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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


FULL TEXT  


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).

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