From the Guidelines
The investigation for a globus sensation potentially related to a jugular tumor should begin with a thorough clinical evaluation by an otolaryngologist (ENT specialist) and imaging studies, with head/neck magnetic resonance imaging (MRI) being the first modality to screen for assessment of multifocality and tumor extension, as recommended by the latest clinical consensus guideline 1. Initial assessment should include a complete head and neck examination with flexible nasolaryngoscopy to visualize the pharynx and larynx.
- Imaging studies are essential, with MRI providing superior soft tissue detail and being particularly valuable for evaluating the relationship of the tumor to surrounding structures, including blood vessels and cranial nerves.
- Angiography may be necessary to assess vascular involvement, especially if the tumor appears to be a paraganglioma (glomus jugulare), and preoperative angiography with embolization is recommended for all jugular, large, or locally invasive carotid/vagal PGLs 1.
- Laboratory tests should include complete blood count, basic metabolic panel, and in cases of suspected paragangliomas, measurement of plasma or urinary catecholamines and metanephrines to rule out functionality.
- A biopsy may be required for definitive diagnosis, though this should be approached cautiously in suspected vascular tumors.
- Early referral to a multidisciplinary team including neurosurgery, vascular surgery, and radiation oncology is recommended as jugular tumors often require complex management strategies, and an individualized approach is recommended for patients with multifocal HNPGLs, with particular attention paid to avoiding compromising important neurovascular structures 1.
From the Research
Investigation for Globus Jugulare Tumor
- The investigation for globus jugulare tumor involves various imaging techniques, including magnetic resonance imaging (MRI) and computed tomography (CT) scans, as well as angiography 2.
- MRI is considered the modality of choice for assessing middle ear, skull base, or posterior fossa involvement and monitoring growth of paraganglioma 2.
- CT scans are useful for showing subtle destruction of the temporal bone on early stage of disease 2.
- Angiography, in combination with embolization, is mainly used prior to surgical resection 2.
Surgical Management
- The surgical management of glomus jugulare tumors is complex and challenging due to their highly vascular nature and involvement of critical neurovascular structures 3, 4.
- A combined transmastoid retro- and infralabyrinthine transjugular transcondylar transtubercular high cervical approach can be performed in a stepwise fashion for the resection of complex jugular foramen tumors 5.
- Planned subtotal resection followed by radiotherapy (RT) or stereotactic radiosurgery for the residual tumor may yield a better outcome with lower morbidity and mortality 4.
Radiosurgery
- Radiosurgery has emerged as a promising approach to the management of glomus jugulare tumors, with high rates of tumor and clinical control 6.
- The gamma knife, linear accelerator, and CyberKnife technologies all exhibit high rates of tumor and clinical control 6.
- Radiosurgery may be considered for the primary management of glomus jugulare tumors due to its high effectiveness 6.
Diagnostic Criteria and Treatment Outcomes
- Tumors of the glomus jugulare are benign, slow-growing paragangliomas, and their natural history, surgical treatment, and outcome have been well addressed in the literature 3.
- Complex tumors, including multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously with an adverse outcome, are high-risk and present surgical challenges 3.
- Treatment decisions, avoidance of complications, surgical refinements, and patient outcomes are crucial in the management of complex glomus jugulare tumors 3.