Primary anaplastic pleomorphic xanthoastrocytoma in adults. Case report and review of literature

Choudry, Usama Khalid; Khan, Saad Akhtar; Qureshi, Amjad; Bari, Ehsan

doi:10.1016/j.ijscr.2016.08.022

Cited by 22 publications

(16 citation statements)

References 21 publications

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“…In fact, the existing literature includes both pediatric and adult cases where APXA was either challenging to diagnose or misdiagnosed as either low-grade tumors or WHO grade 4 gliomas. 3 , 12 , 18 , 24–26 …”

Section: Discussionmentioning

confidence: 99%

A case series of pediatric survivors of anaplastic pleomorphic xanthoastrocytoma

Ronsley

Dunham

Yip

et al. 2021

Neuro-Oncology Advances

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Background Anaplastic pleomorphic xanthoastrocytoma (APXA) is a rare subtype of CNS astrocytoma. They are generally treated as high-grade gliomas; however, uncertainty exists regarding the optimal therapy. Here, we report on 3 pediatric cases of APXA. Methods Our institutional database was queried for cases of APXA and 3 cases were identified. Surgical samples were processed for methylation profiling and chromosomal microarray analysis. Methylation data were uploaded to the online CNS tumor classifier to determine methylation-based diagnoses to determine copy number variations (CNVs). Results Two patients were male, 1 female, and all were aged 12 years at diagnosis. All underwent a gross total resection (GTR) and were diagnosed with an APXA. Immunohistochemical analysis demonstrated that 2 cases were BRAF V600E positive. Methylation-based tumor classification supported the APXA diagnosis in all cases. CNV analyses revealed homozygous CKDN2A deletions in all and chromosome 9p loss in 2 cases. All patients received radiation therapy (54 Gy in 30 fractions) with concurrent temozolomide. Two patients received maintenance chemotherapy with temozolomide and lomustine for 6 cycles as per the Children’s Oncology Group ACNS0423. The third patient recurred and went on to receive a second GTR and 6 cycles of lomustine, vincristine, and procarbazine. All are alive with no evidence of disease >4 years post-treatment completion (overall survival = 100%, event free survival = 67%). Conclusions The natural history and optimal treatment of this rare pediatric tumor are not well understood. This case series supports the use of adjuvant chemoradiotherapy in the treatment of APXA. The genetic landscape may be informative for optimizing treatment and prognosis.

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Section: Discussionmentioning

confidence: 99%

A case series of pediatric survivors of anaplastic pleomorphic xanthoastrocytoma

Ronsley

Dunham

Yip

et al. 2021

Neuro-Oncology Advances

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“…In addition, there were abundant reticular fibers and lymphocyte infiltrations around the blood vessels. 12 About 50%–78% of PXA cases have BRAF gene mutations, most of which are BRAF V600E mutations. Compared with PXA, APXA has a lower BRAF mutation rate.…”

Section: Discussionmentioning

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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“…APXA mostly occur in the first and third decade of life, and similar to PXA they tend to assume a superficial location in the cerebral cortex and leptomeninges with a predilection for the temporal and parietal lobes in majority of the cases [ 3 , 4 ]. Rarely tumours have been seen in the ventricular system [ 5 , 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…These tumours mostly originate from malignant transformation of PXA in about 9-20%, but very rarely can arise de novo described as primary APXA [ 7 ]. Several cases of primary APXA have been reported in literature [ 4 , 8 ]. The BRAF-V600E mutation has been implicated in about 78% of PXA and a significant proportion of APXA [ 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

Anaplastic Pleomorphic Xanthoastrocytoma Presenting with Musical Hallucination

Oladiran

Nwosu

Obanor

et al. 2018

Case Reports in Neurological Medicine

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Musical hallucinations are a relatively rare form of auditory hallucination characterized by hearing of music in the absence of any external stimuli. This phenomenon has been linked to both psychiatric and structural lesions. We present the case of a previously healthy young male whose presentation with musical hallucinations led to the diagnosis of a rare tumour, anaplastic pleomorphic xanthoastrocytoma.

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Primary anaplastic pleomorphic xanthoastrocytoma in adults. Case report and review of literature

Abstract: HighlightsA case report and literature review of anaplastic pleomorphic xanthoastrocytoma in adults.The tumor shows an aggressive clinical course compared with its benign variant.APXA shows histological characteristics comparable with grade III astrocytomas.

Cited by 22 publications

References 21 publications

A case series of pediatric survivors of anaplastic pleomorphic xanthoastrocytoma

A case series of pediatric survivors of anaplastic pleomorphic xanthoastrocytoma

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

Anaplastic Pleomorphic Xanthoastrocytoma Presenting with Musical Hallucination

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