A Case of Pleomorphic Xanthoastrocytoma with Intracranial Hemorrhage in a Child

Takamine, Yusuke; Yamamuro, Shun; Sumi, Koichiro; Ohta, Takashi; Shijo, Katsunori; Nakanishi, Yoko; Homma, Taku; Yoshino, Atsuo

doi:10.2176/nmccrj.cr.2018-0174

Cited by 2 publications

(1 citation statement)

References 9 publications

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“…However, AGs generally lack such enhancement ( 19 ). On a CT scan, PXA shows hemorrhage more commonly, but calcification is rare ( 34 ). There is typically adjacent cortical thickening and fewer calcifications in DNET ( 10 ).…”

Section: Characteristics Of Pdlggs Of Different Tumor Typesmentioning

confidence: 99%

Review on neuroimaging in pediatric-type diffuse low-grade gliomas

Chen,

Qi,

Zhang

et al. 2023

Front. Pediatr.

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The fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System (WHO CNS5) has identified a new classification system for tumors of the brain and spinal cord, highlighting the pivotal role of molecular diagnosis in accurately categorizing neoplasms. In addition to previous classifications, one of the key distinctions lies in categorizing pediatric-type diffuse low-grade gliomas (pDLGGs) and pediatric-type diffuse high-grade gliomas (pDHGGs) as distinct tumor types. Although similar in histology and morphology, pediatric diffuse gliomas are completely different from the adult type with respect to the molecular genetic characteristics, prognosis, and treatment strategies. pDLGG includes four tumor types, namely, diffuse astrocytoma, MYB- or MYBL1-altered; angiocentric glioma; polymorphous low-grade neuroepithelial tumor of the young (PLNTY); and diffuse low-grade glioma, MAPK pathway-altered, three types of which are newly recognized tumor types. Herein, we review the clinical characteristics, histopathological and molecular genetic characteristics, neuroimaging features, and prognosis of pDLGG and summarize the neuroimaging key points in diagnosing different tumor types. This review aims to evaluate and update the relevant pDLGG features and advances in neuroimaging that may assist in differential diagnosis, surgery planning, and prognostic determination of these tumor types and provide accurate diagnostic information for clinical colleagues.

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Section: Characteristics Of Pdlggs Of Different Tumor Typesmentioning

confidence: 99%

Review on neuroimaging in pediatric-type diffuse low-grade gliomas

Chen,

Qi,

Zhang

et al. 2023

Front. Pediatr.

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Pleomorphic xanthoastrocytoma with NTRK fusion presenting as spontaneous intracranial hemorrhage—case report and literature review

Wu,

Aw,

Jain

et al. 2024

Front. Pediatr.

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BackgroundPleomorphic xanthoastrocytoma (PXA) is a rare brain tumor that accounts for <1% of all gliomas. An in-depth understanding of PXA's molecular makeup remains a work in progress due to its limited numbers globally. Separately, spontaneous intracranial hemorrhage (pICH) is an uncommon but potentially devastating emergency in young children, often caused by vascular malformations or underlying hematological conditions. We describe an interesting case of a toddler who presented with pICH, later found to have a PXA as the underlying cause of hemorrhage. Further molecular interrogation of the tumor revealed a neurotrophic tyrosine receptor kinase (NTRK) gene fusion and CDKN2A deletion more commonly seen in infantile high-grade gliomas. The unusual clinicopathological features of this case are discussed in corroboration with published literature.Case presentationA previously well 2-year-old male presented with acute drowsiness and symptoms of increased intracranial pressure secondary to a large right frontoparietal intracerebral hematoma. He underwent an emergency craniotomy and partial evacuation of the hematoma for lifesaving measures. Follow-up neuroimaging reported a likely right intra-axial tumor with hemorrhagic components. Histology confirmed the tumor to be a PXA (WHO 2). Additional molecular investigations showed it was negative for BRAFV600E mutation but was positive for CDKN2A homozygous deletion and a unique neurotrophic tyrosine receptor kinase (NTRK) gene fusion. The patient subsequently underwent second-stage surgery to proceed with maximal safe resection of the remnant tumor, followed by the commencement of adjuvant chemotherapy.ConclusionTo date, there are very few pediatric cases of PXA that present with spontaneous pICH and whose tumors have undergone thorough molecular testing. Our patient's journey highlights the role of a dedicated multidisciplinary neuro-oncology team to guide optimal treatment.

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A Case of Pleomorphic Xanthoastrocytoma with Intracranial Hemorrhage in a Child

Cited by 2 publications

References 9 publications

Review on neuroimaging in pediatric-type diffuse low-grade gliomas

Review on neuroimaging in pediatric-type diffuse low-grade gliomas

Pleomorphic xanthoastrocytoma with NTRK fusion presenting as spontaneous intracranial hemorrhage—case report and literature review

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