UNASSIGNED: Pleomorphic 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.
UNASSIGNED: A 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.
UNASSIGNED: To 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.

We present a unique case of a 45-year-old male with cerebral palsy, who experienced walking difficulties and altered consciousness. The initial MRI revealed an intraventricular mass that rapidly enlarged over a month, consisting of two distinct components with different characteristics on CT and MRI, and was associated with agenesis of the corpus callosum. Despite initial treatment, surgical intervention was necessary, where preoperative imaging suggested an exophytically growing glioblastoma. However, postsurgical pathological examination identified the mass as pleomorphic xanthoastrocytoma (PXA), World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) grade 3. This study is notable for its rarity and complexity, challenging standard diagnostic approaches. PXA is an uncommon astrocytic tumor, and its occurrence intraventricularly is extremely rare. This study highlights its unique imaging features and the critical role of MRI in preoperative assessment, underlining the tumor\'s unusual intraventricular location, and its relationship with corpus callosum agenesis. Our comprehensive review of PXA\'s history and imaging spectrum offers valuable insights for neuroradiologists and neurosurgeons, emphasizing the diagnostic challenges of such rare tumor locations and the importance of meticulous MRI analysis for accurate diagnosis.

UNASSIGNED: High grade pleomorphic xanthoastrocytomas (HGPXAs) are very rare and their management and prognostic outcomes remain unclear. To better understand the disease, we aimed to evaluate the risk factors for progression-free survival (PFS) and overall survival (OS), and propose a treatment protocol based on cases from our institute and cases from the literature.
UNASSIGNED: The authors reviewed the clinical data of 26 patients with HGPXAs who underwent surgical treatment in Department of Neurosurgery of Beijing Tiantan Hospital between August 2014 and September 2021. We also searched the PubMed database using the keywords \"anaplastic\" combined with \"pleomorphic xanthoastrocytoma(s)\" between January 1997 and October 2022. Risk factors for PFS and OS were evaluated in the pooled cases.
UNASSIGNED: The authors\' cohort included 11 males and 15 females with a mean age of 36.7 ± 20.3 years (range: 5.5-71 years). Gross-total resection (GTR) and non-GTR were achieved in 17 (65.4%) and 9 (34.6%) patients, respectively. Radiotherapy and chemotherapy were administered to 22 and 20 patients, respectively. After a mean follow-up of 20.5 ± 21.2 months (range: 0.5-78.1 months), 7 patients suffered tumor recurrence and 6 patients died with a mean OS time of 19.4 ± 10.8 months (range: 8-36 months). In the literature between January 1997 and October 2022, 56 cases of HGPXAs were identified in 29 males and 27 females with a mean age of 29.6 ± 19.6 years (range; 4-74 years). Among them, 24 (44.4%) patients achieved GTR. Radiotherapy and chemotherapy was administered to 31 (62%) patients and 23 (46%) patients, respectively. After a median follow-up of 31.4 ± 35.3 months (range: 0.75-144 months), the mortality and recurrence rates were 32.5% (13/40) and 70% (28/40), respectively. Multivariate Cox regression model demonstrated that non-GTR (HR 0.380, 95% CI 0.174-0.831, p=0.015), age≥30 (HR 2.620, 95% CI 1.183-5.804, p=0.018), no RT (HR 0.334,95% CI 0.150-0.744, p=0.007) and no CT (HR 0.422, 95% CI 0.184-0.967, p=0.042) were negative prognostic factors for PFS. Non-GTR (HR 0.126, 95% CI 0.037-0.422, p=0.001), secondary HGPXAs (HR 7.567, 95% CI 2.221-25.781, p=0.001), age≥30 (HR 3.568, 95% CI 1.190-10.694, p=0.023) and no RT (HR 0.223,95% CI 0.073-0.681, p=0.008) were risk factors for OS.
UNASSIGNED: High grade pleomorphic xanthoastrocytomas are very rare brain tumors. Children and younger adults have better clinical outcome than elderly patients. Secondary HGPXAs had worse OS than primary HGPXAs. Complete surgical excision plus RT and CT is recommended for this entity. The frequency of BRAF mutations in HGPXAs is 47.5% (19/40) in this study, however, we do not find the connections between BRAF mutations and clinical outcomes. Future studies with larger cohorts are necessary to verify our findings.

BACKGROUND:  Pleomorphic xanthoastrocytoma (PXA) is a rare low-grade brain tumor. To date, limited studies have analyzed factors affecting survival outcomes and defined the therapeutic strategy. The aim of this retrospective analysis was to investigate the clinicopathologic characteristics of PXA and identify factors associated with outcomes.
METHODS:  We retrospectively analyzed a cohort of 16 adult and children patients with PXA who underwent primary resection from 1997 to 2019, referred to our Radiation Oncology Unit and to Meyer\'s Paediatric Hospital. We also reviewed the relevant literature.
RESULTS:  All patients underwent primary surgical resection; 10 patients received adjuvant radiation treatment course, ranging from DTF 54 to 64 Gy; 8 of them received, in addition, concurrent adjuvant chemotherapy; 6 patients underwent only radiological follow-up. After a median follow up was 60 months: median OS was 34.9 months (95% CI 30-218), 1-year OS 87%, 5-years OS 50%, 10-years OS 50%; median PFS 24.4 months (95% CI 13-156), 1-year PFS 80%, 5-years PFS 33%, 10-years PFS 33%. A chi-square test showed a significant association between OS and recurrent disease (p = 0.002) and with chemotherapy adjuvant treatment (p = 0.049). A borderline statistical significant association was instead recognized with BRAF mutation (p = 0.058).
CONCLUSIONS: Despite our analysis did not reveal a strong prognostic or predictive factor able to address pleomorphic xanthoastrocytoma management; however, in selected patients could be considered the addition of adjuvant radiation chemotherapy treatment after adequate neurosurgical primary resection. Furthermore, recurrent disease evidenced a detrimental impact on survival.

In the central nervous system, the presence of pigment in astrocytic tumors is rare. Only nine cases were reported in the English literature: one ganglioglioma, one pilocytic astrocytoma, and seven cases of pleomorphic xanthoastrocytoma (PXA). Though squash cytology is a common and useful tool for intraoperative diagnosis during brain tumor surgeries, the cytologic findings of pigmented PXA have not been recorded previously. We present a 32-year-old woman with a mass in her medial right temporal lobe. Intraoperative squash cytology examination demonstrated pleomorphic tumor cells containing cytoplasmic pigment granules. The subsequent tissue section and additional workup revealed a PXA with melanosomal melanin pigment deposits. While conventional PXA has to be differentiated from high-grade tumors such as glioblastoma, in the pigmented variant the presence of pigment can cause further diagnostic confusion with other pigmented tumors, in particular melanoma. It should be added into the differential diagnoses when a brain tumor smear shows nuclear pleomorphism and intracytoplasmic pigment particles.

Tuberous sclerosis complex (Bourneville-Pringle syndrome) is a rare genetic condition included in the group of diseases called phakomatoses. Most of the patients are diagnosed with abnormalities within the central nervous system and tend to develop tumors more frequently, especially gliomas. We present a case of 50-year-old patient suffering from tuberous sclerosis complex, who had been diagnosed with pleomorphic xanthoastrocytoma (PXA). The patient underwent surgery and adjuvant radiotherapy and has remained free from local recurrence for 5 years.

Pediatric pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor. To date, there are few studies dedicated to this kind of pediatric tumor. The aim of this study was to investigate the clinicopathologic characteristics of pediatric PXA.
We retrospectively analyzed 17 pediatric patients diagnosed with PXA histologically between July 2009 and December 2018. We also reviewed the relevant literature.
The majority of pediatric PXAs had cystic components and peritumoral edema, and approximately 40% of the tumors had calcifications. All large tumors (≥5 cm) were located in the nontemporal lobes except 1 (P = 0.05). Furthermore, the large tumors were primarily solid-cystic or cystic with mural nodules radiologically, while tumors measuring <5 cm were mainly solid or solid with cystic changes (P = 0.02). All patients underwent surgery, and 15 patients experienced complete tumor removal. Histologically, 11 patients had grade II PXAs and 6 patients had grade III PXAs. After the operation, most of the patients recovered uneventfully and the seizures were well controlled. The mean follow-up time was 43 months. Five patients received radiotherapy or chemotherapy. One patient had tumor recurrence 5 years after the first operation and underwent repeat surgery.
Cystic components and peritumoral edema could be seen in most pediatric PXAs, and calcification was also not uncommon. The size of the tumor was correlated with the tumor site and radiologic subtype. Maximal safe resection of pediatric PXA is recommended and was shown to be beneficial for seizure control and survival.

UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

Pleomorphic xanthoastrocytomas (PXAs) are rare low-grade astrocytic tumors that typically present as superficial nodular cystic tumors of the cerebrum attached to the leptomeninx. Histologically, they are pleomorphic, hypercellular glial neoplasms. Despite the presence of microscopic pleomorphism, patients\' postoperative prognosis is generally good. Anaplastic PXAs (APXAs) have a high mitotic index and patients with APXAs have a worse prognosis than patients with PXAs. Here, we report an autopsy case of APXA initially diagnosed as PXA. After gross total resection, the tumor recurred and was diagnosed as an APXA; thereafter, the patient died. An autopsy revealed that the tumor had relapsed at the primary site and had spread to the leptomeningeal space while concurrently invading the cerebrum including the periventricular area forming multifocal lesions. The histological findings of the autopsy were similar to those for epithelioid glioblastoma (EGBM) and small cell glioblastoma (SCGBM). In particular, the periventricular area with multifocal lesions was composed of SCGBM-like cells. It has been shown that multifocal lesions are frequently identified in patients with SCGBM. This is the first histopathologically confirmed case of APXA-related tumor presenting with periventricular extension and multifocal lesion formation. The periventricular extension might be a feature of PXAs and APXAs. However, suspected periventricular spread on imaging in past cases of PXAs and APXAs might instead represent the malignant transformation of these tumors to glioblastoma-like high-grade tumors, which often show SCGBM-like histological patterns.

Pleomorphic xanthoastrocytoma (PXA) is a relatively rare, low grade astrocytic tumor that usually affects children as well as young adults. The reported cases were predominantly located superficially in the temporal lobe. To our knowledge, so far only two cases of PXA occurring in lateral ventricle were reported in English literature. Herein, we present the third case of PXA intra-lateral ventricle in a 28-year-old Chinese male. Histologically, the tumor was relatively well circumscribed and consisted of spindle-shaped, ovoid, and multinuclear giant cells admixed with scattered eosinophilic granular bodies, inflammatory cells, and xanthomatous cells. Immunohistochemically, the tumor cells were strongly positive for S-100, GFAP, oligo-2 and vimentin, focally positive for synaptophysin and CD34, and negative for cytokeratin, EMA, NeuN and IDH1. Ki-67 proliferation index was approximately 2%. A BRAF V600E mutation was then identified in the tumor. Based on morphologic features, the immunohistochemical staining and BRAF V600E mutation, the tumor was diagnosed as a PXA. Because of the presence of the bizarre multinuclear giant cells and xanthomatous cells and the unusual location, PXA was easily misdiagnosed as a high-grade tumor. It should be noted that PXA was also an important differential diagnosis for intraventricular tumors.