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Journal of Child Neurology Oct 2020Pilocytic astrocytomas are the primary tumors most frequently found in children and adolescents, accounting for approximately 15.6% of all brain tumors and 5.4% of all... (Review)
Review
Pilocytic astrocytomas are the primary tumors most frequently found in children and adolescents, accounting for approximately 15.6% of all brain tumors and 5.4% of all gliomas. They are mostly found in infratentorial structures such as the cerebellum and in midline cerebral structures such as the optic nerve, hypothalamus, and brain stem. The present study aimed to list the main characteristics about this tumor, to better understand the diagnosis and treatment of these patients, and was conducted on search of the published studies available in NCBI, PubMed, MEDLINE, Scielo, and Google Scholar. It was possible to define the main histologic findings observed in these cases, such as mitoses, necrosis, and Rosenthal fibers. We described the locations usually most affected by tumor development, and this was associated with the most frequent clinical features. The comparison between the molecular diagnostic methods showed great use of fluorescent in situ hybridization, polymerase chain reaction (PCR), and reverse transcriptase-PCR, important techniques for the detection of mutation and fusion, characteristic molecular alterations in pilocytic astrocytomas.
Topics: Astrocytoma; Brain Neoplasms; Humans; In Situ Hybridization, Fluorescence; Polymerase Chain Reaction
PubMed: 32691644
DOI: 10.1177/0883073820937225 -
Neuropathology : Official Journal of... Apr 2023
Topics: Humans; Astrocytoma; Brain Neoplasms
PubMed: 36161674
DOI: 10.1111/neup.12871 -
CNS Oncology Mar 2021Adult pilocytic astrocytoma (PA) is less prevalent than pediatric PA and is associated with a worse prognosis. In a literature review, we found that 88.3% of the... (Review)
Review
Adult pilocytic astrocytoma (PA) is less prevalent than pediatric PA and is associated with a worse prognosis. In a literature review, we found that 88.3% of the molecular alterations in adult PA are associated with MAPK pathway dysregulation. The most common alterations are fusions of . Understanding of the mechanisms underlying this pathway has evolved substantially, heralding advancements in specific targeted therapy. Here, we review clinical and molecular features of adult PA, characteristics predicting aggressive behavior and approaches to standard and investigational therapies. We highlight epigenetic profiling and integrated diagnosis as an essential component of classifying PA.
Topics: Adult; Astrocytoma; Brain Neoplasms; Child; Humans; Prognosis; Proto-Oncogene Proteins B-raf
PubMed: 33448230
DOI: 10.2217/cns-2020-0027 -
Neuro-oncology Apr 2023
Topics: Humans; Senotherapeutics; Astrocytoma; Brain
PubMed: 36702510
DOI: 10.1093/neuonc/noad016 -
Neurology India 2022Intraventricular pilocytic astrocytomas are a rare occurrence, accounting for approximately 4% -15.6% of all pilocytic astrocytomas .The aim of the study was to describe...
BACKGROUND
Intraventricular pilocytic astrocytomas are a rare occurrence, accounting for approximately 4% -15.6% of all pilocytic astrocytomas .The aim of the study was to describe the radiology, surgical management and outcome in 15 patients with histopathologically proven intraventricular pilocytic astrocytoma(IVPA).
OBJECTIVE
To study the clinical presentation radiology and operative challenges in rare intra ventricular pilocytic astrocytomas.
MATERIALS AND METHODS
Between January 2010 and August 2018, 15 patients with histopathologically proven IVPA were identified. The radiological images were obtained from PACS. Patient and surgical details were obtained from the computerized discharge summary, OT records and operative notes, whereas follow up was obtained from the record section.
RESULTS
Headache with progressive loss of vision was the most common presentation. Duration of symptoms varied from 4 months to 2 years (mean 9. 88 months). Except one patient, all patients with preoperative CT scan revealed calcifications in the lesion, with extensive calcification in 3 patients. All the tumors were predominantly hypointense on T1WI and iso to hyperintense on T2WI. Lesion in all patients showed heterogenous contrast enhancement on post gadolinium images. Mean blood loss in the series was 1969 ml (range 250 ml- 4500 ml).There was one death in this series due to meningitis and septic shock.
CONCLUSION
IVPAs are rare tumors and are difficult to diagnose in the preoperative period based on the radiologic profile alone. These tumors can be extremely vascular with potential for massive blood loss. These tumors can be associated with extensive calcification and the calcified tumors have less bleeding as expected.
Topics: Astrocytoma; Brain Neoplasms; Calcinosis; Gadolinium; Humans; Tomography, X-Ray Computed
PubMed: 36076645
DOI: 10.4103/0028-3886.355185 -
Acta Neuropathologica Jun 2015Pilocytic astrocytomas (PAs) were recognized as a discrete clinical entity over 70 years ago. They are relatively benign (WHO grade I) and have, as a group, a 10-year... (Review)
Review
Pilocytic astrocytomas (PAs) were recognized as a discrete clinical entity over 70 years ago. They are relatively benign (WHO grade I) and have, as a group, a 10-year survival of over 90%. Many require merely surgical removal and only very infrequently do they progress to more malignant gliomas. While most show classical morphology, they may present a spectrum of morphological patterns, and there are difficult cases that show similarities to other gliomas, some of which are malignant and require aggressive treatment. Until recently, almost nothing was known about the molecular mechanisms involved in their development. The use of high-throughput sequencing techniques interrogating the whole genome has shown that single abnormalities of the mitogen-activating protein kinase (MAPK) pathway are exclusively found in almost all cases, indicating that PA represents a one-pathway disease. The most common mechanism is a tandem duplication of a ≈2 Mb-fragment of #7q, giving rise to a fusion between two genes, resulting in a transforming fusion protein, consisting of the N-terminus of KIAA1549 and the kinase domain of BRAF. Additional infrequent fusion partners have been identified, along with other abnormalities of the MAP-K pathway, affecting tyrosine kinase growth factor receptors at the cell surface (e.g., FGFR1) as well as BRAF V600E, KRAS, and NF1 mutations among others. However, while the KIAA1549-BRAF fusion occurs in all areas, the incidence of the various other mutations identified differs in PAs that develop in different regions of the brain. Unfortunately, from a diagnostic standpoint, almost all mutations found have been reported in other brain tumor types, although some retain considerable utility. These molecular abnormalities will be reviewed, and the difficulties in their potential use in supporting a diagnosis of PA, when the histopathological findings are equivocal or in the choice of individualized therapy, will be discussed.
Topics: Astrocytoma; Central Nervous System Neoplasms; Genetic Predisposition to Disease; Humans; Models, Molecular; Molecular Biology; Neuroimaging
PubMed: 25792358
DOI: 10.1007/s00401-015-1410-7 -
Acta Cytologica 2004
Topics: Astrocytes; Astrocytoma; Brain Neoplasms; Capillaries; Child; Diagnosis, Differential; Humans; Inclusion Bodies
PubMed: 14969172
DOI: 10.1159/000326274 -
British Journal of Neurosurgery Feb 2022Pilocytic astrocytoma is a benign low-grade tumor with a favorable prognosis. We present a 47-year-old- lady with a posterior fossa pilocytic astrocytoma who underwent... (Review)
Review
Pilocytic astrocytoma is a benign low-grade tumor with a favorable prognosis. We present a 47-year-old- lady with a posterior fossa pilocytic astrocytoma who underwent surgical decompression. She developed multiple early local recurrences Along with malignant transformation of the cranial lesion she developed skeletal dissemination within a very short time frame. There were no features or family history of neurofibromatosis 1. She did not receive radiotherapy or chemotherapy prior to the recurrences.
Topics: Astrocytoma; Cell Transformation, Neoplastic; Decompression, Surgical; Female; Humans; Middle Aged; Neurofibromatosis 1; Recurrence
PubMed: 29745267
DOI: 10.1080/02688697.2018.1472214 -
World Neurosurgery Jun 2019Intramedullary glial neoplasms affecting the entire spinal cord from the cervicomedullary junction to conus medullaris are termed holocord tumors. Pilocytic astrocytomas... (Review)
Review
BACKGROUND
Intramedullary glial neoplasms affecting the entire spinal cord from the cervicomedullary junction to conus medullaris are termed holocord tumors. Pilocytic astrocytomas are relatively uncommon tumors forming holocord involvement. Hitherto 24 holocord astrocytoma cases were reported in the literature. Here, a case of an adult holocord pilocytic astrocytoma was presented.
CASE DESCRIPTION
A 29-year-old female patient presented with a subtotal resection. Histopathologic examination revealed pilocytic astrocytoma, World Health Organization grade 1, presenting holocord involvement. Magnetic resonance imaging showed a mass containing necrotic and cystic foci leading to an expansion along the entire spinal cord extending from C4 to conus medullaris.
CONCLUSIONS
Reported holocord pilocytic astrocytomas in the relevant literature cases were reviewed. When evaluating magnetic resonance imaging scans of adult patients with various neurologic symptoms, i.e., weakness and fecal and urinary incontinence, pilocytic astrocytoma should be considered among differential diagnoses of intramedullary mass lesions.
Topics: Adult; Astrocytoma; Female; Humans; Spinal Cord; Spinal Cord Neoplasms
PubMed: 30902768
DOI: 10.1016/j.wneu.2019.03.103 -
World Neurosurgery Feb 2018Pilocytic astrocytoma is a classically benign tumor that most often affects pediatric patients. Rarely, it occurs during adulthood. We present a case series and... (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Pilocytic astrocytoma is a classically benign tumor that most often affects pediatric patients. Rarely, it occurs during adulthood. We present a case series and systematic literature review of adult pilocytic astrocytoma (APA) to examine the clinical presentation, extent of resection, and recurrence rate associated with this tumor in this population.
MATERIALS AND METHODS
Our institutional records were retrospectively reviewed for cases of pilocytic astrocytoma in adults. A PubMed search identified English-language studies of pathology-proven APA. A meta-analysis was performed to determine the relationship between extent of tumor resection and recurrence.
RESULTS
Forty-six patients with APA were diagnosed at our institution (mean age 33.6 ± 13.3; 24 [52%] female). Twenty-four patients (52%) underwent gross total resection, 11 (24%) subtotal resection, 4 (9%) near total resection, 4 (9%) observation after biopsy, and 3 (6%) radiotherapy alone. Tumors recurred or progressed in 6 (13%) patients, of whom 4 were treated by STR and 2 were treated by radiotherapy alone. Thirty-nine (95%) patients were still alive at last follow-up. A systematic literature review identified 415 patients with APA in 38 studies. Including our case series, 7 studies reported extent of resection, follow-up, and recurrence. Of 254 patients with a weighted mean follow-up of 77.7 ± 49.6 (31-250) months, 129 (51%) were treated with gross total resection, and 125 (49%) underwent subtotal resection. Tumor recurred in 79 (31%) patients, 22 (27%) after gross total resection and 57 (73%) after subtotal resection (P < 0.001).
CONCLUSIONS
Pilocytic astrocytoma rarely presents during adulthood. Overall, prognosis is favorable and survival rates are high. APA recurrence is more likely after STR, and the goal of surgery should always be GTR when feasible.
Topics: Adolescent; Adult; Astrocytoma; Brain Neoplasms; Female; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Neurosurgical Procedures; PubMed; Young Adult
PubMed: 29180079
DOI: 10.1016/j.wneu.2017.11.102