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

Copyright © 2000 EDIZIONI MINERVA MEDICA

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


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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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