Population based analysis ependymoma patients in Alberta from 1975 to 2007.

BACKGROUND: Ependymomas are rare tumors of the central nervous system whose management is controversial. This population-based study of adults and children with ependymoma aims to (1) identify clinical and treatment-related factors that impact survival and (2) determine if postoperative radiotherapy (RT) can improve survival of patients with subtotal resection (STR) to levels similar to patients who had gross total resection (GTR). METHODS: This retrospective population-based study evaluated 158 patients with ependymoma diagnosed between 1975-2007 in Alberta, Canada. RESULTS: Younger patients (<7 years of age) were more likely to be diagnosed with grade III tumors compared with adults in whom grade I tumors were more common (p=0.003). Adults were more likely to have spinally located tumors compared to young children whose tumors were typically found in the brain. Overall, young children with ependymoma were more likely to die than older children or adults (p=0.001). An equivalent number of patients underwent GTR as compared with STR (48% vs 45%, respectively). Overall, older age, spinal tumor location, lower grade, and GTR were associated with improved progression free survival but only GTR was associated with significant improvement in overall survival. Median survival after STR and RT was 82 months compared with 122 months in patients who had GTR (p=0.0022). CONCLUSIONS: This is the first Canadian population-based analysis of patients with ependymoma including adults and children. Extent of resection appears to be the most important factor determining overall survival. Importantly, the addition of RT to patients initially treated with STR does not improve survival to levels similar to patients receiving GTR.

Extended adjuvant temozolomide for treatment of newly diagnosed glioblastoma multiforme.

The standard of care for newly diagnosed glioblastoma multiforme (GBM) is temozolomide (TMZ) chemotherapy given concurrently with radiation for 6 weeks followed by 6 months of adjuvant TMZ. Originally, patients in Alberta were treated with only six cycles of adjuvant TMZ regardless of clinical status but institutional policy was amended to allow up to 12 cycles of adjuvant therapy for patients experiencing at least stable disease and minimal toxicity. We conducted a population-based analysis to determine if extended adjuvant TMZ treatment (i.e., more than six cycles) confers a survival advantage as compared to the standard six cycles for newly diagnosed GBM patients. Patient data was collected from the Alberta Cancer Registry and patient charts. Progression free--and overall survival was determined in patients receiving six cycles of adjuvant TMZ and compared with that of patients receiving more than six cycles. Patients in whom adjuvant chemotherapy was stopped at cycle six experienced a median survival of 16.5 months, whereas, those who received more than six cycles survived for 24.6 months (p = 0.031). Extended adjuvant therapy was not associated with increased toxicity. In multivariate analysis, adjuvant monthly Temozolomide for more than six cycles was an independent prognostic factor for both progression free--and overall survival. These data suggest extended adjuvant temozolomide (i.e., more than six cycles) should be considered in patients with newly diagnosed GBM.

Plasma cell tumors of the skull.

BACKGROUND: Plasma cell tumors are rare neoplasms, which include extramedullary plasmacytoma, solitary plasmacytoma of the bone, and multiple myeloma. Although indistinguishable histopathologically, these entities need to be differentiated as treatment and prognosis vary. METHODS: This study was conducted by retrospective chart review and correspondence with patients diagnosed to have plasma cell tumors of the skull from 1992 to 2004. The clinico-diagnostic investigations, treatment, and subsequent follow-up of the patients were obtained. RESULTS: We report 5 cases of plasma cell tumors of the skull. The lesion involved the base of skull in 2 and the calvarium in 3 patients. In 2 cases, the histopathology was reported as plasmacytoma and further investigations revealed systemic involvement, indicative of multiple myeloma. In 1 patient, the biopsy was reported as multiple myeloma. The above 3 patients underwent radiation, followed by chemotherapy, and there was improvement in their neurologic status at subsequent follow-up. Another patient with a scalp swelling and symptoms of systemic involvement underwent investigations to detect multiple myeloma and, hence, was not subjected to an invasive procedure for histopathologic diagnosis. Chemotherapy was initiated but was discontinued because of social reasons. The last patient in our series died in the immediate postoperative period and although her histopathology was reported as plasmacytoma, she could not be evaluated for systemic involvement. CONCLUSION: The treatment options are different for the systemic form of disease and the localized disease; hence, it is imperative that a rigorous search for systemic involvement is conducted in a case of solitary or extramedullary plasmacytoma.