Systematic Review and Case Report of Multimodal Therapy for Treatment of Advanced Thoracic Spine Paraganglioma.

Background: Paragangliomas are rare neuroendocrine tumors that can manifest with endocrinological symptoms from excessive catecholamine secretion and symptoms from mass effect depending on biochemical behavior and tumor location.

Methods: Following an encounter with a patient with a rare case of paraganglioma of the thoracic spine, a systematic review was performed of patients with a diagnosis of paraganglioma and SDHB mutation. Inclusion criteria were met by 14 studies for a total of 66 patients.

Results: A 55-year-old man with a known history of treated metastatic paraganglioma with SDHB mutation presented with progressive weakness in his extremities. He was found to have significant spinal cord compression from a lesion localized to T1-T3 vertebrae with vertebral body as well as epidural involvement. This area was previously treated with laminectomy and fusion as well as radiotherapy. Given his neurological symptoms and failure of previous radiotherapy treatment, our treatment approach was aggressive multidisciplinary intervention. He successfully underwent endovascular embolization, surgical resection with instrumented fusion, radiotherapy, and chemotherapy with temozolomide to treat this lesion as well as an incidentally located T12 lesion. Given the rarity of this disease, we performed a systematic review of paraganglioma with SDHB mutation, which demonstrated a wide variety of treatment options.

Conclusions: Although metastatic/multifocal paragangliomas are rare, their occasional relentless course may require complex multidisciplinary treatment to maximize neurological and oncological outcomes.