Paragangliomas: A Case Series from Burdenko Center of Neurosurgery

Paragangliomas (PGLs), also known as chemodectomas or glomus tumors, are typically benign, slow-growing tumors arising from sympathetic nerve cells (chromaffin paraganglion cells) located along major blood vessels. Paragangliomas of the head and neck (HNPs) are very rare, representing only 0.01% of all head and neck tumors. At the same time, PGLs are the most frequent lesions of the middle ear and jugular foramen. Current treatment options for HNPs include surgical resection, endovascular embolization, conventionally fractionated external beam radiation therapy, stereotactic radiosurgery (SRS), or a combination of these modalities. Surgery has traditionally been the preferred method of treatment. In practice, however, approximately 40–70% of patients are initially seen with unresectable tumors (van der Mey et al., 1992). That is, the surgical removal in these patients is obviously subtotal. Due to the highly vascular nature and relatively inaccessible anatomical location of HNPs, excision is often problematic and carries a considerable risk of morbidity and mortality to the patient, including cerebrospinal fluid leak, wound infection, pulmonary embolism, and lower cranial nerve deficits (Guss et al., 2011). Radiation-based therapies, including SRS, 3D conformal radiotherapy, and intensity-modulated radiation therapy, offer a similar chance of tumor control with lower risks of morbidity compared to surgery in patients with HNPs.