We describe two cases of metastatic retroperitoneal paraganglioma associate
d with extradural spinal cord compression. Both occurred in young men; one
being metastatic at presentation, the other becoming metastatic 19 years af
ter attempted surgical resection. Despite a long, relatively stable natural
history after diagnosis (10 and 19 years, respectively) both had an accele
ration of their disease once extradural disease was detected. These cases i
llustrate the potentially aggressive nature of this disease, the need for l
ong-term follow-up and the effectiveness of a variety of therapies for pall
iation, and also raise the possibility of "prophylactic" treatment to preve
nt spinal cord compression.