EC Clinical and Medical Case Reports

Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal paraganglionic cells of the autonomic nervous system. In the head and neck region, jugulotympanic paragangliomas are among the most common tumors of the temporal bone. Although typically benign and slow-growing, they may lead to significant morbidity due to local extension. We report the case of a 62-year-old patient presenting with progressive left-sided hearing loss associated with pulsatile tinnitus. Magnetic resonance imaging revealed a tympanojugular paraganglioma measuring 32 x 13 x 12 mm with extension to adjacent structures. Surgical resection was contraindicated due to the highly vascular nature of the lesion. The patient was treated with fractionated stereotactic radiotherapy (30 Gy in 5 fractions). At three-month follow-up, clinical improvement was observed with minimal residual symptoms and no treatment-related toxicity. Imaging demonstrated a reduction in tumor size and disease stabilization. Stereotactic radiotherapy represents a safe, effective, and minimally invasive therapeutic option for unresectable tympanojugular paragangliomas, offering favorable tumor control with low morbidity.

Keywords: Tympanojugular Paraganglioma; Stereotactic Radiotherapy; Fractionated Radiotherapy; Temporal Bone Tumor; Pulsatile Tinnitus; Case Report

1. Williams MD. “Paragangliomas of the head and neck: an overview from diagnosis to genetics”. Head and Neck Pathology 11 (2017): 278-287.
2. Fisch U. “Infratemporal fossa approach for glomus tumors of the temporal bone”. Annals of Otology, Rhinology, and Laryngology 91 (1982): 474-479.
3. Offergeld C., et al. “Head and neck paragangliomas: clinical and molecular genetic classification”. Clinics (Sao Paulo)1 (2012): 19-28.
4. Valentin G. “Ueber eine gangliöse Anschwellung im Bereiche des Nervus glossopharyngeus und seiner Äste”. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin (1841): 287-290.
5. Guild SR. “The glomus jugulare, a nonchromaffin paraganglion, in man”. Annals of Otology, Rhinology, and Laryngology 4 (1953): 1045-1071.
6. Rosenwasser H. “Carotid body tumor of the middle ear and mastoid”. Archives of Otolaryngology 1 (1945): 64-67.
7. Alford BR and Guilford FR. “A comprehensive study of tumors of the glomus jugulare”. Laryngoscope 6 (1962): 765-805.
8. Lack EE. “Tumors of the adrenal gland and extra-adrenal paraganglia. Atlas of tumor pathology, 3^rd Series, Fascicle 19”. Armed Forces Institute of Pathology, Washington, DC (1997).
9. Borba LA and Al-Mefty O. “Intravagal paragangliomas: report of four cases”. Neurosurgery 3 (1996): 569-575.
10. Stewart KL. “Paragangliomas of the temporal bone”. American Journal of Otolaryngology4 (1993): 219-226.
11. van Baars FM., et al. “Familial non-chromaffin paragangliomas (glomus tumors): clinical and genetic aspects”. Acta Oto-Laryngologica 1-6 (1981): 589-593.
12. Pellitteri PK., et al. “Paragangliomas of the head and neck”. Oral Oncology6 (2004): 563-575.
13. Manolidis S., et al. “Malignant glomus tumors”. Laryngoscope1 (1999): 30-34.
14. Lack EE., et al. “Paragangliomas of the head and neck region: a pathologic study of tumors from 71 patients”. Human Pathology2 (1979): 191-218.
15. Lee JH., et al. “National cancer data base report on malignant paragangliomas of the head and neck”. Cancer 3 (2002): 730-737.
16. Boedeker CC., et al. “Paragangliomas of the head and neck: diagnosis and treatment”. Familial Cancer 4 (2005): 55-59.
17. Remley KB., et al. “Pulsatile tinnitus and the vascular tympanic membrane: CT, MR, and angiographic findings”. Radiology2 (1990): 383-389.
18. Forest JA 3rd., et al. “Long-term control of surgically treated glomus tympanicum tumors”. Otology and Neurotology 2 (2001): 232-236.
19. Gerosa M., et al. “Glomus jugulare tumors: the option of gamma knife radiosurgery”. Neurosurgery3 (2006): 561-569.
20. Shulkin BL., et al. “Pheochromocytomas and paragangliomas: imaging with 111In-pentetreotide”. Radiology 188 (1993): 35-40.
21. Fisch U and Mattox DE. “Microsurgery of the skull base”. Thieme Medical Publishers, New York (1988).