Although the histogenesis of meningeal hemangiopericytomas (HMP) remains controversial, both biological and clinical evidence seems to identify these neoplasms as a separate entity with respect to meningiomas. In order to assess the current prognosis of HMP we reviewed our personal experience limited to the last decade (1986-1995): during this period 7 patients (4M, 3F) were treated by surgery alone or surgery combined with postoperative radiotherapy. In spite of meticulous attempts at radical resection, the tumors recurred in all but two cases with a mean interval of 85 months, and a total of 18 operations were performed (2.57/patient; range 1-4). Massive intratumoral hemorrhage determined acute deterioration and required emergency surgery in two cases while in one patient diffuse visceral metastases were discovered at autopsy. Five patients are still alive at follow-up but only 2 of them are in good neurological conditions and without evidence of disease. These results are similar to those reported in other series. In view of our results we conclude that intracranial hemangiopericytomas still have a dismal prognosis. Advances in neuroimaging, neuroanesthesia, microneurosurgery and adjuvant therapy do not seem to have significantly affected the recurrence rate, quality of life and mortality.
The isolated retroperitoneal malignant schwannoma unassociated with Von Recklinghausen's disease is an unusual neoplasm, representing 0.01% of all retroperitoneal malignant neoplasms, with a poor prognosis, and an average survival at 5 years of 50% in patients treated by radical exeresis. At present, it is impossible, without histologic and immunohistochemical examinations, to differentiate it from other isolated retroperitoneal sarcomatous neoplasms. The authors report a case of retroperitoneal malignant schwannoma 20 cm in diameter in a 62-year-old woman surgically treated by radical exeresis. Postoperative complications were absent, and the patient, discharged from the hospital on the 12th postoperative day, died 8 months later of diffuse metastases, without local relapse. Despite the patient's short survival, the authors believe radical surgery to be the best therapeutic choice. Only surgery can establish a final diagnosis and can offer the best chance of survival and a significant and sometimes prolonged relief of symptomatology.
The authors report a case of spinal subarachnoid hemorrhage caused by neurofibromas of the cauda equina. An examination of the literature revealing 13 similar cases and an analysis of this case showing three neurofibromas of the cauda equina prompted an hypothesis emphasizing the origin of bleeding and the difficulties associated with a correct diagnosis.
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