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.