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Surgical Neurology Apr 1994The authors report a case of leptomeningeal spinal metastasis of chiasmatic juvenile pilocytic astrocytoma. A 7-year-old boy underwent surgery and radiation therapy for... (Review)
Review
The authors report a case of leptomeningeal spinal metastasis of chiasmatic juvenile pilocytic astrocytoma. A 7-year-old boy underwent surgery and radiation therapy for a chiasmatic pilocytic astrocytoma. Three years later he experienced a syndrome of medullary compression. A lumbosacral intradural extra-axial tumor was discovered and subtotally excised. Pathologic examination revealed a metastasis of the chiasmatic tumor without any evidence of malignant transformation. Only five cases of leptomeningeal spinal dissemination of pilocytic astrocytoma have been previously reported. The authors review and discuss the clinical and pathologic features of these cases.
Topics: Astrocytoma; Brain Neoplasms; Child; Humans; Male; Meningeal Neoplasms; Spinal Cord Neoplasms
PubMed: 8165503
DOI: 10.1016/0090-3019(94)90182-1 -
Clinical Cancer Research : An Official... Mar 2019Pilocytic astrocytoma is the most common childhood brain tumor, characterized by constitutive MAPK activation. MAPK signaling induces oncogene-induced senescence (OIS),...
PURPOSE
Pilocytic astrocytoma is the most common childhood brain tumor, characterized by constitutive MAPK activation. MAPK signaling induces oncogene-induced senescence (OIS), which may cause unpredictable growth behavior of pilocytic astrocytomas. The senescence-associated secretory phenotype (SASP) has been shown to regulate OIS, but its role in pilocytic astrocytoma remains unknown. The patient-derived pilocytic astrocytoma cell culture model, DKFZ-BT66, was used to demonstrate presence of the SASP and analyze its impact on OIS in pilocytic astrocytoma. The model allows for doxycycline-inducible switching between proliferation and OIS. Both states were studied using gene expression profiling (GEP), Western blot, ELISA, and cell viability testing. Primary pilocytic astrocytoma tumors were analyzed by GEP and multiplex assay.
RESULTS
SASP factors were upregulated in primary human and murine pilocytic astrocytoma and during OIS in DKFZ-BT66 cells. Conditioned medium induced growth arrest of proliferating pilocytic astrocytoma cells. The SASP factors IL1B and IL6 were upregulated in primary pilocytic astrocytoma, and both pathways were regulated during OIS in DKFZ-BT66. Stimulation with rIL1B but not rIL6 reduced growth of DKFZ-BT66 cells and induced the SASP. Anti-inflammatory treatment with dexamethasone induced regrowth of senescent cells and inhibited the SASP. Senescent DKFZ-BT66 cells responded to senolytic BCL2 inhibitors. High and SASP expression in pilocytic astrocytoma tumors was associated with favorable progression-free survival.
CONCLUSIONS
We provide evidence for the SASP regulating OIS in pediatric pilocytic astrocytoma, with IL1B as a relevant mediator. SASP expression could enable prediction of progression in patients with pilocytic astrocytoma. Further investigation of the SASP driving the unpredictable growth of pilocytic astrocytomas, and its possible therapeutic application, is warranted.
Topics: Animals; Astrocytoma; Brain Neoplasms; Cell Proliferation; Cellular Senescence; Child; Culture Media, Conditioned; Datasets as Topic; Disease Models, Animal; Female; Gene Expression Profiling; Humans; Interleukin-1beta; Male; Mice; Primary Cell Culture; Prognosis; Progression-Free Survival; Tumor Cells, Cultured
PubMed: 30530705
DOI: 10.1158/1078-0432.CCR-18-1965 -
World Neurosurgery Jan 2020Intracranial tumor growth associated with pregnancy is not an uncommon phenomenon. Pilocytic astrocytoma is typically considered to be an indolent tumor with little to...
BACKGROUND
Intracranial tumor growth associated with pregnancy is not an uncommon phenomenon. Pilocytic astrocytoma is typically considered to be an indolent tumor with little to no risk of progression to higher-grade lesion. We present a rare case of cerebellar pilocytic astrocytoma transformation to hemorrhagic high-grade glioma during pregnancy.
CASE DESCRIPTION
Patient EK was a 32-year-old female with neurofibromatosis type 1 and known cerebellar pilocytic astrocytoma. For nearly a decade before her pregnancy, her cerebellar tumor was stable on imaging. Routine magnetic resonance imaging (MRI) of the head obtained at 20 weeks' gestation continued to demonstrate tumor stability. At 24 weeks' gestation, the patient had sudden, severe headaches. MRI of the head showed evidence of significant tumor expansion. The following day, the patient was found unresponsive. Computed tomography of the head demonstrated hemorrhage within the tumor and tonsillar herniation. Her neurologic examination revealed no brainstem reflexes; however, given her age and pregnancy, she underwent emergent decompression and tumor debulking. Unfortunately, she never improved neurologically. Final pathology identified the lesion as high-grade glioma with anaplastic changes and hemorrhagic conversion.
CONCLUSIONS
This is a unique case of indolent cerebellar pilocytic astrocytoma that transformed to high-grade glioma during pregnancy, proven by tumor growth on MRI and anaplasia on pathology. We hypothesize that increased levels of pregnancy hormones (progesterone, vascular endothelial growth factor, placental growth factor, among others) likely contributed to tumor growth. We recommend that all glial tumors be monitored extremely closely throughout pregnancy, and perhaps one should consider surgical treatment (if possible) before patients become pregnant.
Topics: Adult; Astrocytoma; Cell Proliferation; Cerebellar Neoplasms; Craniotomy; Decompression, Surgical; Disease Progression; Fatal Outcome; Female; Humans; Neurofibromatosis 1; Pregnancy; Pregnancy Complications, Neoplastic
PubMed: 31574330
DOI: 10.1016/j.wneu.2019.09.125 -
La Clinica Terapeutica 2022Histograms can be determined throughout tumors, relying partly on existing tumor microstructure knowledge and the sampling effect from area of interest analyses. We...
PURPOSE
Histograms can be determined throughout tumors, relying partly on existing tumor microstructure knowledge and the sampling effect from area of interest analyses. We aimed to investigate the impact of ADC histogram parameters in discriminating medulloblastoma, ependymoma, and pilocytic astrocytoma.
METHODS
This study received approval from the Institutional Ethics Review Committee of Children's Hospital 02. Processes were conducted according to relevant laws and regulations, and requirements for written informed consent were fulfilled. The study involved 24 patients at Children's Hospital 02 from February-December 2019. Group 1 included 12 children with medulloblastoma, group 2 included 5 with ependymoma, and group 3 included 7 with pilocytic astrocytoma. All patients underwent MRI followed by surgery or biopsy to obtain histopathological confirmations.
RESULTS
Our analysis indicated that AUC, sensitivity, and specificity were 96.7%, 91.7%, and 100%, respectively when ADCkurtosis (cut-off point = 2.34) was taken to differentiate between medulloblasto-mas and ependymomas. To distinguish between medulloblastomas and pilocytic astrocytomas, the cut-off points of ADCmean, ADCmedian, ADCmax, ADCmin, rADCmean, rADCmax, and rADCmin of 0.985, 0.910, 1.305, 0.710, 1.349, 1.738, and 1.251, were taken respectively with AUC, sensitivity, and specificity elicited at 100%. To discriminate between ependymomas and pilocytic astrocytomas, the cut-off points of ADCmean, ADCmedian, ADCmax, ADCmin, rADCmean, rADC-median, rADCmax and rADCmin were 1.010, 0.930, 1.270, 0.735, 1.346, 1.324, 1.676, and 1.273, respectively, with AUC, sensitivity, and specificity at 100%.
CONCLUSION
ADC histograms can facilitate differentiation among juvenile medulloblastoma, ependymoma, and pilocytic astrocytoma, providing reliable, objective evidence of tumor differentiation.
Topics: Astrocytoma; Cerebellar Neoplasms; Child; Diffusion Magnetic Resonance Imaging; Ependymoma; Humans; Medulloblastoma; Retrospective Studies
PubMed: 35857056
DOI: 10.7417/CT.2022.2448 -
Journal of Clinical Neuroscience :... Aug 2019Ganglioglioma (GG) is a mixed glio-neuronal tumour, comprised of a neoplastic glial component and dysplastic ganglion cells. GG is a tumour of unclear histogenesis;... (Comparative Study)
Comparative Study
Ganglioglioma (GG) is a mixed glio-neuronal tumour, comprised of a neoplastic glial component and dysplastic ganglion cells. GG is a tumour of unclear histogenesis; previous studies examining BRAF mutations and chromosome imprinting has provided evidence that both the neuronal and glial components likely arise from a common precursor. Both p.V600E mutation and KIAA1549-BRAF fusion have been described in pilocytic astrocytoma (PA) and GG, but they differ with regards to the rates of different BRAF alterations, and careful histological examination is an important component of patho-molecular correlations. More recently, cases of PA with gangliocytic differentiation (PA-GD) have been described, and these cases are thus far restricted to those with the KIAA1549-BRAF fusion. Here, we describe three cases of GGs in patients with history of previously diagnosed PAs. The cases differ with respect to the chronologic intervals between the PA and the GG diagnoses. In two of the cases, where the PA-GG diagnostic intervals are less than ten years, pathological review revealed the older specimens to have been misdiagnosed as PAs. In the third case, where the interval spanned multiple decades, the GG was found to be positive for both BRAF p.V600E immunohistochemistry (IHC) and for the KIAA1549-BRAF fusion. Molecular study for the BRAF p.V600E mutation was negative, proving the IHC result to be a false-positive. Our case demonstrates that cases of GG can harbour the KIAA1549-BRAF fusion, even with positive BRAF p.V600E IHC results, and the case highlights a diagnostic challenge that may be encountered.
Topics: Adolescent; Adult; Astrocytoma; Brain Neoplasms; Diagnostic Errors; Disease Progression; Female; Ganglioglioma; Humans; Male; Middle Aged; Mutation; Proto-Oncogene Proteins B-raf
PubMed: 31147230
DOI: 10.1016/j.jocn.2019.05.002 -
British Journal of Neurosurgery Feb 2020We present a rare case of a 55-yr old patient of pilocytic astrocytoma of the cerebello-pontine angle mimicking a vestibular schwannoma. The tumor protruded into the...
We present a rare case of a 55-yr old patient of pilocytic astrocytoma of the cerebello-pontine angle mimicking a vestibular schwannoma. The tumor protruded into the porus acusticus causing enlargement of the internal auditory meatus, which is quite an unusual feature of glial tumours.
Topics: Astrocytoma; Cerebellar Neoplasms; Cerebellopontine Angle; Cranial Nerve Neoplasms; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuroma, Acoustic; Neurosurgical Procedures; Tomography, X-Ray Computed; Vestibulocochlear Nerve
PubMed: 29278012
DOI: 10.1080/02688697.2017.1419163 -
Experimental and Molecular Pathology Aug 2021Ganglioglioma, pleomorphic xanthoastrocytoma (PXA) and pilocytic astrocytoma are rare brain neoplasms with frequent activation of mitogen-activated protein (MAP) kinase...
Ganglioglioma, pleomorphic xanthoastrocytoma (PXA) and pilocytic astrocytoma are rare brain neoplasms with frequent activation of mitogen-activated protein (MAP) kinase pathway. A downstream marker of MAP-kinase pathway activation is cyclin D1. However, the expression of cyclin D1 has not been studied in the differential diagnosis between these brain tumors. The aim of this work is to compare the expression of cyclin D1 in ganglioglioma, PXA, pilocytic astrocytoma. We also compared cyclin D1 expression in giant cell glioblastoma and in IDH wild type glioblastoma. Our work shows that roughly half of gangliogliomas have ganglion cells stained by cyclin D1 while two third of PXA have pleormophic cells stained by cyclin D1 and 15% of giant cell glioblastoma have pleomorphic cells stained by cyclin D1 (p < 0.001). Cyclin D1 never stains normal neurons either in the adjacent cortex of circumscribed tumor, or in entrapped neurons in IDH wild type glioblastomas. The expression of cyclin D1 is correlated to the presence of BRAF V600E mutation in ganglioglioma and PXA (p = 0.002). To conclude, cyclin D1 positivity might be used to confirm the neoplastic nature of ganglion cells. Cyclin D1 is expressed in most cases of BRAF V600E mutated gangliogliomas but also in cases without BRAF mutations suggesting an activation of MAP-kinase pathway through another way. Cyclin D1 immunohistochemistry has currently no or little role in the differential diagnosis of pilocytic astrocytoma. Its role in the differential diagnosis between PXA and giant cell glioblastoma needs to be further investigated on external series.
Topics: Adolescent; Adult; Astrocytoma; Cyclin D1; Diagnosis, Differential; Female; Ganglioglioma; Glioblastoma; Humans; Male; Middle Aged; Mutation; Prognosis; Proto-Oncogene Proteins B-raf; Young Adult
PubMed: 34022185
DOI: 10.1016/j.yexmp.2021.104652 -
MedGenMed : Medscape General Medicine Dec 2004Pilomyxoid astrocytoma (PMA) is a recently described type of brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous... (Review)
Review
Pilomyxoid astrocytoma (PMA) is a recently described type of brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous system (CNS) tumor in the pediatric population, yet displays subtle histologic differences. Previous studies have shown PMA to behave more aggressively than PA, with shorter progression-free and overall survival as well as a higher rate of recurrence and CNS dissemination. These findings suggest that PMA may be a unique and distinct neoplasm. This review summarizes the histologic, clinical, and radiographic characteristics of PMA. In addition, the current treatment options and research endeavors involving this disease are described. Increased recognition of PMA within the medical community has the potential to affect the treatment and prognosis of pediatric low-grade astrocytomas.
Topics: Astrocytoma; Brain; Brain Neoplasms; Humans; Radiography
PubMed: 15775869
DOI: No ID Found -
Radiologic Technology Sep 2019
Topics: Astrocytoma; Brain Neoplasms; Humans; Magnetic Resonance Imaging; Neoplasm Grading; Tomography, X-Ray Computed
PubMed: 31471483
DOI: No ID Found -
Brain Tumor Pathology Apr 2019Pilomyxoid astrocytoma is a variant of pilocytic astrocytoma and the clinical, histological and molecular data point to a very close relationship as well as a more... (Review)
Review
Pilomyxoid astrocytoma is a variant of pilocytic astrocytoma and the clinical, histological and molecular data point to a very close relationship as well as a more aggressive biological behavior for the former. WHO 2016 classification does not provide a specific grade for these neoplasms, but there is sufficient evidence in the literature that pilomyxoid astrocytoma has slightly worse prognosis than typical pilocytic astrocytoma. There is increasing evidence that in addition to the MAPK pathway alterations, pilomyxoid astrocytomas harbor genetic alterations that distinguish them from typical pilocytic astrocytoma.
Topics: Astrocytoma; Brain Neoplasms; Glioma; Humans; Mitogen-Activated Protein Kinases; Neoplasm Recurrence, Local; Proto-Oncogene Proteins B-raf
PubMed: 30945015
DOI: 10.1007/s10014-019-00343-0