[Neurofibrosarcoma of the infra-orbital branches of the superior maxillary nerve].
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A patient with neurofibromatosis developed a large inoperable malignant schwannoma on the posterior neck. The tumor underwent complete local regression following combined-modality treatment with radiotherapy, vinblastine, and doxorubicin. Vinblastine may be effective in combined-modality therapy.
We present a type 1 neurofibromatosis case with sarcomatous degeneration of a cranio-orbital neurofibroma, its surgical evolution and the cytogenetics and cellular proliferative potential features (CMF, Ki-67 antibody).
This case report is about a relapsing conjunctive sarcoma affecting a 67 year old woman, involved with Von Recklinghausen's neurofibromatosis. Surgical orbital exenteration was necessary for curative treatment and reconstruction used a temporal muscle flap. Through this report, emphasis is placed on diagnosis difficulties and possibility of serious development concerning these malignant tumors in the scope of neurofibromatosis.
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