Metastatic Paraganglioma

Overview

Journal Semin Oncol

Specialty Oncology

Date 2010 Dec 21

PMID 21167381

Citations 48

Authors

Stephanie M J Fliedner

Hendrik Lehnert

Karel Pacak

Affiliations

Soon will be listed here.

Abstract

Paragangliomas (PGLs) are chromaffin cell tumors arising from ganglia; when arising in the adrenal gland they are called pheochromocytomas. In recent years the opinion that metastatic disease is rare in PGL had to be revised, particularly in patients presenting with extra-adrenal PGL, with PGLs exceeding 5 cm in diameter, and/or those carrying an SDHB germline mutation. Metastases are expected to be present at the time of diagnosis in more than 10% of these patients. Measurement of plasma and urinary metanephrine levels is well established in diagnosing PGL. Recently, a dopaminergic phenotype (excess dopamine or methoxytyramine) was recognized as a good indicator of metastatic disease. Vast progress in targeted positron emission tomography (PET) imaging (eg, (18)F-FDA, (18)F-FDOPA, (18)F-FDG) now allows for reliable early detection of metastatic disease. However, once metastases are present, treatment options are limited. Survival of patients with metastatic PGL is variable, and frequently short. Here we review recent advances involving findings about the genetic background, the molecular pathogenesis, new diagnostic indicators, pathologic markers, and emerging treatment options for metastatic PGL.

Citing Articles

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PMID: 40033739 PMC: 11876828. DOI: 10.5758/vsi.240099.

Genetic and Molecular Biomarkers in Aggressive Pheochromocytomas and Paragangliomas.

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Management of Abdominal Paraganglioma: A Single Center's Experience.

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Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas.

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Fluorescence-guided minimally-invasive resection of abdominal paragangliomas using indocyanine green.

van Dam M, Crobach A, Boekestijn B, Corssmit E, Bonsing B, Vahrmeijer A Sci Rep. 2024; 14(1):3983.

PMID: 38368490 PMC: 10874414. DOI: 10.1038/s41598-024-54718-1.

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