Pheochromocytoma
ICD-10 E27.5 · ICD-11 5A75
Clinical Scenario

Treatment of Pheochromocytoma with Unresectable Primary Head and Neck Paraganglioma

This protocol addresses pheochromocytoma presenting as an unresectable primary head and neck paraganglioma (HNPGL) — a situation where surgical resection is not feasible and alternative locoregional management is required.

Clinical Situation

In patients with unresectable primary head and neck paraganglioma, the primary challenge is achieving local disease control without the option of surgical removal. This scenario calls for evidence-based non-surgical approaches, as established by clinical consensus.

Treatment Approach

Radiation therapy represents the best studied and consensus-supported option in this setting. Several radiation-based modalities have been evaluated, and certain systemic approaches may also be considered under specific conditions.

Full regimen details, modality selection criteria, and sequencing are available in the complete protocol below.
References
DOI: 10.1097/MPA.0000000000001792
  1. In patients with unresectable primary HNPGL, RT represents the best studied option (consensus).
  2. Numerous reports have demonstrated excellent local control with either conventionally fractionated RT to 45 to 50 Gy, stereotactic radiosurgery (SRS), or fractionated stereotactic RT (SBRT).
  3. 131I-MIBG or 177Lu-DOTATATE is also a possible option if HNPGL uptake with 123I-MIBG or 68Ga-DOTATATE can be confirmed, respectively, although studies confirming benefits in HNPGL are lacking (consensus).
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