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dc.contributor.authorLowery, Aoife J
dc.contributor.authorWalsh, Siun
dc.contributor.authorMcDermott, Enda W
dc.contributor.authorPrichard, Ruth S
dc.date.accessioned2014-08-06T10:46:28Z
dc.date.available2014-08-06T10:46:28Z
dc.date.issued2013
dc.identifier.citationLowery AJ et al. Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas. 2013, 18 (4):391-407 Oncologisten_GB
dc.identifier.issn1549-490X
dc.identifier.pmid23576482
dc.identifier.doi10.1634/theoncologist.2012-0410
dc.identifier.urihttp://hdl.handle.net/10147/324301
dc.description.abstractPheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies.
dc.language.isoenen
dc.rightsArchived with thanks to The oncologisten_GB
dc.subjectCANCERen_GB
dc.subjectTHERAPYen_GB
dc.subject.meshAdrenal Gland Neoplasms
dc.subject.meshAdrenalectomy
dc.subject.meshDiagnostic Imaging
dc.subject.meshHumans
dc.subject.meshMicroRNAs
dc.subject.meshMutation
dc.subject.meshNeoplasm Recurrence, Local
dc.subject.meshParaganglioma
dc.subject.meshPheochromocytoma
dc.subject.meshPrognosis
dc.subject.otherNEOPLASMS, ADRENAL GLANDen_GB
dc.titleMolecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas.en_GB
dc.typeArticleen
dc.contributor.departmentDepartment of Surgery, St. Vincent's University Hospital, Dublin, Ireland.en_GB
dc.identifier.journalThe oncologisten_GB
dc.description.fundingNo fundingen
dc.description.provinceLeinsteren
dc.description.peer-reviewpeer-reviewen
html.description.abstractPheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes that contribute to the development of these tumors, and increasing knowledge of genotype-phenotype interactions has facilitated more accurate determination of malignant potential. Elucidating the molecular mechanisms responsible for malignant transformation in these tumors has opened avenues of investigation into targeted therapeutics that show promising results. There have also been significant advances in functional and radiological imaging and in the surgical approach to adrenalectomy, which remains the mainstay of treatment for PCC. In this review, we discuss the currently available diagnostic and therapeutic options for patients with malignant PCCs and PGLs and detail the molecular rationale and clinical evidence for novel and emerging diagnostic and therapeutic strategies.


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