Microsurgical resection of a giant exclusively dopamine- secreting jugular foramen paraganglioma: A case report.

Background: Temporal bone paragangliomas are complex pathologies presenting close relationships with many critical neurovascular structures. Exclusively, dopamine-secreting paragangliomas are rare and may present a major hemodynamic challenge during intraoperative and postoperative periods, with great blood pressure lability due to the dose-dependent properties of dopamine. However, preoperative α or β blockage is usually not advised. Microsurgical resection is the treatment of choice; nevertheless, these tumors commonly present with greater size at diagnosis due to their non-specific clinical manifestations.

Case description: A 43-year-old male patient presented with headache, tinnitus, hearing loss, hypoglossal and facial nerve compromise, as well as vocal cord palsy. Magnetic resonance imaging depicted a giant posterior fossa mass centered in the left jugular foramen extending to the cervical space, associated with important bone erosion. Laboratory investigation depicted elevated serum dopamine concentration of >2.500 pg/mL (reference <30 pg/mL) and increased 24-h urine dosage of 3306 μg (reference <540 μg). Values of epinephrine, norepinephrine, and metanephrines were within normal range. The patient underwent preoperative embolization, and microsurgical resection was performed the next day. Serum and urinary dopamine concentrations were normalized following the procedure. The patient presented a favorable outcome, with no new neurological deficits.

Conclusion: Exclusively dopamine-secreting temporal bone paragangliomas may be successfully resected with a favorable outcome. A multidisciplinary, well-trained team is essential to manage intraoperative challenges up to postoperative rehabilitation adequately. Extensive laboratory training is essential to develop the surgical skills to master this skull-based approach.