Pleomorphic xanthoastrocytoma

Giannini, Caterina; Scheithauer, Bernd W.; Burger, Peter C.; Brat, Daniel J.; Wollan, Peter C.; Lach, B.; O’Neill, Brian Patrick

doi:10.1002/(sici)1097-0142(19990501)85:9%3c2033::aid-cncr22%3e3.0.co;2-z

Cited by 6 publications

(3 citation statements)

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“…Histopathologically, it is composed of pleomorphic, spindle‐shaped and foamy cells with abundant reticulin fibers surrounding individual tumor cells. Pilocytic astrocytoma rarely shows malignant transformation, 27,28 while PXA more often does 29–32 . Features such as a thalamic origin, reticulin‐poor stroma and RF‐like cytoplasmic granules are not typical in PXA.…”

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

Yokoo

Kamiya

Sasaki

et al. 2001

Pathology International

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Patients with neurofibromatosis type 1 (NF1) often have gliomas as a complication, most of which are benign pilocytic astrocytomas which have arisen in optic pathways. In the present case, a 17-year-old girl (at death) with stigmata of NF1, initially had a bulky tumor mass in the left thalamus, developing into the lateral ventricle, at 13 years of age. Partially resected tissue samples showed pleomorphic astrocytoma with abundant xanthoma cells and degenerative structures such as Rosenthal fibers (RF) and eosinophilic granular bodies. Fine eosinophilic granules identical to RF, both immunophenotypically and ultrastructurally, were also seen. The residual tumor was subtotally resected 6 months later, and the tumor histology was essentially similar as before, accompanying the regenerative structures; this was believed to be a good prognostic indicator. However, several anaplastic features such as mitosis, necrosis and vascular proliferation appeared even in areas rich in the regenerative structures. After a 2-year, disease-free interval, multiple tumor relapse occurred in June 1997. Partially resected tumor tissues were composed of monotonous small anaplastic cells with prominent proliferative activity. Surprisingly, the tumor cells had retained eosinophilic granules within the cell bodies. Postoperative chemotherapy with procarbazine, MCNU and vincristine (PCV) suppressed the residual tumor dramatically, but the regrowing tumor finally became uncontrollable, leading to the patient's death. TP53 mutation was not detected, while p27 immunopositivity was constantly high during malignant progression, suggesting acquisition of proliferative activity to overcome p53 and p27 inhibitory functions. A review of previously published reports failed to reveal any cases of this type.

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Section: Discussionmentioning

confidence: 99%

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

Yokoo

Kamiya

Sasaki

et al. 2001

Pathology International

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“…Giannini C et al in their study concluded that PXA was commoner in younger age group while as GBM was more frequent in older age group. 12 On histological subtyping GBM was commonest tumor in temporal lobe (17.4%) followed by PXA and oligodendroglioma (14.6%) each. In 2014 Shrestha et al conducted a prospective study on 60 patients in which they observed GBM was the most common tumor.…”

Section: Discussionmentioning

confidence: 95%

Intraoperative squash cytology and histopathological correlation of primary temporal lobe lesions: a 6 year study at tertiary care centre of Kashmir, India

Lone¹,

Jeelani²,

Rashid³

et al. 2018

Int J Res Med Sci

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Background: Central nervous system (CNS) tumors constitute about 1-2% of all the tumors. They are the 6th most common tumors in adults and 2nd most common among childhood tumors. Gliomas are the most common CNS neoplasms. In addition to gliomas temporal lobe has predilection for some peculiar tumor subtypes having good prognosis, including pleomorphic xanthoastrocytoma (PXA), low grade astrocytic tumors and dysembryoplastic neuroepithelial tumor (DNET) in children.Methods: Study was conducted in the department of pathology Sher-e-Kashmir institute of medical sciences (SKIMS) Soura, Srinagar Kashmir India. This was six-year study.Results: Temporal lobe tumors were commonly seen in 3rd to 4th decade of life accounting for 37.9% of the cases followed by 4th to 5th decade (27.5%). The mean age of presentation being 40±10 yrs. Males outnumbered females with a male: female ratio of 1.7:1. Intraoperative squash smears were mostly reported as High grade gliomas (WHO Grade III-IV) accounting to a total of 42 cases (36.2%). On histopathology frequent temporal lobe tumor seen was Glioblastoma multiforme (GBM) 17.4%, followed by pleomorphic xanthoastrocytoma and oligodendroglioma accounting for 14.6% of cases each. On comparing the diagnostic accuracy of intraoperative squash smears to that of Histopathological diagnosis an overall accuracy of 86.2% was observed.Conclusions: Intra operative squash technique in CNS tumors is universally accepted method. In temporal lobe lesions, on adequate sampling and careful examination, squash cytology has fairly good diagnostic accuracy rate of 86.2% when compared to histopathology.

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“…The proportion of PXA is less than 1% among all astrocytomas, and it is mostly seen in children and adolescents, with a median age of onset of 22 years old. 1 PXA was first described in 1979 as a tumor with a relatively good prognosis in young patients. 2 While the tumor can develop in different regions across the brain, it usually originates in the temporal lobe and induces epilepsy.…”

Section: Introductionmentioning

confidence: 99%

<p>Anaplastic Pleomorphic Xanthoastrocytoma: A Case Report and Literature Review</p>

Liu¹,

Sun²,

Liu³

2020

IJGM

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Background With an incidence of less than 1% among astrocytomas, pleomorphic xanthoastrocytoma (PXA) is rare. When its mitotic activity exceeds 5 mitoses/10 high-power fields, PXA is defined as anaplastic pleomorphic xanthoastrocytoma (APXA). This report documents the clinical manifestations and histopathological characteristics of APXA to help prevent future misdiagnoses. Case Presentation A 28-year-old male patient had a sudden limb twitch and visited a local hospital. A head magnetic resonance imaging scan showed large patches of abnormal signal intensity that were approximately 6.0×3.3 cm in size in the right frontal and parietal lobes, with iso- to slightly hypointense signals on T1-weighted images (T1WI) and mixed hyperintense signals on T2-weighted images (T2WI). Optical microscopic imaging found pleomorphic tumor cells with sheet-like growth, as well as foamy tumor cells, multinucleated giant cells, pleomorphic cells with atypical nuclei, and acidophilic bodies. Some areas were densely packed with obvious atypia and visible mitoses. The patient tested positive for glial fibrillary acidic protein (GFAP), vimentin (Vim), neuronal nuclear antigen (NeuN), P53, oligodendrocyte transcription factor-2 (OLIG-2), and ATRX, while he tested negative for synaptophysin (Syn), CD34, S-100, BRAF V600E, and IDH1 R132H. The Ki-67 labeling index was 15%. Genetic sequencing showed that IDH1 and IDH2 genes were wild-type, but that his BRAF gene harbored the V600E mutation. Conclusion APXA is a WHO grade III astrocytoma that can be distinguished from WHO grade II PXA according to the level of mitosis. Imaging may help to inform the difficult differentiation between APXA and epithelioid glioblastoma. Nonetheless, a clear diagnosis warrants carrying out a comprehensive analysis, including histomorphological, immunophenotypic, and molecular assessments.

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Pleomorphic xanthoastrocytoma

Cited by 6 publications

References 0 publications

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

Intraoperative squash cytology and histopathological correlation of primary temporal lobe lesions: a 6 year study at tertiary care centre of Kashmir, India

<p>Anaplastic Pleomorphic Xanthoastrocytoma: A Case Report and Literature Review</p>

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