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

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

Cardiac paragangliomas (PGs) are very rare tumors that comprise less than 1% of all cardiac tumors. PGs can occur sporadically, but inherited syndromes may also play a role in the development of PGs. Approximately one-third of PGs are associated with mutations in the succinate dehydrogenase (SDH) complex, specifically SDHB, as part of syndrome-associated PGs or sporadic PGs. SDH mutations have been assessed by SDHB immunohistochemistry, as negative staining indicates a high likelihood of mutation in PGs in other sites, but not in cardiac PGs. This study aims to evaluate the clinical and pathologic characteristic of cardiac PG cases and assess the expression of SDHB by immunohistochemistry. A retrospective chart analysis of 10 patients with cardiac PG was performed to assess the patient age, sex, size, site of the tumor, and clinical symptoms. Histologically the tumors showed the classic pattern of nested tumor cells surrounded by sustentacular cells. Immunohistochemistry for SDHB was performed in five cases. One case showed a complete absence of SDHB immunohistochemical staining and the others showed staining ranging from a weak-to-strong granular cytoplasmic staining pattern. We conclude that SDHB immunostaining is cost-effective in identifying cases with SDH mutation. It is recommended to assess SDH mutation in patients with cardiac PG to predict the aggressive behavior that has been reported by previous studies from PGs of other sites.

Original languageEnglish (US)
Article number151477
JournalAnnals of Diagnostic Pathology
Volume45
DOIs
StatePublished - Apr 1 2020

PMID: 32062474

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Cardiac paragangliomas : A case series with clinicopathologic features and succinate dehydrogenase B immunostaining. / Alakeel, Fadi; Al Sannaa, Ghadah; Ibarra-Cortez, Sergio H.; Reardon, Michael J.; Czerniak, Bogdan; Chan, Edward Y.; Ro, Jae.

In: Annals of Diagnostic Pathology, Vol. 45, 151477, 01.04.2020.

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Harvard

Alakeel, F, Al Sannaa, G, Ibarra-Cortez, SH, Reardon, MJ, Czerniak, B, Chan, EY & Ro, J 2020, 'Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining' Annals of Diagnostic Pathology, vol. 45, 151477. https://doi.org/10.1016/j.anndiagpath.2020.151477

APA

Alakeel, F., Al Sannaa, G., Ibarra-Cortez, S. H., Reardon, M. J., Czerniak, B., Chan, E. Y., & Ro, J. (2020). Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining. Annals of Diagnostic Pathology, 45, [151477]. https://doi.org/10.1016/j.anndiagpath.2020.151477

Vancouver

Alakeel F, Al Sannaa G, Ibarra-Cortez SH, Reardon MJ, Czerniak B, Chan EY et al. Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining. Annals of Diagnostic Pathology. 2020 Apr 1;45. 151477. https://doi.org/10.1016/j.anndiagpath.2020.151477

Author

Alakeel, Fadi ; Al Sannaa, Ghadah ; Ibarra-Cortez, Sergio H. ; Reardon, Michael J. ; Czerniak, Bogdan ; Chan, Edward Y. ; Ro, Jae. / Cardiac paragangliomas : A case series with clinicopathologic features and succinate dehydrogenase B immunostaining. In: Annals of Diagnostic Pathology. 2020 ; Vol. 45.

BibTeX

@article{fd76e362e6ac4d62ab9f6fba8cd5a51a,
title = "Cardiac paragangliomas: A case series with clinicopathologic features and succinate dehydrogenase B immunostaining",
abstract = "Cardiac paragangliomas (PGs) are very rare tumors that comprise less than 1{\%} of all cardiac tumors. PGs can occur sporadically, but inherited syndromes may also play a role in the development of PGs. Approximately one-third of PGs are associated with mutations in the succinate dehydrogenase (SDH) complex, specifically SDHB, as part of syndrome-associated PGs or sporadic PGs. SDH mutations have been assessed by SDHB immunohistochemistry, as negative staining indicates a high likelihood of mutation in PGs in other sites, but not in cardiac PGs. This study aims to evaluate the clinical and pathologic characteristic of cardiac PG cases and assess the expression of SDHB by immunohistochemistry. A retrospective chart analysis of 10 patients with cardiac PG was performed to assess the patient age, sex, size, site of the tumor, and clinical symptoms. Histologically the tumors showed the classic pattern of nested tumor cells surrounded by sustentacular cells. Immunohistochemistry for SDHB was performed in five cases. One case showed a complete absence of SDHB immunohistochemical staining and the others showed staining ranging from a weak-to-strong granular cytoplasmic staining pattern. We conclude that SDHB immunostaining is cost-effective in identifying cases with SDH mutation. It is recommended to assess SDH mutation in patients with cardiac PG to predict the aggressive behavior that has been reported by previous studies from PGs of other sites.",
keywords = "Cardiac paraganglioma, Neuroendocrine tumor, Paraganglioma, Succinate dehydrogenase B",
author = "Fadi Alakeel and {Al Sannaa}, Ghadah and Ibarra-Cortez, {Sergio H.} and Reardon, {Michael J.} and Bogdan Czerniak and Chan, {Edward Y.} and Jae Ro",
year = "2020",
month = "4",
day = "1",
doi = "10.1016/j.anndiagpath.2020.151477",
language = "English (US)",
volume = "45",
journal = "Annals of Diagnostic Pathology",
issn = "1092-9134",
publisher = "W.B. Saunders Ltd",

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RIS

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T1 - Cardiac paragangliomas

T2 - Annals of Diagnostic Pathology

AU - Alakeel, Fadi

AU - Al Sannaa, Ghadah

AU - Ibarra-Cortez, Sergio H.

AU - Reardon, Michael J.

AU - Czerniak, Bogdan

AU - Chan, Edward Y.

AU - Ro, Jae

PY - 2020/4/1

Y1 - 2020/4/1

N2 - Cardiac paragangliomas (PGs) are very rare tumors that comprise less than 1% of all cardiac tumors. PGs can occur sporadically, but inherited syndromes may also play a role in the development of PGs. Approximately one-third of PGs are associated with mutations in the succinate dehydrogenase (SDH) complex, specifically SDHB, as part of syndrome-associated PGs or sporadic PGs. SDH mutations have been assessed by SDHB immunohistochemistry, as negative staining indicates a high likelihood of mutation in PGs in other sites, but not in cardiac PGs. This study aims to evaluate the clinical and pathologic characteristic of cardiac PG cases and assess the expression of SDHB by immunohistochemistry. A retrospective chart analysis of 10 patients with cardiac PG was performed to assess the patient age, sex, size, site of the tumor, and clinical symptoms. Histologically the tumors showed the classic pattern of nested tumor cells surrounded by sustentacular cells. Immunohistochemistry for SDHB was performed in five cases. One case showed a complete absence of SDHB immunohistochemical staining and the others showed staining ranging from a weak-to-strong granular cytoplasmic staining pattern. We conclude that SDHB immunostaining is cost-effective in identifying cases with SDH mutation. It is recommended to assess SDH mutation in patients with cardiac PG to predict the aggressive behavior that has been reported by previous studies from PGs of other sites.

AB - Cardiac paragangliomas (PGs) are very rare tumors that comprise less than 1% of all cardiac tumors. PGs can occur sporadically, but inherited syndromes may also play a role in the development of PGs. Approximately one-third of PGs are associated with mutations in the succinate dehydrogenase (SDH) complex, specifically SDHB, as part of syndrome-associated PGs or sporadic PGs. SDH mutations have been assessed by SDHB immunohistochemistry, as negative staining indicates a high likelihood of mutation in PGs in other sites, but not in cardiac PGs. This study aims to evaluate the clinical and pathologic characteristic of cardiac PG cases and assess the expression of SDHB by immunohistochemistry. A retrospective chart analysis of 10 patients with cardiac PG was performed to assess the patient age, sex, size, site of the tumor, and clinical symptoms. Histologically the tumors showed the classic pattern of nested tumor cells surrounded by sustentacular cells. Immunohistochemistry for SDHB was performed in five cases. One case showed a complete absence of SDHB immunohistochemical staining and the others showed staining ranging from a weak-to-strong granular cytoplasmic staining pattern. We conclude that SDHB immunostaining is cost-effective in identifying cases with SDH mutation. It is recommended to assess SDH mutation in patients with cardiac PG to predict the aggressive behavior that has been reported by previous studies from PGs of other sites.

KW - Cardiac paraganglioma

KW - Neuroendocrine tumor

KW - Paraganglioma

KW - Succinate dehydrogenase B

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ID: 58804475