Pheochromocytoma and Paraganglioma

Pacák, Karel; Wimalawansa, Sunil J.

doi:10.4158/ep14481.ra

Cited by 57 publications

(41 citation statements)

References 206 publications

(280 reference statements)

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“…Interestingly, most of the susceptibility genes for HNPGL are related to the Krebs cycle [1]. Collectively the most prevalent are SDHx genes [12].…”

Section: Succinate Dehydrogenase (Sdhx) Genesmentioning

confidence: 99%

“…These characteristics enable normal cells to survive and grow in low-oxygen environments [1]. A second susceptibility gene cluster with a greater predilection for pheochromocytomas and sympathoadrenal paragangliomas involves RET, RAS/MAPK/PI3K /mTOR pathways [3].…”

Section: Geneticsmentioning

confidence: 99%

“…Since that time, five syndromes with susceptibility genes have been defined and their associations with PGL in the head and neck region are being elucidated (Table 1) [1]. Other susceptibility genes for PGL and pheochromocytoma were also known including VHL, RET (MEN2), and NF1, though these mutations have a relatively low association 1 3 with PGL development in the head and neck (HN) region [1] and are identified in both sporadic and hereditary PGLs [3,4]. Through both germline and somatic tumor testing of individuals with PGL, the prevalence of germline susceptibility genes altered in HNPGL is now ~30 to 40%, involving predominantly genes encoding subunits of succinate dehydrogenase ("SDHx" genes) [3,5].…”

Section: Geneticsmentioning

confidence: 99%

“…During the past decade, germline mutations in more than 19 hereditary susceptibility genes resulting in PGL development have been identified [1]. More than 10 genes are associated with head and neck paragangliomas [2].…”

Section: Introductionmentioning

confidence: 99%

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Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Paragangliomas

Williams

Tischler

2017

Head and Neck Pathol

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“…Interestingly, most of the susceptibility genes for HNPGL are related to the Krebs cycle [1]. Collectively the most prevalent are SDHx genes [12].…”

Section: Succinate Dehydrogenase (Sdhx) Genesmentioning

confidence: 99%

Section: Geneticsmentioning

confidence: 99%

Section: Geneticsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Paragangliomas

Williams

Tischler

2017

Head and Neck Pathol

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“…Approximately 40% of PHEOs and PGLs carry a germline mutation in one of at least 20 genes (Martucci & Pacak 2014, Pacak & Wimalawansa 2015. These mutations are associated with transcriptome changes that are currently subdivided into two main clusters.…”

Section: Influence Of Genotype On Imaging Phenotypementioning

confidence: 99%

15 YEARS OF PARAGANGLIOMA: Imaging and imaging-based treatment of pheochromocytoma and paraganglioma

Castinetti¹,

Kroiss²,

Kumar³

et al. 2015

Endocrine-Related Cancer

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Although anatomic imaging to assess the precise localization of pheochromocytomas/ paragangliomas (PHEOs/PGLs) is unavoidable before any surgical intervention on these tumors, functional imaging is becoming an inseparable portion of the imaging algorithm for these tumors. This review article presents applications of the most up-to-date functional imaging modalities and image-based treatment to PHEOs/PGLs patients. Functional imaging techniques provide whole-body localization (number of tumors present along with metastatic deposits) together with genetic-specific imaging approaches to PHEOs/PGLs, thus enabling highly specific and sensitive PHEO/PGL detection and delineation that now greatly impact the management of patients. Radionuclide imaging techniques also play a crucial role in the prediction of possible radioactive treatment options for PHEO/PGL. In contrast to previous imaging algorithms used for either assessement of these patients or their follow-up, endocrinologists, surgeons, oncologists, pediatricians, and other specialists require functional imaging before any therapeutic plan is outlined to the patient, and follow-up, especially in patients with metastatic disease, is based on the periodic use of functional imaging, often reducing or substituting for anatomical imaging. In similar specific indications, this will be further powered by using PET/MR in the assessment of these tumors. In the near future, it is expected that PHEO/PGL patients will benefit even more from an assessement of the functional characteristics of these tumors and new imaging-based treatment options. Finally, due to the use of new targeting moieties, gene-targeted radiotherapeutics and nanobodiesbased theranostic approaches are expected to become a reality in the near future.

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Adrenal Neoplasms

Stewart

Story

2017

Textbook of Uncommon Cancer

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Pheochromocytoma and Paraganglioma

Cited by 57 publications

References 206 publications

Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Paragangliomas

Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Paragangliomas

15 YEARS OF PARAGANGLIOMA: Imaging and imaging-based treatment of pheochromocytoma and paraganglioma

Adrenal Neoplasms

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