✓ Primary meningeal osteosarcomas are rare tumors, with only 19 reported cases in the literature; only 4 of these, including the present case, are in pediatric patients. In this report, the authors present the case of an 8-year-old boy with a history of generalized tonic–clonic seizures who was found to harbor a meningeal osteosarcoma within the sylvian fissure. Initial working diagnoses included meningioma and glioma. After tumor enlargement and progressive symptoms, the patient underwent a large frontotemporal craniotomy and complete resection of the lesion, which recurred 6 and 12 months after the initial surgery and was surgically treated after each recurrence. The rarity of primary meningeal osteosarcomas can make their diagnosis difficult, and histopathological evaluation is mandatory for diagnosis. Because of their fast progression, they must be treated aggressively by means of surgery, chemotherapy, and radiotherapy.
Adnan Dagcinar, Fatih Bayrakli, Ozlem Yapicier and Memet Ozek
Özlem Kurtkaya-Yapicier, Bernd W. Scheithauer, Karl Peter Van Peteghem and John E. Sawicki
✓ An unusual case of a sacral, extradural choroid plexus papilloma involving the S1–3 level is described. This 50-year-old woman presented with a 4-month history of pain involving her right buttock, perineum, and leg. Contrast-enhanced magnetic resonance (MR) imaging of the spine revealed a well-defined, mildly enhancing sacral canal mass at the S1–3 level; its appearance was consistent with that of a benign tumor. Intraoperatively, the lesion was found to be extradural in location and was entwined among nerve roots in the sacral canal. Microscopic examination of the gross totally resected tumor revealed typical features of a choroid plexus papilloma. Despite performing a thorough neuroimaging workup (craniospinal contrast-enhanced MR imaging) for an intracranial or spinal primary mass, none was found. The choroid plexus appeared entirely normal; however, both a cavum septum pellucidum and a cavum vergae were noted. Extraneural choroid plexus papilloma, specifically intrasacral, extradural choroid plexus papilloma has not been previously reported. The present example is thought to have arisen either from ectopic choroid plexus tissue or perhaps by metaplasia from ependymal rests.
Robert D. Ecker, W. Richard Marsh, Bruce E. Pollock, Özlem Kurtkaya-Yapicier, Robyn McClelland, Bernd W. Scheithauer and Jan C. Buckner
Object. The authors reviewed the Mayo Clinic experience with the treatment of hemangiopericytoma in the primary central nervous system (CNS).
Methods. A retrospective study of all patients at the Mayo Clinic revealed 38 who had been treated for hemangiopericytoma in the CNS. Twenty of these patients were diagnosed in the decade between 1990 and 2000; 18 were initially diagnosed and underwent surgery before 1990.
In the patients treated since 1990, the 5-year Kaplan—Meier survival rate was 93%. The 5-year disease-free survival rate was 89%. Sixty percent of patients treated with the aid of stereotactic radiosurgery for recurrent disease were alive 4.4 years after their initial treatment. Salvage chemotherapy was not effective. No survival benefit was detected in patients who had received initial adjuvant external-beam radiation therapy. High-grade tumors recurred 6.7 years earlier than did low-grade lesions (p = 0.004).
Conclusions. The 5-year survival rate in patients with hemangiopericytoma of the CNS has improved at the authors' institution during the last 10 years. Although the reason for this is not entirely clear, the authors suspect that the improved treatment of patients with cancer, a 0% intraoperative mortality rate, and the use of radiosurgery in the treatment of recurrent disease all likely contribute. High-grade tumors recurred statistically significantly earlier than low-grade lesions. Current chemotherapies are ineffective in the treatment of hemangiopericytoma of the CNS.