PURPOSE: Cerebellar glioblastomas in children are rare. As a result, an optimal treatment strategy has not yet been identified. A review of the characteristics of the disease as well as the effectiveness of various therapeutic modalities would help in optimizing the treatment paradigm. METHODS: We performed a detailed clinical, radiographic, and pathologic retrospective review of five patients (three boys and two girls, average age at presentation 7.2 years (range, 3-14 years)) and surveyed the literature for an additional 55 cases. RESULTS: Computed tomography and magnetic resonance imaging usually revealed a large lesion with minimal edema, heterogeneous contrast enhancement, and a discrete border. Subtotal tumor resection was performed in two patients and gross total resection in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was variably positive. Adjuvant therapy included local radiation and chemotherapy in all followed patients. Tumor recurrence was seen in two patients. Patients were followed from 2 months to 3.5 years (mean, 12 months). Two patients were dead at last follow-up with a mean survival of 9.5 months. CONCLUSIONS: The prognosis for pediatric patients with cerebellar glioblastomas is dismal, even when compared to adult counterparts or other malignant posterior fossa tumors in children. Cerebellar glioblastomas have a tendency to recur and disseminate despite treatment with surgery, chemotherapy, and radiation. The poor outcomes seen with this tumor suggest that the optimal treatment strategy has yet to be elucidated and much work needs to be done.
PURPOSE:Cerebellar glioblastomas in children are rare. As a result, an optimal treatment strategy has not yet been identified. A review of the characteristics of the disease as well as the effectiveness of various therapeutic modalities would help in optimizing the treatment paradigm. METHODS: We performed a detailed clinical, radiographic, and pathologic retrospective review of five patients (three boys and two girls, average age at presentation 7.2 years (range, 3-14 years)) and surveyed the literature for an additional 55 cases. RESULTS: Computed tomography and magnetic resonance imaging usually revealed a large lesion with minimal edema, heterogeneous contrast enhancement, and a discrete border. Subtotal tumor resection was performed in two patients and gross total resection in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was variably positive. Adjuvant therapy included local radiation and chemotherapy in all followed patients. Tumor recurrence was seen in two patients. Patients were followed from 2 months to 3.5 years (mean, 12 months). Two patients were dead at last follow-up with a mean survival of 9.5 months. CONCLUSIONS: The prognosis for pediatric patients with cerebellar glioblastomas is dismal, even when compared to adult counterparts or other malignant posterior fossa tumors in children. Cerebellar glioblastomas have a tendency to recur and disseminate despite treatment with surgery, chemotherapy, and radiation. The poor outcomes seen with this tumor suggest that the optimal treatment strategy has yet to be elucidated and much work needs to be done.
Authors: Rami Hatoum; Jia-Shu Chen; Pascal Lavergne; Nathan A Shlobin; Andrew Wang; Lior M Elkaim; Philippe Dodin; Charles P Couturier; George M Ibrahim; Aria Fallah; Dominic Venne; Sebastien Perreault; Anthony C Wang; Nada Jabado; Roy W R Dudley; Alexander G Weil Journal: JAMA Netw Open Date: 2022-08-01