What is the recommended management approach for paragangliomas according to the 2023 clinical consensus guideline?

Management of Paragangliomas According to 2023 Clinical Consensus Guideline

The 2023 clinical consensus guideline recommends intervention (including surgical resection) only for paragangliomas demonstrating sustained growth, compression of vital head and neck structures, or progression after radiation, with an individualized multidisciplinary approach for patients with multifocal head and neck paragangliomas (HNPGLs). 1

Initial Evaluation and Management

• All patients with paragangliomas should be managed by an expert interdisciplinary tumor team familiar with the disease to ensure favorable outcomes 1
• Complete evaluation should include:
  • Clinical assessment and biochemical measurements (metanephrines in plasma or urine and plasma 3-methoxytyramine) 1
  • Head/neck MRI as first imaging modality for HNPGLs to assess multifocality and tumor extension 1
  • Whole-body anatomic imaging with PET (preferably with radiolabeled somatostatin analogs) to search for paragangliomas on a whole-body scale 1
  • Thorough cranial nerve examination and laryngoscopy before any intervention 1

Surgical Management Decision Algorithm

Indications for Surgical Intervention:

• Sustained growth (especially rapid growth)
• Compression of vital head and neck structures
• Progression after radiation therapy
• Intractable pain
• Extensive cranial neuropathy
• Catecholamine-secreting lesions 1

Contraindications/Caution for Surgery:

• Primary lesions with distant metastasis (surgery only in select circumstances with palliative intent)
• Risk of bilateral cranial nerve deficits in multifocal disease
• Interventions that may cause more harm than relief 1

Preoperative Considerations

• For jugular and large carotid/vagal paragangliomas undergoing surgery:

  • Preoperative angiography with embolization should be considered
  • Balloon occlusion testing should be considered if internal carotid sacrifice with reconstruction is contemplated 1

• Specific surgical planning considerations:

  • Collaboration with vascular surgery when carotid sacrifice may be needed
  • Neurosurgery availability for skull base lesions with intracranial extent
  • For large jugular paragangliomas, subtotal resection with preservation of lower cranial nerves may be considered to minimize morbidity 1

Management of Multifocal Paragangliomas

• Multidisciplinary approach with particular attention to avoiding compromise of important neurovascular structures 1
• Staging resection is key to minimizing potential morbidity 1
• For bilateral tumors:
  • Consider operating on the side with existing cranial neuropathies first
  • Consider observing or radiating the contralateral side to avoid bilateral nerve palsies
  • If no neuropathies exist preoperatively, consider resecting the smaller lesion first 1
  • Stage resection of contralateral tumors several months later to allow for compensation 1

Postoperative Care

• Thorough cranial nerve examination and laryngoscopy after surgical intervention or radiotherapy 1
• In cases of postoperative facial nerve palsy, prioritize corneal protection to avoid exposure keratitis or corneal abrasion 1
• Monitor for potential complications:
  • New cranial neuropathies (common after surgical intervention)
  • Dysphagia and aspiration
  • Facial paralysis 1

Observation Strategy

• If observation is chosen, be aware that median growth rates may be as low as 1.0 mm/year with a median doubling time of 4.2-5.7 years 1
• Regular imaging follow-up is essential to monitor for growth 2

Special Considerations

• For functional paragangliomas (predominantly retroperitoneal), resection should be prioritized in patients with multifocal disease including HNPGLs 1
• Genetic testing should be considered in all patients with paraganglioma, as up to 50% may be hereditary 3
• Long-term follow-up is essential as paragangliomas can recur many years after initial diagnosis 4

The management of paragangliomas requires careful consideration of risks versus benefits, with the primary goal of minimizing morbidity while effectively controlling the disease. The 2023 clinical consensus guideline emphasizes a cautious approach to surgical intervention, reserving it for cases with clear indications of progression or symptomatology.