromocytoma, Prognosis, Succinate Dehydrogenase, Biomarkers, Tumor, Survival Rate, Case-Control Studies, Young Adult, Neoplasm Grading
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