A Catecholamine-Secreting Skull Base Sinonasal Paraganglioma Presenting with Labile Hypertension in a Patient with Previously Undiagnosed Genetic Mutation

Abstract

Sinonasal paragangliomas are very uncommon neuroendocrine tumors that can present as skull base lesions. Functional paragangliomas are exceedingly rare. They can be associated with genetic mutations that have been associated with increased risk of head and neck paragangliomas. We present a case of a rare functioning sinonasal paraganglioma of the skull base in a patient with distant history of prior abdominal paragangliomas. The patient underwent subtotal endoscopic resection of the skull base lesion limited by carotid encasement of the tumor. They were treated with postoperative adjuvant radiation and therapeutic metaiodobenzylguanidine (MIBG) therapy. Genetic testing revealed succinate dehydrogenase B (SDHB) mutation. Skull base paragangliomas are rare tumors that may preclude complete surgical resection. ¹³¹Iodine-MIBG can be used as adjuvant therapy in postoperative external beam radiation and in MIBG avid tumors. Long-term follow-up is needed given locally aggressive nature of these tumors, especially for patients with history of genetic mutations such as SDHB mutations as recurrent paragangliomas may develop.

Keywords

head and neck paraganglioma - sinonasal paraganglioma - endoscopic skull base resection - succinate dehydrogenase mutation

• References

• 1 Lee JH, Barich F, Karnell LH , et al; American College of Surgeons Commission on Cancer; American Cancer Society. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002; 94 (3) 730-737
• 2 Manolidis S, Shohet JA, Jackson CG, Glasscock III ME. Malignant glomus tumors. Laryngoscope 1999; 109 (1) 30-34
• 3 Myssiorek D. Head and neck paragangliomas: an overview. Otolaryngol Clin North Am 2001; 34 (5) 829-836, v
• 4 Brouwers FM, Eisenhofer G, Tao JJ , et al. High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testing. J Clin Endocrinol Metab 2006; 91 (11) 4505-4509
• 5 Morales H, Castillo M, Jewells V. Paraganglioma of the sphenoid sinus: case report and review of literature. Clin Imaging 2007; 31 (1) 32-36
• 6 Kuhn JA, Aronoff BL. Nasal and nasopharyngeal paraganglioma. J Surg Oncol 1989; 40 (1) 38-45
• 7 Kuhweide R, Lanser MJ, Fisch U. Catecholamine-secreting paragangliomas at the skull base. Skull Base Surg 1996; 6 (1) 35-45
• 8 Apple D, Kreines K. Cushing’s syndrome due to ectopic ACTH production by a nasal paraganglioma. Am J Med Sci 1982; 283 (1) 32-35
• 9 Koegel Jr L, Levine HL, Waldman SR. Paraganglioma of the sphenoid sinus appearing as labile hypertension. Otolaryngol Head Neck Surg 1982; 90 (6) 704-707
• 10 Amar L, Bertherat J, Baudin E , et al. Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol 2005; 23 (34) 8812-8818
• 11 Myssiorek D, Halaas Y, Silver C. Laryngeal and sinonasal paragangliomas. Otolaryngol Clin North Am 2001; 34 (5) 971-982, vii
• 12 Gedik GK, Hoefnagel CA, Bais E, Olmos RA. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 2008; 35 (4) 725-733
• 13 Fitzgerald PA, Goldsby RE, Huberty JP , et al. Malignant pheochromocytomas and paragangliomas: a phase II study of therapy with high-dose 131I-metaiodobenzylguanidine (131I-MIBG). Ann N Y Acad Sci 2006; 1073: 465-490