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Astroblastoma is one of the very unusual type of tumors, whose histogenesis has not been clarified. It occurs mainly among children or young adults. Astroblastoma is grossly well-demarcated, and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Perivascular pseudorosettes in astroblastoma have sho...
Citations
... " e name is believed to be associated with the assumption that astroblastomas originate from the astroblast, an intermediate of glioblasts and astrocytes. [8,14] However, more recently, astroblastomas have become more controversial due to the emergence and increasing use of molecular profiling of CNS tumors alongside traditional standalone histological diagnosis. is, in tandem with the rarity of astroblastoma, has resulted in a paucity of molecular information and increasing doubts surrounding historical diagnoses based purely on tumor morphology. ...
Background: Astroblastomas are uncommon neuroepithelial tumors of the central nervous system with a distinct, yet, controversial radiological, histological, and molecular profile. Debatable differences between low- and high-grade astroblastoma have been reported in the medical literature; indeed, despite the increasing relevance of molecular genetic profiling in the realm of astroblastoma, its application is still in its early stages. As a result, the diagnostic criteria for astroblastoma remain undecided with yet no real consensus on the most ideal management.
Case Description: This report describes a case of astroblastoma diagnosed 13 years ago in a young woman who despite six episodes of recurrence, transformation, and progression was able to retain a perfomace status of 0 by World Health Organization standard, throughout.
Conclusion: This report discusses the clinical, radiological, histological features, and management of this rare tumor with an extraordinarily long survival, with an aim to strengthen the literature on management options. To the best of our knowledge, this is the longest surviving case of anaplastic astroblastoma reported in the available medical literature.
Keywords: Astroblastoma, Histomolecular diagnosis, Long-term follow up, Neuro-oncology, Prognosis
... [2,3] Astroblastoma usually develops in cerebral hemispheres, but also in other parts of nervous system. [4] On imaging examination, astroblastomas are well-demarcated masses. [5] Although the microscopic description of astroblastoma in the existing literature is not completely consistent, there are 2 points that can be unified: the perivascular pseudorosette of tumor cells with short and stout cytoplasmic processes, radiating towards central blood vessels that often demonstrate sclerosis; and glial fibrillary acidic protein (GFAP)-positive expression. ...
Rationale:
Astroblastoma is a rare tumor of the central nervous system with uncertain biological behavior and origin. Its histopathological features have been well established, while, to our knowledge, astroblastoma with oligodendroglial-like cells have not been reported.
Patient concerns:
A 15-year-old girl presented with nausea, vomiting, headache, and visual disturbance.
Diagnosis:
Magnetic resonance imaging revealed a large neoplasm in the left temporal. Histologically, the tumor showed solid and pseudopapillary structure. Immunohistochemical staining showed that the tumor cells were positive for glial fibrillary acidic protein and vimentin. The oligodendroglial-like cells were positive for glial fibrillary acidic protein, vimentin, and oligodendrocyte transcription factor 2. The antigen KI67 labeling index was about 4%. Sequencing for isocitrate dehydrogenase (IDH) 1 codon 132 and IDH2 codon 172 gene mutations showed negative results. Furthermore, fluorescent analysis revealed neither 1p nor 19q deletion in the lesion. Based on these findings, the girl was finally diagnosed as astroblastoma.
Interventions:
A craniotomy with total excision of the tumor was performed.
Outcomes:
The follow-up time was 1 year, no evidence of disease recurrence was found in magnetic resonance imaging.
Lessons:
Cerebral astroblastoma with oligodendroglial-like cells is a clinically rare tumor of central nervous system. Clear distinction and diagnosis are critical.
... It can occur at any age, but is more common in children and young adults [2,3]. Astroblastoma usually develops in cerebral hemispheres, but is also found in other parts of the nervous system [4]. On imaging examination, astroblastomas are well-demarcated masses [5]. ...
Background: Astroblastoma is a rare tumor of the central nervous system, with unclear biological behavior and origin. Its histopathological features have been well established. However, to the best of our knowledge, astroblastoma with oligodendroglial-like cells have not been reported.
Case presentation: Herein, we reported a case of astroblastoma with atypical pathological features. A 15-year-old girl presented with nausea, vomiting, headache and visual disturbance. Magnetic resonance imaging (MRI) revealed a large neoplasm in the left temporal lobe. Histologically, the tumor showed solid and pseudopapillary structure. The tumor cells were radially arranged around blood vessels, with a single, prominent process, and as astroblastic pseudorosettes. More importantly, typical oligodendroglial-like cells were observed. In addition to membrane staining for EMA, immunohistochemical staining also showed that the tumor cells were positive for GFAP and Vimentin. The oligodendroglial-like cells were positive for GFAP, Vimentin, and Olig-2. The Ki-67 labeling index was about 4%. Sequencing for IDH1 codon 132 and IDH2 codon 172 gene mutations showed negative results. Furthermore, fluorescent analysis revealed neither 1p nor 19q deletion in the lesion. Based on these findings, the tumor was finally diagnosed as astroblastoma.
Conclusions: Herein, we reported an extremely rare case of astroblastoma, which was morphologically characterized by the appearance of oligodendroglial-like cells.
... ABs may show heterogeneous internal enhancement or rim enhancement on postcontrast T1-weighted images. There is usually a mild perifocal edema that usually does not correlate with the grade of the tumor [22,23]. Although all supratentorial cases discussing DWI had restricted diffusion, our case is the first one showing no restriction on imaging [16]. ...
A 4-year-old girl presented to the hospital with a progressive headache, difficulty walking, and persistent daily vomiting for 3 weeks. Papilledema was observed on fundoscopic examination. A large left cerebellovermian tumor with “bubbly” appearance was discovered. Total removal of the tumor mass was performed, and a diagnosis of low-grade astroblastoma was made. Adjuvant radiotherapy was performed due to the risk of recurrence. The patient is disease-free and has been kept on close follow-up for 6 months. The occurrence of posterior fossa astroblastoma has been rarely reported in the literature. Thus, when a “bubby” appearance enhancing cystic solid tumor is located on the cerebellar hemisphere in a child, an astroblastoma should also be included in the differential diagnosis.
... The tumor cells of astroblastoma were found to be an intermediate between astrocytes and ependymal cells as per an electron microscopy. Astroblastoma is believed to have originated from the astroblast which is an intermediate between glioblasts and astrocytes [7]. ...
Astroblastoma is an uncommon neuroepithelial primary tumor of the brain which is of uncertain origin. We present a case of high-grade astroblastoma in an 18-year-old female with a severe headache, loss of appetite, vomiting and generalized weakness. The patient had undergone a right frontoparietal craniotomy. Large subfalcine meningioma was excised. The lesion was suspected to be a meningioma. Primary radiological investigation revealed a 6.8 cm × 5.8 cm × 5.4 cm lesion. Although the radiological and intraoperative findings were of an extra-axial tumor, the histology and immunophenotype was of an astroblastoma. The patient was treated with cyclophosphamide, cisplatin and etoposide chemotherapy regimen. The patient was later treated with bi-weekly bevacizumab. The patient had improved symptomatically post-chemotherapy. However, there was no significant difference in lesion size. The patient died after 2 weeks. The prognosis of patients with astroblastoma is extremely poor as observed in our case.
... Specific CT and MRI features of meningioma such as tumor touching the inner surface of the skull or erosion of the inner table and diploe may be present also in case of an astroblastoma, as reported in the literature [30]. Microscopically, papillary meningioma is connected to the meninges and the perivascular pattern is similar to pseudorosettes of ependymoma [31]. Immunohistochemical features of glial markers make the difference between them. ...
Background:
Astroblastoma is a poorly defined central nervous system (CNS) tumor, included along with polar spongioblastoma and gliomatosis cerebri in the group of neuroepithelial tumors of uncertain origin in the June 2016 World Health Organization (WHO) Classification of tumors of the CNS. They are rare neoplasms that affect primarily patients of young ages. The purpose of this research is to highlight the uniqueness and rareness of this pathology and to emphasize on the particularities of one case managed in our Clinic.
Case description:
We present the case of a 54-year-old patient with a history of seizures since the age of six years old, who presented on admission with progressive worsening and unresponsiveness to treatment, starting six months prior to presentation. Brain imaging shows a right frontal mass compressing neighboring structures. Gross total resection of the tumor was performed, and histopathological examination of the surgical sample together with immunohistochemistry highlighted the presence of a low-grade astroblastoma.
Conclusions:
We summarized data from the literature in order to highlight aspects of this affliction: clinical presentation, imagery, surgical treatment and pathology, hoping that this will aid physicians in finding useful information on this subject, which can guide them to a good outcome. We also discussed differential diagnosis, as this type of tumor shares common features with ependymoma, meningioma, astrocytoma, etc.
... The histogenesis of anaplastic astroblastoma is controversial; however tanycytes, glial precursor cells, have been suggested as a potential tissue of origin [14][15][16]. The diagnosis requires a well-defined margin with the presence of perivascular pseudorosettes with thick and short, blunted tumor cells which do not taper as they project toward the central blood vessel [7,9,10,17,18]. The perivascular structures can be uniform or loosely scattered structures with round to oval nuclei and may exhibit chromatin aggregation [12]. Hyalinization and fibrotic vessel walls can be visible with occasional areas of infarcted brain tissue [10,19]. ...
... Astroblastomas show a slight female predominance and are often noted in the literature to be a pediatric tumor [4,5,7,[24][25][26] with congenital lesions also reported [17,18,[27][28][29]. In our review however we note many reports of this tumor presenting in adult patients and the incidence of this is well described [3-5, 7, 9, 16, 17, 24, 30-32]. ...
Astroblastomas are rare, potentially curable primary brain tumors which can be difficult to diagnose. We present the case of astroblastoma in a 73-year-old male, an atypical age for this tumor, more classically found in pediatric and young adult populations. Through our case and review of the literature, we note that this tumor is frequently reported in adult populations and the presentation of this tumor in the elderly is well described. This tumor is an important consideration in the differential diagnosis when managing both pediatric and adult patients of any age who present with the imaging findings characteristic of this rare tumor.
... 3) Rarefied spaces between pseudorosettes, 4) Absence of abundant fibrillary pattern, 5) Difference in nuclear characteristics (9,12,21,22,33) Pathological grading of astroblastoma is classified into low and high grade. The low-grade type includes astroblastomas with uniform perivascular arrangement of pseudorosettes, low to moderate number of mitotic figures, little cellular atypia, minimal or no vascular endothelial proliferation, and predominant sclerosis of the vascular walls. ...
... The low-grade type includes astroblastomas with uniform perivascular arrangement of pseudorosettes, low to moderate number of mitotic figures, little cellular atypia, minimal or no vascular endothelial proliferation, and predominant sclerosis of the vascular walls. High-grade astroblastomas show high cytological atypia, increased mitotic figures, compact cellularity, perivascular cells with high mitotic rates, and hypertrophy of vascular endothelium without hyalinization (5,12,21). This has prognostic value, as patients with a low-grade astroblastoma survive for a longer time than those with the anaplastic form. ...
... Gross total resection may result in long-term survival. An anaplastic histology has been associated with recurrence and progression, suggesting that more aggressive treatment, including radiotherapy, is necessary for high-grade lesions (5,7,21,25,29,34,38). Combination chemotherapy has had encouraging success regarding the prognosis (30). ...
... Astroblastoma generally develops in the cortex, subcortical area, and periventricular area of the cerebral hemispheres (15). It not develops in the ventricle itself (26) as present in our patient. ...
Astroblastoma is a rare distinct type of aggressive glial tumor which has a much confusion that arisen regarding criteria for their diagnosis. We introduce a case of astroblastoma and reviewing all available literature of astroblastoma aiming to discuss astroblastoma from the clinical, pathological, management, and prognostic points of view in a trial to discover more secrets of astroblastoma and to introduce a standard way of its diagnosis and management. The recurrence rate is 34% after surgical resection and radiotherapy with overall survival 2.4 years. Astroblastoma is a rare distinct type of aggressive glial tumours commonly presented in the frontal and parietal lobe. The recurrence rate is 34% after surgical resection and radiotherapy with overall survival 2.4 years.
... [3][4][5][6][7] Bell et al. reported their 12 tumors were all well demarcated from normal brain. 5 Despite gross total resection of circumscribed tumors as observed by neuroimaging, tumor recurred in some patients and/or the patients died [8][9][10][11][12] ; a few others survived without recurrence for 5 13 and 8 years. 14 We can fi nd a poorly demarcated astroblastoma 15 and a diffusely infi ltrating astroblastoma (case 1 by Hoag). ...
... 17 As to immunohistochemistry, at least some glial fi brillary acidic protein (GFAP)-positive cells exist in all reported tumors expressing GFAP. 8,[10][11][12][13][14][15][16][17][18][19][20][21] The interesting feature of the present case is that only the intervascular cells in the last surgical specimens lost GFAP expression. We identifi ed two reports stating that intervascular cells are occasionally GFAP negative. ...
... A few reports have investigated the expression of neuronal markers in the case of astroblastoma: three tumors were found to be neuron-specifi c enolase (NSE) positive; 10,12,24 one, NSE negative; 11 one, synaptophysin negative; 10 and one, neurofi lament negative. 25 In the present case, the tumor cells were positive for NSE, TUJ1, and nestin, and weakly and partially positive for synaptophysin. ...
A 17-year-old male patient underwent surgery five times (four consecutive intracranial tumor removal surgeries and a final spinal tumor removal surgery). After the third surgery, this case was reported as a low-grade astroblastoma that is characterized by perivascular pseudorosettes consisting of elongated tumor cells arranged around the blood vessels. However, the fourth and fifth surgical specimens demonstrated very interesting histological changes in the astroblastoma. Through the course of relapses, the constituent cells of the astroblastic perivascular rosettes became smaller and rounder, and a multilayered cell arrangement was observed. The nucleus-to-cytoplasm ratio increased, and the compact intervascular cells ultimately lost glial fibrillary acidic protein (GFAP) expression. These undifferentiated cells showed high MIB-1 indices and an increased olig2 index. On the other hand, the cells in all the surgical specimens were positive for certain neuronal markers such as NSE, TUJ1, and nestin. Some astroblastomas may be more immature than the usual astrocytes; however, it is necessary to study more cases to confirm this.
