Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant PheochromocytomaParaganglioma
Joint Authors
Ercolino, Tonino
Parenti, Gabriele
Mannelli, Massimo
Giachè, Valentino
Rapizzi, Elena
Zampetti, Benedetta
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-07-17
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract EN
Malignant pheochromocytomas/paragangliomas are rare tumors with a poor prognosis.
Malignancy is diagnosed by the development of metastases as evidenced by recurrences in sites normally devoid of chromaffin tissue.
Histopathological, biochemical, molecular and genetic markers offer only information on potential risk of metastatic spread.
Large size, extraadrenal location, dopamine secretion, SDHB mutations, a PASS score higher than 6, a high Ki-67 index are indexes for potential malignancy.
Metastases can be present at first diagnosis or occur years after primary surgery.
Measurement of plasma and/or urinary metanephrine, normetanephrine and metoxytyramine are recommended for biochemical diagnosis.
Anatomical and functional imaging using different radionuclides are necessary for localization of tumor and metastases.
Metastatic pheochromocytomas/paragangliomas is incurable.
When possible, surgical debulking of primary tumor is recommended as well as surgical or radiosurgical removal of metastases.
I-131-MIBG radiotherapy is the treatment of choice although results are limited.
Chemotherapy is reserved to more advanced disease stages.
Recent genetic studies have highlighted the main pathways involved in pheochromocytomas/paragangliomas pathogenesis thus suggesting the use of targeted therapy which, nevertheless, has still to be validated.
Large cooperative studies on tissue specimens and clinical trials in large cohorts of patients are necessary to achieve better therapeutic tools and improve patient prognosis.
American Psychological Association (APA)
Parenti, Gabriele& Zampetti, Benedetta& Rapizzi, Elena& Ercolino, Tonino& Giachè, Valentino& Mannelli, Massimo. 2012. Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant PheochromocytomaParaganglioma. Journal of Oncology،Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-505140
Modern Language Association (MLA)
Parenti, Gabriele…[et al.]. Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant PheochromocytomaParaganglioma. Journal of Oncology No. 2012 (2012), pp.1-10.
https://search.emarefa.net/detail/BIM-505140
American Medical Association (AMA)
Parenti, Gabriele& Zampetti, Benedetta& Rapizzi, Elena& Ercolino, Tonino& Giachè, Valentino& Mannelli, Massimo. Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant PheochromocytomaParaganglioma. Journal of Oncology. 2012. Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-505140
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-505140