ArticleLangenbeck's archives of surgery2018
Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms.
Article in Langenbeck's archives of surgery, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 27 citations in OpenAlex.
- A 42-Year-Old Woman Presenting With Pheochromocytoma Multisystem Crisis Associated With Bilateral Pheochromocytomas Due to Multiple Endocrine Neoplasia Type 2A.The American journal of case reports · 2026Article
- Efficacy of iodine-131-metaiodobenzylguanidine therapy for multiple endocrine neoplasia type 2A-associated metastatic pheochromocytoma: case report and literature review.Endocrine journal · 2026Review
- Multi-omics insights into metastatic pheochromocytomas and paragangliomas: Mechanisms, signatures, and prediction models.Hormones (Athens, Greece) · 2026Review
- Multicenter evaluation of the PASS score as a negative predictive tool and the impact of inter-observer variability in pheochromocytoma and paraganglioma risk stratification.Journal of pathology and translational medicine · 2026Article
- The evolving roles of pathologists in paragangliomas and pheochromocytomas.Virchows Archiv : an international journal of pathology · 2026Review
- Diagnostic and prognostic utility of insulinoma-associated protein 1, insulin gene enhancer protein 1, and secretagogin in pheochromocytoma.Revista da Associacao Medica Brasileira (1992) · 2025Article
- The road ahead: a brief guide to navigating the 2022 WHO classification of endocrine and neuroendocrine tumours.Journal of clinical pathology · 2024Review
- Differences between Patients with Sporadic and Familial Pheochromocytoma-Is It Possible to Avoid Genetic Testing in Certain Patients?Biomedicines · 2024Article
- Abdominal nonfunctional paraganglioma in which succinate dehydrogenase subunit B (SDHB) immunostaining was performed: a case report.Journal of medical case reports · 2023Article
- Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.Endocrine pathology · 2022Review
- Challenges in Paragangliomas and Pheochromocytomas: from Histology to Molecular Immunohistochemistry.Endocrine pathology · 2021Review
- Article
- Recent Advances in Histopathological and Molecular Diagnosis in Pheochromocytoma and Paraganglioma: Challenges for Predicting Metastasis in Individual Patients.Frontiers in endocrinology · 2020Review
- Progress in the diagnosis and treatment of paraganglioma.Translational cancer research · 2019Review
- Pheochromocytomas and Paragangliomas: New Developments with Regard to Classification, Genetics, and Cell of Origin.Cancers · 2019Review
- COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas.Virchows Archiv : an international journal of pathology · 2019Article
- Review
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposePheochromocytomas (PCCs) exhibit malignant potential, but current histological modalities for the proper detection of aggressive behavior are debated. The two most widespread algorithms are the "Pheochromocytoma of the Adrenal Gland Scaled Score" (PASS) and the "Grading System for Adrenal Pheochromocytoma and Paraganglioma" (GAPP), both which mostly rely on histological parameters to identify PCC patients at risk of disseminated disease. Since the algorithms are derived from studies using predominantly sporadic PCCs, little is known whether the PASS or GAPP scores can predict malignant potential in hereditary cases.
methodsPASS and GAPP were applied on 41 PCCs; 13 PCCs were diagnosed in ten multiple endocrine neoplasia type 2A (MEN 2A) patients carrying established germline RET proto-oncogene mutations, as well as 28 assumed sporadic PCCs.
resultsSix out of thirteen MEN 2A tumors (46%) exhibited PASS scores ≥ 4, indicative of a potential for aggressive behavior. In addition, 7/13 tumors (54%) exhibited GAPP scores ≥ 3, indicative of a "moderately differentiated type" with risk of future recurrence. All MEN 2A PCCs with an elevated PASS score also displayed an elevated GAPP score. In contrast, 4/28 (14%) sporadic PCCs demonstrated PASS scores ≥ 4, and 9/28 (32%) displayed GAPP scores ≥ 3. Follow-up found all cases in the study are free of metastatic or recurrent disease.
conclusionsWe conclude that the PASS and GAPP scoring systems might be suboptimal for determining true malignant potential in PCCs with constitutional RET mutations and advocate restrictive use of these scores in MEN 2A cases until the results are reproduced in larger numbers.
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