Diagnosis and Treatment of Pheochromocytoma and Paraganglioma
A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Oncology".
Deadline for manuscript submissions: closed (31 December 2020) | Viewed by 313
Special Issue Editor
Interests: pheochromocytoma; paraganglioma; signaling pathways; genetics; metabolomics; targeted therapies
Special Issues, Collections and Topics in MDPI journals
Special Issue Information
Dear Colleagues,
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors arising respectively from chromaffin cells of the adrenal medulla and extra-adrenal paraganglia. PPGLs are characterized by catecholamine secretion, typically noradrenalin and adrenalin, which are responsible for alterations in basically all organ systems. Moreover, they also bear what has so far been an unpredictable potential to metastasize, even years after the primary tumor was found and removed. These tumors, if not diagnosed and treated, are associated with high morbidity and mortality mainly due to cardiovascular complications. Thus, despite the relative uncommonness of PPGLs, they should be taken into consideration, diagnosed, and treated without any delay.
The recent years have witnessed significant progress in understanding of the genetics, pathogenesis, and metabolomics of PPGLs which, in turn, allows for the development of novel diagnostic and therapeutic methods. Although complete surgical resection represents a curative treatment option for these tumors, the available therapeutic options for metastatic disease and non-resectable tumors are still not able to eradicate the disease. Therefore, the treatment of metastatic PPGL represents a major health concern. Current research shows that as many as 69% of PPGLs harbor germline and/or somatic genetic alterations leading to changes in cellular signaling and metabolism. New genes associated with disease development have been identified, and specific transcriptional profiling allows for clustering of these genes into four subtypes (hypoxia, kinase signaling, Wnt signaling, and cortical admixture). Accurate localization of the tumors has markedly improved with the use of new compounds for positron emission tomography, such as 68Ga-DOTATATE. Novel research approaches and technologies are being used to advance knowledge regarding the molecular basis of this metastatic disease as well as to promote the development of potentially effective therapies.
The journal Medicina is launching this Special Issue in recognition of the importance of PPGL in the field of medicine and research.
We encourage you and your co-workers to submit your articles reporting on PPGL. Reviews or original articles dealing with the genetic, biochemical, and molecular aspects of PPGL, pathogenesis in experimental models and humans, as well as articles providing an up-to-date overview of the use of circulating/tissue biomarkers in the early diagnosis and management of PPGL and metastatic disease are particularly welcome. In addition, we warmly invite you to submit articles reporting on evidence and expectations from innovative and personalized treatments.
Dr. Ivana Jochmanová
Guest Editor
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Keywords
- pheochromocytoma and paraganglioma
- catecholamines
- genetics
- epigenetics
- metabolomics
- biomarkers
- signaling pathways
- experimental models
- targeted therapies
- precision medicine
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