Anaplastic Astroblastoma Presenting as Massive, Sudden-Onset, Intraparenchymal Hemorrhage

Abstract

Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular, often cystic enhancing mass. The existence of astroblastoma as a distinct clinicopathologic entity has long been debated but is recognized in the 2007 WHO classification of CNS tumors. The grading of these tumors remains unsettled. Currently, no grade has been established and there are no clearly defined diagnostic criteria for low- or high-grade tumors. Astroblastomas in general are thought to have a favorable outcome with low rates of recurrence following gross total resection. We report a case of astroblastoma with ‘high-grade/anaplastic’ histology in a 12-year-old, previously healthy girl. The patient experienced severe, sudden-onset headache, and quickly became comatose. Head CT showed a massive intraparenchymal hemorrhage in the right frontal lobe with surrounding edema. She underwent emergent posterior frontal craniotomy for decompression and hematoma evacuation. Pathologic examination revealed a sharply demarcated hemorrhagic heterogeneous glial tumor with focal papillary architecture, densely hyalinized blood vessels, and intensely GFAP-positive perivascular cells. The tumor showed unequivocal high-grade features including an elevated proliferative index. The diagnosis of anaplastic astroblastoma was rendered. The patient expired on postoperative day 30. This case illustrates the potential poor outcome of high-grade astroblastoma and highlights the morphologic heterogeneity of this rare neoplasm.

Pediatric Astroblastoma: A Rare Case with a Review of the Literature

“…Apart from our case, 29 patients less than 18 years of age with high-grade astroblastomas have been reported in the literature (table 1) [1,8,9,10,11,12,13,14,15,16,17]. One patient in a series by Brat et al [10] was not evaluable.…”

Cerebral astroblastoma in an adult: An immunohistochemical, ultrastructural and genetic study

“…In the present tumor, the frequent occurrence of tumor cells with a rhabdoid appearance was a feature of the malignant (high‐grade) area. This feature has been pointed out in a few astroblastomas reported previously . In CNS tumors, the occurrence of cells with rhabdoid morphology (rhabdoid cells) is a characteristic feature of atypical teratoid/rhabdoid tumor (AT/RT), in which the absence of nuclear immunoreactivity for INI1 protein is important for establishing the diagnosis .…”

“…This feature has been pointed out in a few astroblastomas reported previously. [31][32][33] In CNS tumors, the occurrence of cells with rhabdoid morphology (rhabdoid cells) is a characteristic feature of atypical teratoid/rhabdoid tumor (AT/RT), in which the absence of nuclear immunoreactivity for INI1 protein is important for establishing the diagnosis. 34 In the present tumor, nuclear expression of INI1 protein was evident in most of the tumor cells, including those with a rhabdoid appearance.…”

Astroblastoma in a Young Female Patient: A Case Report and Literature Review of Clinicopathological, Radiological and Prognostic Characteristics and Current Treatment Strategies

“…In general, astroblastomas are large, peripheral and solid (with occasional cystic component) brain tumors. Typical clinical manifestation of these tumors includes headache, seizures, nausea, vomiting, and decay in conscious level [ 4 5 6 ]. The characteristic radiological appearance of astroblastoma more likely illustrate heterogeneous hyperintense signal on T2-wieghted sequences (T2WS), and fluid-attenuated inversion recovery (FLAIR) images, hypointense to isointense on T1-weighted sequences (T1WS), with characteristic “bubbly” appearance.…”

“…Tumor associated vasogenic edema may also be present [ 5 7 8 9 10 ]. The histopathological features of astroblastoma include perivascular arrangement of neoplastic cells having thick, stout and short cytoplasmic processes with peripheral nuclei, forming pseudorosettes giving the characteristic “cartwheel” appearance [ 4 5 6 11 12 13 14 ].…”