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Cancers (Basel). 2019 Feb 15;11(2). pii: E225. doi: 10.3390/cancers11020225.

The Value of Histological Algorithms to Predict the Malignancy Potential of Pheochromocytomas and Abdominal Paragangliomas-A Meta-Analysis and Systematic Review of the Literature.

Author information

1
Department of Oncology-Pathology, Karolinska Institutet, 171 76 Stockholm, Sweden. adam.stenman@ki.se.
2
Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, Sweden. adam.stenman@ki.se.
3
Department of Breast, Endocrine Tumours and Sarcoma, Karolinska University Hospital, 171 76 Stockholm, Sweden. adam.stenman@ki.se.
4
Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76 Stockholm, Sweden. jan.zedenius@ki.se.
5
Department of Breast, Endocrine Tumours and Sarcoma, Karolinska University Hospital, 171 76 Stockholm, Sweden. jan.zedenius@ki.se.
6
Department of Oncology-Pathology, Karolinska Institutet, 171 76 Stockholm, Sweden. christofer.juhlin@ki.se.
7
Department of Pathology and Cytology, Karolinska University Hospital, 171 76 Stockholm, Sweden. christofer.juhlin@ki.se.

Abstract

Pheochromocytomas (PCCs) and abdominal paragangliomas (PGLs), collectively abbreviated PPGLs, are neuroendocrine tumors of the adrenal medulla and paraganglia, respectively. These tumors exhibit malignant potential but seldom display evidence of metastatic spread, the latter being the only widely accepted evidence of malignancy. To counter this, pre-defined histological algorithms have been suggested to stratify the risk of malignancy: Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP). The PASS algorithm was originally intended for PCCs whereas the GAPP model is proposed for stratification of both PCCs and PGLs. In parallel, advances in terms of coupling overtly malignant PPGLs to the underlying molecular genetics have been made, but there is yet no combined risk stratification model based on histology and the overall mutational profile of the tumor. In this review, we systematically meta-analyzed previously reported cohorts using the PASS and GAPP algorithms and acknowledge a "rule-out" way of approaching these stratification models rather than a classical "rule-in" strategy. Moreover, the current genetic panorama regarding possible molecular adjunct markers for PPGL malignancy is reviewed. A combined histological and genetic approach will be needed to fully elucidate the malignant potential of these tumors.

KEYWORDS:

GAPP; PASS; histology; meta-analysis; paraganglioma; pheochromocytoma

PMID:
30769931
DOI:
10.3390/cancers11020225
Free PMC Article

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