RESUMEN
INTRODUCTION: Paragangliomas are tumors of neuroendocrine origin, may appear in different localizations, and are related to the autonomic nervous system. Paragangliomas are generally asymptomatic and may rarely appear with adrenergic symptoms, and clinical findings depend on the catecholamines they secrete. Extra-adrenal paragangliomas are mostly benign, like all paragangliomas. Malignancy criteria consist of local recurrence, metastasis after total resection, and presence of distant metastasis during primary diagnosis. CASE PRESENTATION: This report presents the case of a 31-year-old man with jugular paraganglioma, multiple skeletal metastases, and a long-segment tumor thrombus. Imaging procedures showed a continuous tumor thrombus extending from the posterior fossa to the right atrium and metastases in C2, T1, T6, T8, L5, and right humerus. Histopathological assessment of the metastasis in C2 identified malignant paraganglioma. Curative surgery was not an option for this patient, hence combined chemotherapy was given. CONCLUSION: In cases of malignant paraganglioma with multiple distant metastases, chemotherapy and radiotherapy are feasible treatment methods.