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The Journal of Cardiovascular Surgery 2000 October;41(5):781-4

Copyright © 2009 EDIZIONI MINERVA MEDICA

lingua: Inglese

Huge malignant localized fibrous tumor of the pleura

Veronesi G., Spaggiari L., Mazzarol G. *, De Pas M. **, Leo F., Solli P., Pastorino U.

From the Department of Thoracic Surgery *Department of Pathology **Division of Medical Oncology European Institute of Oncology, Milan, Italy

Local­ized ­fibrous ­tumor is an unfre­quent mesen­chymal neo­plasm. The malig­nant var­iant of the ­pleura is excep­tional and dif­fe­ren­tial diag­nosis ­with the ­more fre­quent ­benign ­type or ­with ­other neo­plasms ­such as ­soft ­tissue sar­coma and mesoth­e­lioma is ­rarely pos­sible in a pre­op­er­a­tive set­ting. The ­best treat­ment of ­this dis­ease is rad­ical sur­gical resec­tion. No defin­i­tive ­data ­exist ­about the ­role of chem­o­therapy. We ­report a ­case of a ­giant ­right intra­tho­racic ­mass ­whose pre­op­er­a­tive diag­nosis, ­from an ­open ­biopsy, was con­sis­tent ­with sar­coma and, in a ­second ­review, ­with ­fibrous ­tumor of the ­pleura ­without any indi­ca­tion ­about malig­nancy. A ­right pleu­rop­neu­mo­nec­tomy and per­i­car­dial resec­tion was per­formed ­through a ­right hem­i­clam­shell ­approach. His­tology dem­on­strated an aggres­sive beha­viour: ­high ­mitosis ­rate, Ki 67 of 34% and dif­fuse ­necrosis ­were ­present. In con­sid­er­a­tion of the ­apparent ­local radi­cality we ­did not per­form any adju­vant treat­ment. Six ­months ­after the oper­a­tion a ­wide ­local recur­rence was evi­dent and a ­systemic treat­ment ­with Ifos­fa­mide and Adri­am­i­cina is ­still in ­progress. So far a ­good ­response has ­been doc­u­mented. Pre­op­er­a­tive diag­nosis of malig­nancy has an impor­tant ­role as a ther­a­peutic ­strategy in man­age­ment of ­fibrous ­tumours of the ­pleura. ­When ­there is sus­pi­cion of a malig­nant ­form neo­ad­ju­vant chem­o­therapy can rep­re­sent a fur­ther ­tool to con­trol ­poorly dif­fer­en­tiated and ­large ­tumors, and a ­wide sur­gical resec­tion of the ­lesion ­must be per­formed.