Systematic Review of Multimodal Therapy for Metastatic Spine Paraganglioma: Focus on the Management of Succinate Dehydrogenase B-Mutated (SDHB) Subtype
Neurosurgeon King Abdulaziz University Milwaukee, WI, US
Introduction: Paragangliomas are rare neuroendocrine tumors that may cause symptoms from catecholamine secretion or mass effect. SDHB mutations, associated with a higher risk of metastasis, frequently lead to more aggressive paragangliomas. Nearly 50% of SDHB-mutated paragangliomas are malignant, with spine metastasis being common, affecting over 81% of cases. While no standardized adjuvant treatment exists, chemotherapy regimens, including cyclophosphamide, vincristine, and dacarbazine, are commonly used. Emerging treatments based on molecular profiles are under investigation. This systematic review focuses on current treatment strategies for SDHB-mutated paragangliomas to provide an updated overview of management approaches.
Methods: We conducted a systematic review using PRISMA guidelines, accessing PubMed and MEDLINE up to June 2024 with the keywords "paraganglioma" and "SDHB." After screening 94 articles, 14 studies met the inclusion criteria.
Results: The review included 66 patients, with an average age of 36.1 years. Of the identified patients, 82.4% were male. Primary tumor locations were most commonly in the abdomen (58.8%) and neck (29.4%). Surgical resection was the primary treatment for 74.2% of patients, with only 4.1% undergoing preoperative embolization. Radiotherapy was used in 11 patients, while 8 received chemotherapy with varied responses. Temozolomide showed promising results, particularly in patients with SDHB mutations. Table 1 summarizes the results of the reported cases.
Conclusion : Temozolomide is effective in treating metastatic paragangliomas, especially in patients with SDHB mutations, a key biomarker for treatment response. A multidisciplinary approach is recommended for managing rare cases. Continued tracking of outcomes is essential for optimizing treatment strategies for these rare tumors.