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The Journal of Cardiovascular Surgery 1999 October;40(5):691-4

lingua: Inglese

Sur­gical treat­ment of par­a­gan­gli­omas of the ­carotid body and ­other ­rare local­isa­tions

Marchesi M., Biffoni M., Jaus M. O., Nobili Benedetti R., Tromba L., Berni A., Campana F. P.

From the Depart­ment of Sur­gery Uni­ver­sity “La ­Sapienza”, ­Rome, ­Italy


Back­ground. Cer­vical par­a­gan­gli­omas are ­uncommon ­benign or malig­nant neo­plasms, ­deriving ­from ­stem ­cells of the ­neural ­crest. Com­pared to all the ­tumors of the ­head and ­neck, ­they ­occur ­less fre­quently. ­They can be ­found in any ­part of the ­body ­where ­there are sym­pa­thetic gan­glia ­including chem­o­re­cep­tors, supra-r­enal ­medulla, ret­ro­per­i­to­neal gan­glia and the ­extreme ­branches of the ­vagus ­nerves. It is not ­easy now­a­days to ­define prop­erly ­their bio­log­ical beha­viour, the pos­sible mul­ti­cen­tric loca­tion and the asso­ci­a­tion ­with Mul­tiple Endo­crine Neo­plasms; ­this is con­sid­ered par­tic­u­larly impor­tant and ­occurs in 42 per ­cent of the ­cases of ­familial neo­plasms of the par­a­gang­lion ­system.
­Methods. ­After a ­review of ­recent diag­nostic, path­o­log­ical and clin­ical find­ings, the ­authors ­report ­their expe­ri­ence, ­between 1970-1995, of 10 ­patients ­affected by spo­radic par­a­gan­glioma and 1 by ­familial mul­ti­cen­tric neo­plasm loc­al­ised in the ­carotid ­bodies of ­both ­sides, ­left ­vagus ­nerve and ­left hypo­glossus ­nerve. All ­patients but one ­were ­treated by a cura­tive resec­tion of the neo­plasm. In one ­case ­only an explo­ra­tive lap­a­ratomy was pos­sible ­because of vis­ceral and vas­cular involve­ment.
­Results. ­There is no mor­tality. ­There are no mod­ifi­ca­tions in arte­rial ­blood pres­sure and cat­e­chol­a­mine ­values in all ­patients. The com­pli­ca­tions ­were a recur­ren­tial ­palsy in a ­patient oper­ated on for ­vagal par­a­gan­glioma; a recur­ren­tial ­palsy and tem­po­rary dys­ar­thria in the ­patient ­affected by mul­tiple ­familial par­a­gan­glioma; ­another ­patient oper­ated on for ­carotid ­body par­a­gan­glioma ­showed a cere­bral ­ischemic ­lesion ­which ­caused a ­slightly tran­si­tory ­facial-bra­chial ­motor def­icit on the ­right ­side and ­speech impair­ment.
Con­clu­sions. We can ven­ture to say ­that any ­type of cer­vical, med­i­as­tinal or ret­ro­per­i­to­neal ­swelling in per­sons ­belonging to a genet­i­cally ­prone ­family ­must be ­first of all con­sid­ered a pos­sible par­a­gan­glioma. For ­this ­reason the ­patient ­with ­more ­than one ­growth of ­this ­type, ­whether syn­chro­nous or not, ­must ­undergo ­genetic inves­ti­ga­tion, ­along ­with the ­rest of his ­family.

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