Cerebral astroblastoma: analysis of six cases and critical review of treatment options

Astroblastoma is one of the rarest tumors of the central nervous system (CNS), and its classification, histogenesis, diagnosis and therapeutic management are still being debated. The typical histopathological appearance is the perivascular, astroblastic pseudorosette, which is however present in other CNS tumors. To clarify the clinical, radiological, histopathological, prognostic and therapeutic characteristics, which have been treated only recently and are not well established yet due to the rarity of this tumor, six cases of histologically proven astroblastoma were retrospectively analyzed in light of more pertinent literature and paying special attention to therapeutic remarks. Between 1996 and 2005, six patients with cerebral astroblastoma were surgically treated at the Department of Neurosciences-Neurosurgery of Sapienza University in Rome. In three cases the lesion was termed low-grade astroblastoma, and high grade in the other three, according to current standard parameters. Median age of the six patients was 36 years. The time to diagnosis ranged from 1 week to 18 months. The radiological and anatomopathological features of this lesion are described. Surgical removal was total in four cases and subtotal in two. All patients received radiotherapy: two also had chemotherapy with temozolomide (TMZ). The three patients with low-grade astroblastoma are still alive today after a follow-up of 2, 5 and 19 years, respectively. Of the three patients with high-grade lesions, one is still alive after a 7-year follow-up, while the other two survived for 17 months (progression time 15 months) and 35 months (progression-reoperation time 23 months), respectively. Conclusions radical surgical resection is the treatment of choice for astroblastomas. Radiotherapy may play an adjuvant role in the treatment of high-grade lesions. The role of chemotherapy is still very debatable. We propose an aggressive standardized treatment for those lesions that meet anaplastic criteria, owing to their postulated glial origin and the propensity to have aggressive courses, and we advocate the use of a safe adjuvant chemotherapeutic regimen with TMZ, used concomitantly and subsequently to radiotherapy, especially for the high-grade astroblastoma cases. Multicenter studies, taking into account molecular biological findings, are necessary to define a common therapeutic strategy for astroblastomas.