Neurofibrossarcoma associado a neurofibromatose

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.

Neurofibrossarcoma retroperitoneal primário

Malignant schwannoma or neurofibrosarcoma usually grows from the peripheral nerves and rarely from the retroperitoneum. When found in the retroperitoneum, it usually has a worse prognosis. This kind of tumor seems to be resistant to adjuvant therapy and the best treatment is radical surgery. We report a case of a primary retroperitoneal malignant schwannoma, in a 64 year-old man, treated surgically.