Malignant Jugulotympanic Paraganglioma: Report of Two Cases

Background Malignant paragangliomas in the head and neck are extremely rare; we report two cases of malignant jugulotympanic paraganglioma.

Case 1 A 45-year-old woman presented with complaint of pulsatile tinnitus, hearing loss, pain, and otorrhea that began in 2005. At clinical examination was seen a red pulsatile mass in the external auditory channel.

The MRI demonstrated a lesion in the jugular foramen extending to the petrous apex, middle ear, and external auditory channel, with the typical “salt and pepper” pattern and important postcontrast enhancement.

Diagnosed as jugulotympanic paraganglioma was submitted to surgical excision. Suspect lymph node was seen in surgery and was opted to selective neck dissection. A micrometastasis was found in one lymph node in level V. Her treatment was completed with adjuvant radiotherapy. At 12-month follow-up, the patient maintained only a facial palsy grade IV HB scale, without other symptoms.

Case 2 A 33-year-old woman presented with complaints of vertigo and pain. She had previous surgery for resection of a jugulotympanic paraganglioma 10 years before in other hospital. At clinical examination, she was seen a red mass in the external auditory channel and ipsilateral facial palsy grade VI in HB scale.

It was demonstrated in the MRI a lesion in the jugular foramen with extension to the petrous apex, internal auditory channel, middle ear, external auditory channel, and caudally to the level of C2, with an important enhancement postcontrast.

She had surgical excision via infratemporal approach. In the histopathological exam, invasion of a nonidentified nerve in the lacerum foramen was demonstrated. She was referred to adjuvant radiotherapy. At 3-month follow-up, the patient maintained dysphonia due to a paramedian vocal cord but presented no other symptoms.

Discussion The paragangliomas represent 3% of all the tumors in the head and neck. They occur mainly in the carotid body, vagal paraganglia, tympanic paraganglia, and in the jugular paraganglia, near the jugular bulb.

The carotid body and vagal paraganglioma usually manifest as a painless, sometimes pulsatile, neck mass. The main complaints in the jugulotympanic are pulsatile tinnitus, hearing loss, vertigo, facial palsy, and otorrhea.

Malignant paragangliomas are extremely rare and represent 6 to 19% of all head and neck paragangliomas. There is not a consensus around the criteria to classify a paraganglioma as malignant. The most accepted is the evidence of regional or distant metastasis to nonendocrine tissues. Vagal paragangliomas are the most susceptible (19%), followed by carotid body (6%) and jugulotympanic (2–4%).

The patients are usually younger, with a higher risk to multifocal and secreting tumors. There is a strong relation between mutations in the SDH-B gene and malignant paragangliomas. The most affected site is the regional lymph nodes.

The prognosis is usually good in the overall 5-year survival rate (59.5–84%), except when distant metastasis are present.

The classical treatment is the surgical excision, associated with neck dissection and postoperative radiotherapy. Chemotherapy is usually reserved for patients with distant metastasis.

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