Clinical features and treatment of spinal metastatic pheochromocytoma and paraganglioma: a single institutional experience with 18 patients

Abstract

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors, which can potentially metastasize to the spine. Clinical researches and treatment guideline on this entity are lacking. This study described the clinical features and treatment outcomes of metastatic PPGLs on the spine. We reviewed 18 patients with spinal PPGLs who were consecutively treated in our spinal center. The cohort included ten cases of pheochromocytomas and eight paragangliomas. The local pain and neurological deficits were the two most common symptoms. One third of the spinal PPGLs were diagnosed as functional tumors. The surgical strategies for the cohort included percutaneous vertebroplasty, neurological decompression and partial tumor resection with or without internal fixation, and total en-bloc resection. The postoperative courses were uneventful except one patient developed heart failure. The adjuvant therapies were implemented in six patients with 131I-MIBG, five with radiotherapy, two with chemotherapy and one with target therapy. The median survival period was 39 months. The patients’ Karnofsky performance scores were positively correlated with the overall survival (p < 0.05).Therefore, surgery is indicated for intractable local pain and neurological impairment in the patients with spinal PPGLs, while a holistic set of adjuvant therapies shall be incorporated for a better tumor control.