-
Cirugia Y Cirujanos 2017Pilocytic astrocytoma is a rare tumour, usually occurring in paediatric ages, and mainly located in the posterior fossa. It can cause hydrocephalus and intracranial... (Review)
Review
BACKGROUND
Pilocytic astrocytoma is a rare tumour, usually occurring in paediatric ages, and mainly located in the posterior fossa. It can cause hydrocephalus and intracranial hypertension and, less frequently, seizures, or a focal neurological deficit. The main imaging study by magnetic resonance imaging, which shows a tumour with solid and cystic components without peri-lesional swelling. The election treatment is surgical, and the patient is considered cured if a total resection is accomplished.
CLINICAL CASE
The case is presented of 22-year-old female patient with a supratentorial pilocytic astrocytoma and epilepsy. Histopathology reported a low grade glial proliferation, with an extensive fibrillar matrix, small cells without atypia, extensive calcifications and piloid areas consisting of bipolar fusiform cells, and some Rosenthal fibres. There were also spongiotic areas consisting of multipolar cells and associated microcysts. The final report was a pilocytic astrocytoma.
CONCLUSIONS
Pilocytic astrocytoma is more frequent in paediatric patients and in the posterior fossa. The case presented is of a young female adult with supratentorial location, making it a special case. The surgery achieved a total resection. The long-term prognosis is good, but it is necessary to perform a follow-up, particularly in adult patients because of a higher risk of recurrence.
Topics: Anticonvulsants; Astrocytoma; Cholecystitis, Acute; Craniotomy; Female; Humans; Levetiracetam; Magnetic Resonance Imaging; Neuroimaging; Piracetam; Remission Induction; Seizures; Supratentorial Neoplasms; Young Adult
PubMed: 27417707
DOI: 10.1016/j.circir.2016.05.009 -
World Neurosurgery Jan 2020Pilocytic astrocytoma (PA) is rare in adults comprising 5.1% of the primary central nervous system tumors. The aim is to describe the first Brazilian series of adult... (Observational Study)
Observational Study
OBJECTIVE
Pilocytic astrocytoma (PA) is rare in adults comprising 5.1% of the primary central nervous system tumors. The aim is to describe the first Brazilian series of adult patients with PA and compare its features with the available literature.
METHODS
We retrospectively review all patients 18 years or older with PA from our institution's database from 1991 to 2018. We analyzed information regarding clinical presentation, location, imaging features, extent of resection, adjuvant treatments, and follow-up.
RESULTS
Twenty-three patients with PA were analyzed: 60.9% male; median age 26 years. The most frequent symptoms were headache (34.8%) and seizure (26.1%). Temporal and parietal lobes were the most common locations, 21.7% each. All patients underwent a surgical procedure, gross total resection in 40.9%, subtotal resection in 22.7%, and biopsy in 27.3%. Adjuvant treatment with radiotherapy was performed in 2 patients. Only 4 patients had disease progression, 2 after gross total resection and 2 after subtotal resection. They were all alive and without evidence of new progression at the last follow-up (October 2018). Median overall survival was not reached after a median follow-up time of 88.9 months.
CONCLUSIONS
This is the first Brazilian series regarding adults with PA, and our patients had a favorable outcome as reported in recent literature reviews. The tumor's prevalence reduces within older patients and supratentorial lesions are more frequent, especially on the temporal lobe. There was no significant relationship between location and progression, although according to the literature the extent of resection remains the most important prognostic factor.
Topics: Adolescent; Adult; Astrocytoma; Brain Neoplasms; Brazil; Combined Modality Therapy; Cranial Irradiation; Disease Progression; Female; Follow-Up Studies; Headache; Humans; Male; Middle Aged; Radiotherapy, Adjuvant; Retrospective Studies; Seizures; Treatment Outcome; Young Adult
PubMed: 31550545
DOI: 10.1016/j.wneu.2019.08.146 -
Child's Nervous System : ChNS :... Jan 2020Pilocytic astrocytomas (PAs) are the most common pediatric central nervous system tumors. They constitute around 30% of all primary central nervous tumors in the...
Pilocytic astrocytomas (PAs) are the most common pediatric central nervous system tumors. They constitute around 30% of all primary central nervous tumors in the pediatric age group. Their clinical behavior may vary but most of them are indolent and do not undergo malignant transformations compared with their adult counterparts. PAs are primarily treated with surgery and in cases of progression; chemotherapy may be needed. They usually carry a good prognosis, with a 10-year survival rate of 90%. BRAFV600E mutations have been identified in approximately 9-15% of patients with PA. These relatively high mutation frequencies in PA open avenues for treatment using targeted therapies such as BRAFV600E inhibitors (e.g., dabrafenib). There have been a few published case reports and case series showing clinical benefits with BRAF inhibitors in BRAF-positive tumors. We report a case of successful treatment of BRAFV600E immunopositive optic pathway PA in a child with dabrafenib.
Topics: Adult; Astrocytoma; Brain Neoplasms; Child; Humans; Imidazoles; Mutation; Oximes; Proto-Oncogene Proteins B-raf
PubMed: 31418082
DOI: 10.1007/s00381-019-04346-2 -
Journal of Child Neurology Jul 1994Biologically malignant pilocytic astrocytomas are rare. Precise morphologic criteria permitting their identification and clinically meaningful distinction from more... (Review)
Review
Biologically malignant pilocytic astrocytomas are rare. Precise morphologic criteria permitting their identification and clinically meaningful distinction from more ordinary pilocytic astrocytomas have not been developed. The histologic grading schemes applied to fibrillary astrocytomas--ones based on nuclear atypia, increased cellularity, mitotic activity, endothelial proliferation, and necrosis--are thought not to correlate with prognosis in pilocytic astrocytomas. Nonetheless, these features were systematically sought in 107 cerebellar pilocytic astrocytomas, four of which were histologically malignant. The clinical, morphologic, and flow cytometric features of these cases were studied. The incidence of histologic malignancy occurring spontaneously in otherwise typical pilocytic astrocytomas was 0.9%; that occurring after radiation was 1.8%. The four histologically malignant tumors occurred in two males and two females, ages 6 to 18 years. All showed increased mitotic activity (three to five mitoses per high-power microscopic field [250x]). Endothelial proliferation and necrosis were present in three and two cases each. DNA ploidy analysis showed the spontaneously occurring malignant-appearing tumors to be aneuploid, whereas both previously radiated tumors were tetraploid; 5% to 11% of cells were in S phase. The appearance of histologic malignancy in pilocytic astrocytoma is rare and less reliably correlated with prognosis than in patients with fibrillary astrocytomas. Essential features of such tumors include a high mitotic index and perhaps a high percentage of cells in S phase.
Topics: Adolescent; Astrocytoma; Cerebellar Neoplasms; Cerebellum; Child; Craniotomy; DNA, Neoplasm; Female; Flow Cytometry; Humans; Male; Neoplasm Recurrence, Local; Ploidies
PubMed: 7930411
DOI: 10.1177/088307389400900317 -
Child's Nervous System : ChNS :... Mar 2023Pilocytic astrocytoma is a slow-growing tumor that predominantly develops in children, but has a broad age spectrum. A notable characteristic of pilocytic astrocytoma is...
PURPOSE
Pilocytic astrocytoma is a slow-growing tumor that predominantly develops in children, but has a broad age spectrum. A notable characteristic of pilocytic astrocytoma is that the tumor arises in diverse locations and the clinical course is not always benign. Therefore, it is necessary to elucidate the clinical spectrum of the disease and analyze the relevant prognostic factors.
METHODS
Demographic and treatment-related factors were retrospectively reviewed in a cohort of 254 patients with histologically confirmed pilocytic astrocytoma. Clinical features were compared between the pediatric group (N = 208; age < 18 years) and the adult group (N = 46; age ≥ 18 years). Cox regression analysis was performed to identify relevant prognostic factors.
RESULTS
There was no difference in progression-free survival (PFS) between the pediatric and adult groups (p = 0.36); however, patients under 8 years of age exhibited worse PFS (p < 0.01). Leptomeningeal seeding at diagnosis and pilomyxoid histology was observed only in pediatric patients. In the pediatric group, nine patients experienced recurrence after complete resection. Increasing age (hazard ratio (HR) = 0.89, p < 0.01) and adjuvant therapy (HR = 0.32, p < 0.01) were protective factors against tumor progression. In the adult group, no progression occurred after complete resection. Age and adjuvant therapy were not significant factors in the adult group.
CONCLUSION
Pilocytic astrocytoma presents with a diverse clinical spectrum. Complete resection is of utmost importance, and appropriate adjuvant treatment is recommended if complete resection cannot be achieved. Children with younger age are associated with more aggressive tumors, and recurrence may occur even after complete resection.
Topics: Child; Humans; Adult; Adolescent; Retrospective Studies; Astrocytoma; Treatment Outcome; Progression-Free Survival; Combined Modality Therapy; Brain Neoplasms
PubMed: 36662276
DOI: 10.1007/s00381-023-05839-x -
Journal of Clinical Neuroscience :... Dec 2009We report two adult patients with pilocytic astrocytomas with anaplastic features at initial diagnosis. Pilocytic astrocytomas are low-grade astrocytomas that occur...
We report two adult patients with pilocytic astrocytomas with anaplastic features at initial diagnosis. Pilocytic astrocytomas are low-grade astrocytomas that occur rarely in adults. Initial presentation of a pilocytic astrocytoma with anaplastic features is particularly uncommon and making a definitive diagnosis of pilocytic astrocytoma with anaplastic features can be challenging. It is critical to differentiate glioblastoma (World Health Organization [WHO] grade 4) and pilocytic astrocytoma with anaplastic features (WHO grade 3) from pilocytic astrocytoma (WHO grade 1) as there are significant therapeutic and prognostic implications. Improved therapeutic strategies are required for pilocytic astrocytomas with anaplastic features.
Topics: Anaplasia; Astrocytoma; Brain Neoplasms; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nystagmus, Pathologic
PubMed: 19815416
DOI: 10.1016/j.jocn.2009.04.012 -
Pediatrics International : Official... 2023
Topics: Humans; Proto-Oncogene Proteins B-raf; Astrocytoma; Brain Neoplasms; Mitogen-Activated Protein Kinase Kinases; Mutation
PubMed: 37804006
DOI: 10.1111/ped.15653 -
Arquivos de Neuro-psiquiatria Sep 2003Pilocytic astrocytoma (PA) is a benign tumor that rarely spread along the neuraxis. At the moment there are no more than five cases of leptomeningeal dissemination (LD)... (Review)
Review
Pilocytic astrocytoma (PA) is a benign tumor that rarely spread along the neuraxis. At the moment there are no more than five cases of leptomeningeal dissemination (LD) from PA at diagnosis described in the literature. Different patterns of presentation or recurrence may be noted: local recurrence, malignant transformation, multicentric disease or metastatic disease. LD and multicentric disease can be distinct pathological entities. We report two cases and analyse literature, emphasizing leptomeningeal spread at presentation. Hydrocephalus, biopsy and parcial ressection are likely to be favorable factors to the occurrence of LD. Otherwise, LD may be part of natural history of PA, as evidenced by its ocurrence in non-treated cases.
Topics: Astrocytoma; Cerebellar Neoplasms; Child; Fatal Outcome; Female; Humans; Hydrocephalus; Magnetic Resonance Imaging; Meningeal Neoplasms; Neoplasm Recurrence, Local; Subarachnoid Space; Tomography, X-Ray Computed
PubMed: 14595493
DOI: 10.1590/s0004-282x2003000500025 -
Journal of Neuropathology and... Feb 2019Rare pilocytic astrocytomas (PA) have been described to arise in the ventricles of children. They are even less common in this location for the adult population. We...
Rare pilocytic astrocytomas (PA) have been described to arise in the ventricles of children. They are even less common in this location for the adult population. We present the case of a 44-year old man presenting with vision and mental status changes. Brain imaging revealed an intraventricular mass within the right ventricular atrium, most consistent with a meningioma. Microscopic examination revealed a neoplasm composed of elongated to plump bipolar astrocytes arranged in a fascicular architecture, accompanied by foci containing numerous Rosenthal fibers. By immunohistochemistry, the tumor cells were positive for vimentin and glial fibrillary acid protein, whereas negative for epithelial membrane antigen. Isocitrate dehydrogenase 1 (R132H) was also negative. By fluorescence in situ hybridization, we detected a KIAA1549/BRAF fusion gene. These findings supported the diagnosis of intraventricular PA arising in an adult.
Topics: Adult; Astrocytoma; Cerebral Ventricle Neoplasms; Humans; Male; Oncogene Proteins, Fusion
PubMed: 30561708
DOI: 10.1093/jnen/nly116 -
Acta Neuropathologica Communications Aug 2016
Topics: Adult; Astrocytoma; Brain; Brain Neoplasms; Child; Female; Glioblastoma; Histones; Humans; Neoplasm Recurrence, Local
PubMed: 27519587
DOI: 10.1186/s40478-016-0361-0