RESUMO
Se presenta un caso de tumor sacro voluminoso con características de Tumor Maligno de Vaina de Nervio Periférico (MPNST) que tras una resección inicial y recidiva se reintervino y trató con radioterapia complementaria. Tras ocho años se ha observado una buena evolución.
A case of voluminous sacral tumor with Malignant Peripheral Nerve Sheath Tumor (MPNST) characteristics is reported, that after a first resection and later recurrence is reoperated and treated with complementary radiotherapy. After eight years a good evolution has been observed.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/diagnóstico , Neoplasias de Bainha Neural/radioterapia , Sacro/patologia , Espectroscopia de Ressonância Magnética , SarcomaRESUMO
A malignant peripheral nerve sheath tumour (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. It is even more infrequent for these tumours to affect cranial nerves. We report the case of a 53-year-old man who presented a MPNST involving the infra-orbital nerve, which extended through the orbit and the base of the skull, progressing intracranially. Histological studies identified the tumour as an MPNST. Response to radiotherapy was not complete and radical surgical resection was impossible, so the patient died 10months later. This rare case of MPNST with intracranial involvement illustrates the dismal prognosis for patients with these lesions. Prognosis is poor because of the difficulty of performing radical surgery with free margins in these locations.
