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Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome
by
Rivera, Michael
, Gupta, Sounak
, Erickson, Lori A.
, Zhang, Jun
in
Adult
/ Aged
/ Biomarkers, Tumor - analysis
/ Endocrinology
/ Female
/ Humans
/ Immunohistochemistry
/ Male
/ Medicine
/ Medicine & Public Health
/ Middle Aged
/ Oncology
/ Paraganglioma, Extra-Adrenal - enzymology
/ Paraganglioma, Extra-Adrenal - pathology
/ Pathology
/ Succinate Dehydrogenase - analysis
/ Succinate Dehydrogenase - biosynthesis
/ Urinary Bladder Neoplasms - enzymology
/ Urinary Bladder Neoplasms - pathology
2016
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Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome
by
Rivera, Michael
, Gupta, Sounak
, Erickson, Lori A.
, Zhang, Jun
in
Adult
/ Aged
/ Biomarkers, Tumor - analysis
/ Endocrinology
/ Female
/ Humans
/ Immunohistochemistry
/ Male
/ Medicine
/ Medicine & Public Health
/ Middle Aged
/ Oncology
/ Paraganglioma, Extra-Adrenal - enzymology
/ Paraganglioma, Extra-Adrenal - pathology
/ Pathology
/ Succinate Dehydrogenase - analysis
/ Succinate Dehydrogenase - biosynthesis
/ Urinary Bladder Neoplasms - enzymology
/ Urinary Bladder Neoplasms - pathology
2016
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Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome
by
Rivera, Michael
, Gupta, Sounak
, Erickson, Lori A.
, Zhang, Jun
in
Adult
/ Aged
/ Biomarkers, Tumor - analysis
/ Endocrinology
/ Female
/ Humans
/ Immunohistochemistry
/ Male
/ Medicine
/ Medicine & Public Health
/ Middle Aged
/ Oncology
/ Paraganglioma, Extra-Adrenal - enzymology
/ Paraganglioma, Extra-Adrenal - pathology
/ Pathology
/ Succinate Dehydrogenase - analysis
/ Succinate Dehydrogenase - biosynthesis
/ Urinary Bladder Neoplasms - enzymology
/ Urinary Bladder Neoplasms - pathology
2016
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Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome
Journal Article
Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome
2016
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Overview
Paragangliomas of the urinary bladder can arise sporadically or as a part of hereditary syndromes including those with underlying mutations in the succinate dehydrogenase (
SDH
) genes, which serve as tumor suppressors. SDH deficiency can be screened for by absence of immunohistochemical detection of SDHB. In this study of 11 cases, clinical follow-up was available for 9/11 cases. The cases were reviewed and graded based on the grading system for adrenal pheochromocytomas and paragangliomas (GAPP) criteria. Immunohistochemistry was performed for Ki67 and SDHB. Proliferative index was calculated by quantification of Ki67-positive cells at hot spots. The medical record was accessed for documentation of germline
SDH
mutations. Urinary bladder paragangliomas had a female predilection (8/11 cases), and 5/11 cases exhibited metastatic behavior. Patients with metastatic disease tended to be younger (mean age 43 vs 49 years), have larger lesions (5.8 vs 1.5 cm), and presented with catecholamine excess (4/4 vs 2/6 patients with non-metastatic lesions). Patients with metastatic disease had a higher mean Ki67 proliferation rate (4.9 vs 1.3 %) and GAPP score (mean of 5.8 vs 3.8) (
p
= 0.01). IHC for SDHB expression revealed loss of expression in 2/6 cases of non-metastatic paragangliomas compared to 4/5 patients with metastatic paragangliomas. Interestingly, of these four patients, two had a documented mutation of
SDHB
, one patient had a
SDHC
mutation, and another patient had a history of familial disease without mutation analysis being performed. Our study, suggests that SDH loss was suggestive of metastatic behavior in addition to younger age at diagnosis, larger tumor size, and higher Ki67 proliferation rate and catecholamine type.
Publisher
Springer US,Springer Nature B.V
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