Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining

Alakeel, Fadi; Sannaa, Ghadah Al; Ibarra-Cortez, Sergio H.; Reardon, Michael J.; Czerniak, Bogdan; Chan, Edward Y.; Ro, Jae Y.

doi:10.1016/j.anndiagpath.2020.151477

Cited by 2 publications

(4 citation statements)

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“…Genetic testing is recommended for all PGL patients, not only because genetic diagnosis provides important information for clinical management, prognosis, and translational research, but also to increase understanding of the hereditary nature of these tumors. Currently, 22 genes have been linked to the pathogenesis of PGLs, and 30% to 40% of PGLs are hereditary (23)(24)(25)(26). Among CPGL patients, mutations in SDHB and SDHD have been most commonly reported; mutations in PGL-associated genes such as the rearranged during transfection and von Hippel-Lindau genes have not been detected (6).…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China

Dong

Meng

Zhang

et al. 2022

Front. Cardiovasc. Med.

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Background: Cardiac paragangliomas (CPGLs) are rare neuroendocrine tumors that are easily overlooked and difficult to diagnose. Detailed comprehensive data regarding CPGL diagnosis and outcome are lacking.Methods: We retrospectively analyzed a cohort of 27 CPGL patients. This cohort represents the largest such cohort reported to date.Results: The prevalence of trilogy symptoms (concurrent palpitations, hyperhidrosis, and headache) was frequent (9/27, 33.3%). Sensitivity of echocardiography and contrast-enhanced computed tomography for localization of CPGL were 81.8% and 87%, respectively. Octreotide scintigraphy showed 100% sensitivity for detecting GPCLs, while sensitivity of I131-metaiodoben-zylguanidine scintigraphy was only 32.9%. Multiple tumors were found in 29.6% of patients. Most CPGLs originated from the epicardium or root of the great vessels (92.9%) and were mostly supplied by the coronary arteries and their branches (95.7%). Twenty-four patients underwent surgical treatment. Although local invasion was present in 40.0% of patients, it did not affect long-term outcome. Mean follow-up was 6.9 ± 3.6 years. Biochemical remission was achieved in 85% of patients. The recurrence rate was 15%.Conclusions: Manifestations of CPGLs are non-specific and they can be difficult to detect on imaging examinations. Octreotide scintigraphy should be performed in patients with suspected paragangliomas to screen for multiple lesions. Surgical resection of CPGLs can achieve symptom relief and biochemical remission.

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Section: Discussionmentioning

confidence: 99%

Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China

Dong

Meng

Zhang

et al. 2022

Front. Cardiovasc. Med.

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“…Negative SDHB immunostaining is seen in ~40% of patients with cardiac PGs. We have previously demonstrated that SDHB-negative cases are correlated with aggressive histologic features [2].…”

Section: The Genetic Basis Of Paragangliomamentioning

confidence: 95%

“…The average age at diagnosis ranged from 42 to 52 years [1,2]. PGs are slightly more common in females with male to female ratio of 0.85:1 [3].…”

Section: Clinical Presentationmentioning

confidence: 99%

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Cardiac Paraganglioma, Recent Advances in Clinicopathologic Features

Alakeel

Reardon

2021

Multidiscip Cancer Investig

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Cardiac paragangliomas (PGs) are rare neuroendocrine tumors comprising less than 1% of cardiac tumors. Few cardiac PG case reports and series are described in the literature. Cardiac PGs may be sporadic or arise from a syndromic association. Clinical presentations vary depending on the biochemical activity and location of the tumor. The left atrium, the right atrium, AP window, left ventricle, and atrioventricular groove are the most common sites for cardiac PGs, respectively. Many cardiac PGs are associated with succinate dehydrogenase (SDH) gene mutations. SDH-mutated PGs have aggressive histologic and clinical behavior. Therefore, PG patients should be screened for SDH mutations and provided with appropriate genetic counseling. SDH immunostaining can be used as a substitute diagnostic modality for SDH gene mutation and negative staining is associated with SDH mutation. Biochemical analysis, anatomical imaging, and functional imaging are also used for diagnostic workup of the tumor. Surgery is the only curative treatment for this tumor. Adrenoceptor blockers should be administered in functional PGs. PGs are highly vascular and frequently situated close to vital vessels. Accordingly, surgical complications such as bleeding are a leading cause of mortality in PGs. Metastatic PGs are only seen in a small subset of patients and are associated with poor clinical outcomes. Herein, we summarize clinical and pathological advances in cardiac PGs.

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Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining

Cited by 2 publications

References 25 publications

Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China

Diagnosis and Outcome of Cardiac Paragangliomas: A Retrospective Observational Cohort Study in China

Cardiac Paraganglioma, Recent Advances in Clinicopathologic Features

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