Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Mar 12;10(3):109-112.
doi: 10.1016/j.aace.2024.03.003. eCollection 2024 May-Jun.

Dopamine-Secreting Carotid Body Paraganglioma in a Patient With SDHB Mutation

Katherine Mustafa  1 , Sara Zadeh  2 , Silas A Culver  1
Affiliations
Case Reports

Dopamine-Secreting Carotid Body Paraganglioma in a Patient With SDHB Mutation

Katherine Mustafa et al. AACE Clin Case Rep. .

Abstract

Background/objective: Exclusively dopamine-secreting paragangliomas (PGLs) are rare, and the majority of head and neck PGLs are nonsecretory. Here, we describe a patient with succinate dehydrogenase subunit B (SDHB) mutation and a dopamine-secreting carotid body PGL to highlight the potential importance of screening for dopamine excess in patients with suspected PGL.

Case report: We report a 34-year-old patient who presented with cranial nerve palsy and was found to have a cerebellopontine PGL. Biochemical testing demonstrated increased circulating dopamine levels with normal levels of other catecholamines. Dopamine excess improved with resection of the PGL, and subsequent genetic testing revealed an SDHB mutation.

Discussion: Secretory head and neck PGLs and exclusively dopamine-secreting PGLs are both uncommon and rarely present together, although PGLs in patients with SDHB mutations often do produce dopamine. Although current guidelines do not recommend routine evaluation of the dopamine levels in patients at risk for PGL, dopamine-secreting PGLs are frequently locally invasive or metastatic.

Conclusion: Screening for dopamine excess in patients with a predisposition to PGL or with suspected PGL may aid in diagnosis and as a marker of successful treatment.

Keywords: SDHB mutation; dopamine; paraganglioma.

PubMed Disclaimer

Conflict of interest statement

The authors have no conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
A, T1 coronal magnetic resonance imaging showing the patient’s right cerebellopontine mass infiltrating the right petrous bone, right internal auditory canal, and jugular foramen. B, Gallium-68 DOTATATE positron emission tomography showing avidity of the right cerebellopontine mass and a right level II neck mass posterior to the carotid.
Fig. 2
Fig. 2
A, Hematoxylin and eosin–stained section of paraganglioma showing nests of bland tumor cells with round nuclei and abundant granular eosinophilic cytoplasm. B, Immunohistochemical staining for synaptophysin was diffusely positive. C, S100 showed sustentacular staining at the periphery of tumor cell nests, supporting the diagnosis. D, Staining for SDHB showed aberrant loss of expression within the tumor.
See this image and copyright information in PMC

References

    1. Erickson D., Kudva Y.C., Ebersold M.J., et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001;86(11):5210–5216. - PubMed
    1. Hayward N., Cousins V. Head and neck paraganglioma: medical assessment, management, and literature update. JOHBM. 2018;1(1)
    1. Miyamoto S., Yoshida Y., Ozeki Y., et al. Dopamine-secreting pheochromocytoma and paraganglioma. J Endocr Soc. 2021;5(12) - PMC - PubMed
    1. Timmers H.J., Kozupa A., Eisenhofer G., et al. Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas. J Clin Endocrinol Metab. 2007;92(3):779–786. - PubMed
    1. Eisenhofer G., Goldstein D.S., Sullivan P., et al. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 2005;90(4):2068–2075. - PubMed

Publication types

LinkOut - more resources