Paraganglioma of the spine: A twenty-years clinical experience of a high volume tumor center

Abstract

Paragangliomas (PGs) are rare tumours with a reported estimated annual incidence of up to 3 per million. Spinal involvement may possible with spinal metastasis and primary extradural localizations.The aim of this paper is to evaluate clinical outcomes of surgical treatment of a rare disease that can involve the spine and that should be considered in the differential diagnosis of spinal injuries.This is a retrospective observational study of the spinal paragangliomas treated at our institute. Five patients have been enrolled: three with metastatic PG and two with extradural PG. Metastatic PGs were treated with intralesional excision plus adjuvant therapies instead, extradural PGs with intralesional excision without adjuvant therapies.Among patients affected by metastastic paraganglioma two patients were Alive with disease (AWD) at the latest follow and one patient died for the spreading of disease at 240 months after surgery. Two patients with extradural paraganglioma of thoracic spine were AWD at the latest follow-up without pain and neurological deficits.Surgical management of spinal localizations can represent a challenge. Surgery has a main role in both diseases where intralesional excision plus adjuvant therapies seems to be able to achieve the local control and a satisfying prognosis in case of undisseminated tumour.