The clinical and pathological aspects of neurofibromatosis are summarized and an updated pathogeny of the tumor is made. The malignancy of the NFS senns to be linled to the NF - 1 gene mutations, with loss of the suppressor gene in the chromosone 17 pl. The surgical treatment is always useful because it lowers the pressure and relieves the patient.
Neurofibromatosis; Von Recklinghausen; Pelvic neurofibrosarcoma