Clinical features and post-surgical outcome of patients with astroblastoma

Department of Neurological Surgery, University of California, San Francisco, California 94117, USA.
Journal of Clinical Neuroscience (Impact Factor: 1.38). 06/2011; 18(6):750-4. DOI: 10.1016/j.jocn.2010.11.007
Source: PubMed


Astroblastoma is a rare tumor, and thus experience with these lesions is very limited. The prognosis and appropriate treatment is not well understood, as few individual centers have enough experience with astroblastoma to guide treatment recommendations. We performed a systematic comprehensive search of the published English language literature on patients undergoing surgery for astroblastoma to summarize what is known about these tumors, and to provide some framework for future efforts in this area. A total of 62 references met our inclusion criteria, and contained individual patient data on 116 patients with astroblastoma. Determination of overall survival rates was performed using Kaplan-Meier analysis. This analysis suggests that the distribution is bimodal, with a prominent peak in young adulthood. Astroblastomas are generally amenable to complete tumor resection, even when very large, with gross total resection (GTR) achieved in 71/85 (84%) of reported patients, including both 9cm tumors reported. Patients undergoing GTR experienced a significant improvement in survival compared to patients who underwent subtotal resection (STR) (5-year progression-free survival: GTR 83% versus [vs.] STR 55%, log rank p=0.011). Although patients receiving external beam radiotherapy or fractionated three-dimensional conformal radiotherapy (XRT) seemed to have lower survival rates, this was not statistically significant (5-year survival: GTR 94% vs. GTR+XRT 73%, log rank p=0.463). Thus, we have reported the results of a summary of the literature on astroblastomas and have accurately described outcome characteristics using a data set that would be difficult to accumulate at a single center treating this tumor.

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    • "It is now, however, considered as a definite glioma entity of astrocytic origin [3, 4]. This tumor is mainly located to the cerebral hemispheres and affects mostly children, adolescents and young adults, however, congenital forms and astroblastomas in patients above 50 years of age have been reported as well [5, 6, 7, 8, 9, 10, 11, 12, 13]. "
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    ABSTRACT: Introduction: Astroblastoma is a rare glial tumor of uncertain origin affecting mostly children, adolescents and young adults. Given the rarity and the definitional problems concerning this tumor entity, the prognosis and appropriate treatment are at this point unclear. Case report: A 50-yearold Caucasian female presented with a seizure. Radiological findings showed a welldefined circumscribed tumor located in the right cerebral frontal lobe. The patient underwent primary surgery followed by postoperative radiotherapy. After 6 months the tumor recurred with multiple small lesions not available for surgery. Chemotherapy was administered with complete radiological response. Seven years after surgery and more than 6 years after completed chemotherapy the patient is free of disease. Histopathology revealed a gliomatous tumor with gemistocyte-like tumor cells arranged in palisades or strings and areas with perivascular pseudorosettes, consistent with astroblastoma. Immunophenotype and ultrastructural findings confirmed the diagnosis and verified the neuroepithelial origin. Conclusion: Astroblastomas are rare brain tumors and pose a challenge in the diagnostic and clinical approach. In general, they have an unpredictable course with a tendency of recurrence. This and other case reports support a survival benefit of chemotherapy, suggesting this as an important treatment option for these patients.
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    ABSTRACT: Brain tumors are the most common solid cancers of childhood. The subject of this chapter are those tumors which have been typically classified as “other” or that remain “unclassified” due to the relative rarity of each tumor type.
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    ABSTRACT: Astroblastoma is a rare glial tumor of unknown origin, usually affecting the cerebral hemispheres of children and young adults. Here we report an unusual cerebral tumor in a 60-year-old woman. On MRI, the tumor appeared as a well circumscribed lesion in the left frontal lobe. Histopathologically, it was composed of rounded eosinophilic cells, and was divisible into two areas. One area was characterized by a collection of GFAP-positive cells around sclerotic blood vessels (astroblastic pseudorosettes and perivascular hyalinization), and had a Ki-67 labeling index of 2.8%. However, the other area was highly cellular, showing many GFAP-negative cells often with a rhabdoid appearance, mitoses and a Ki-67 index of 15.7%. Thus, a final diagnosis of malignant astroblastoma was made. In both areas of the tumor, nearly all the cells were positive for epithelial membrane antigen, and many were positive for oligodendrocyte transcription factor 2 (Olig2). Focal expression of cytokeratin was also evident. With regard to genetic markers, the tumor cells were positive for INI1 and negative for mutant IDH1. The p53 labeling index was <1%. Ultrastructurally, the presence of intra- and intercellular lumina with microvilli was a feature. DNA examination of IDH1/2 and TP53 showed no mutations. In conclusion, although ependymal features were evident ultrastructurally in the present tumor, the immunohistochemical expression pattern of Olig2 was that of diffuse astrocytoma. On the other hand, the absence of mutations in both IDH1/2 and TP53 suggested that the present tumor was not a purely astrocytic neoplasm. Further studies, including molecular and genetic analyses, will provide insight into the histogenesis of astroblastoma.
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