ENDOCRINOLOGY - II
Therapy 

The Quarterly Journal of Nuclear Medicine 1999 December;43(4):344-55

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: English

Nuclear medicine therapy of pheochromocytoma and paraganglioma

Troncone L., Rufini V.

From the Department of Nuclear Medicine Catholic University of the Sacred Heart, Rome, Italy

Pheochromocytomas and par­a­gan­gli­o­mas are ­rare cat­e­chol­a­mine-pro­duc­ing ­tumors ­which ­arise ­from chro­maf­fin tis­sue. When a phe­och­rom­o­cy­to­ma/par­a­gan­gli­o­ma is sus­pect­ed, bio­chem­i­cal con­fir­ma­tion is ­based on 24-­hour uri­nary excre­tion ­rates of cat­e­chol­a­mines and ­their metab­olites (met­a­neph­rines, VMA, etc.). Following bio­chem­i­cal con­fir­ma­tion non inva­sive imag­ing tech­niques ­such as CT and/or MR of the abdo­men and 123I-­MIBG scin­tig­ra­phy are per­formed to local­ize the ­tumor. 111In-octre­o­tide may ­also be ­applied, main­ly to local­ize ­head and ­neck chem­o­dec­to­mas. Malignant par­a­gan­gli­o­mas of ­either adren­al or ­extra-adren­al ori­gin ­show a var­i­able nat­u­ral his­to­ry: ­from a local­ly inva­sive indo­lent ­tumor to a high­ly aggres­sive malig­nan­cy. Surgery ­with com­plete resec­tion or debulk­ing of the pri­mary ­tumor is the stan­dard treat­ment. External radio­ther­a­py and chem­o­ther­a­py are usu­al­ly scarce­ly effec­tive. An alter­na­tive treat­ment is 131I-­MIBG ther­a­py ­which is per­formed ­with ­high spe­cif­ic activ­ity 131I-­MIBG. Usually a stan­dard­ized ­dose rang­ing ­from 3.7 to 9.1 GBq of 131I-­MIBG is admin­is­tered by ­slow i.v. infu­sion. In ­advanced ­stage cas­es 131I-­MIBG ther­a­py ­aims at symp­tom pal­li­a­tion and ­tumor func­tion reduc­tion as ­well as at ­tumor ­arrest or ­tumor regres­sion. In ­these cas­es ­MIBG ther­a­py ­allows pro­longed sur­vi­val and ­good qual­ity of ­life. In ­less ­advanced cas­es the pur­pose of ­MIBG ther­a­py is to com­ple­ment sur­gery and to ­achieve the ­total erad­i­ca­tion of the ­tumor. Non func­tion­ing malig­nant par­a­gan­gli­o­ma can ­some ­time ­also con­cen­trate ­MIBG and can be treat­ed ­with ­high dos­es of the trac­er. 131I-­MIBG ther­a­py is a ­safe treat­ment and is usu­al­ly ­well tol­er­at­ed by the ­patient (­with rath­er low mye­lo­tox­ic­ity).