-
Radiographics : a Review Publication of... 2004Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. This tumor has a noteworthy... (Review)
Review
Pilocytic astrocytoma is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. This tumor has a noteworthy benign biologic behavior that translates into an extremely high survival rate-94% at 10 years-that is by far the best of any glial tumor. Most patients present in the first 2 decades, and clinical symptoms and signs are usually of several months duration and directly related to the specific location of the tumor. The cerebellum, optic nerve and chiasm, and hypothalamic region are the most common locations, but the tumor can also be found in the cerebral hemisphere, ventricles, and spinal cord. Surgical resection is the treatment of choice for all tumors, except for those involving the optic pathway and hypothalamic region, which may be treated with radiation therapy and chemotherapy. Cross-sectional imaging often demonstrates a classic appearance: a cystic mass with an enhancing mural nodule. Less common appearances are quite nonspecific. Surrounding vasogenic edema is rarely present, and this feature provides a valuable clue to the correct diagnosis. Accurate interpretation of imaging studies plays an essential role in directing treatment of these tumors, particularly when they arise in the optic pathway of patients with neurofibromatosis type 1. Disseminated disease and recurrence are extremely rare.
Topics: Adult; Astrocytoma; Child; Humans; Tomography, X-Ray Computed
PubMed: 15537977
DOI: 10.1148/rg.246045146 -
Indian Journal of Pathology &... 2010Pilomyxoid astrocytoma (PMA) is a recently described brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous system...
Pilomyxoid astrocytoma (PMA) is a recently described 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. We describe a case of PMA in a six-year-old male involving sellar and suprasellar region presenting with failure to thrive and delayed developmental milestones. The histological findings revealed a tumor composed of a monotonous population of loosely arranged cells with delicate piloid like processes, within a prominent myxoid background. The tumor lacked biphasic appearance, Rosenthal fibers, eosinophilic granular bodies and calcification that are commonly observed in classical PA. Previous studies have shown PMA to behave more aggressively than PA, with shorter progression-free survival as well as a higher rate of recurrence and CNS dissemination. Thus, recognition of PMA and its distinction from classical PA is very important.
Topics: Astrocytoma; Brain; Brain Neoplasms; Child; Developmental Disabilities; Histocytochemistry; Humans; Male; Microscopy; Sella Turcica; Tomography, X-Ray Computed
PubMed: 20551536
DOI: 10.4103/0377-4929.64283 -
Journal of Neurosurgery. Pediatrics May 2022Pilocytic astrocytomas (PAs) have a generally favorable prognosis; however, progression or recurrence after resection is possible. The prognostic value of...
OBJECTIVE
Pilocytic astrocytomas (PAs) have a generally favorable prognosis; however, progression or recurrence after resection is possible. The prognostic value of histopathological qualifiers (defined below) or BRAF alterations is not well understood. The aim of this study was to identify the prognostic value of genetic and histopathological features of pediatric PAs.
METHODS
Patients treated for a WHO grade I PA at a single institution were analyzed for histopathological and genetic features and outcomes. "Histopathological qualifier" refers to designations such as "WHO grade I PA with increased proliferative index." BRAF alterations include gene fusions and point mutations. Patients with neurofibromatosis type 1 were excluded.
RESULTS
A total of 222 patients were analyzed (51% female, mean age 9.6 years). Tumors were located in the cerebellum/fourth ventricle (51%), optic pathway/hypothalamus (15%), brainstem (12%), and cerebral cortex (11%). BRAF alterations were screened for in 77 patients and identified in 56 (73%). Histopathological qualifiers were present in 27 patients (14%). Resection was performed in 197 patients (89%), 41 (21%) of whom displayed tumor progression or recurrence after resection. Tumor progression or recurrence was not associated with histopathologic qualifiers (p = 0.36) or BRAF alterations (p = 0.77). Ki-67 proliferative indices were not predictive of progression or recurrence (p = 0.94). BRAF alterations, specifically KIAA1549 fusions, were associated with cerebellar/fourth ventricular tumor location (p < 0.0001) and younger patient age (p = 0.03). Patients in whom gross-total resection was achieved had lower rates of progression and recurrence (p < 0.0001).
CONCLUSIONS
Histopathological features/qualifiers and BRAF alterations were not associated with tumor recurrence/progression in pediatric PAs. The extent of resection was the only factor analyzed that predicted outcome.
Topics: Child; Humans; Female; Male; Brain Neoplasms; Proto-Oncogene Proteins B-raf; Neoplasm Recurrence, Local; Astrocytoma; Cerebellar Neoplasms
PubMed: 35148515
DOI: 10.3171/2021.9.PEDS21405 -
BMJ Case Reports Oct 2020Pilocytic astrocytomas comprise the most common central nervous system tumour during childhood and have an excellent response to surgical treatment in this population....
Pilocytic astrocytomas comprise the most common central nervous system tumour during childhood and have an excellent response to surgical treatment in this population. The tumour incidence decreases with age, whereas more aggressive behaviour tends to increase. Haemorrhage as a presenting feature of pilocytic astrocytomas is a rare phenomenon, especially in the adult population. We present a case of a 55-year-old patient with progressive headaches and dizziness. MRI confirmed a sellar and predominantly retrochiasmal suprasellar lesion with heterogeneous signal, enhancement and blood products. Management via transsphenoidal approach was performed, and histopathology revealed the unexpected diagnosis of haemorrhagic pilocytic astrocytoma. Haemorrhagic pilocytic astrocytoma is an infrequent entity in the adult population and it is essential to recognise the peculiarities regarding diagnostic evaluation and management, which differ from the paediatric population. During adulthood, this tumour carries an overall unfavourable prognosis, with higher rates of progression and recurrence.
Topics: Astrocytoma; Brain; Brain Neoplasms; Cerebral Ventricles; Humans; Intracranial Hemorrhages; Magnetic Resonance Imaging; Male; Middle Aged; Sella Turcica; Tomography, X-Ray Computed
PubMed: 33122224
DOI: 10.1136/bcr-2020-235662 -
Pediatric Neurosurgery 2019The incidence of bilateral thalamic glioma in children is not reported in the literature. The majority of cases comprise either diffuse astrocytoma, anaplastic... (Review)
Review
The incidence of bilateral thalamic glioma in children is not reported in the literature. The majority of cases comprise either diffuse astrocytoma, anaplastic astrocytoma, or glioblastoma. Partial surgical resection or biopsy followed by adjuvant therapy is the usual treatment for bilateral thalamic gliomas. Prognosis is dependent on tumor grade and extent of tumor spread to surrounding critical structures. We present a rare case of bilateral thalamic pilocytic astrocytoma. Endoscopic biopsy, septostomy, and placement of a ventriculoperitoneal shunt was done followed by radiotherapy. The 36-month follow-up demonstrated radiological control of the tumor.
Topics: Astrocytoma; Brain Neoplasms; Child; Disease Management; Female; Humans; Thalamus; Treatment Outcome
PubMed: 30677772
DOI: 10.1159/000495990 -
Brain Tumor Pathology Apr 2014Dysembryoplastic neuroepithelial tumor (DNT) rarely has aggressive behavior with recurrence. The authors describe a case of DNT with rapid re-growth and features of... (Review)
Review
Dysembryoplastic neuroepithelial tumor (DNT) rarely has aggressive behavior with recurrence. The authors describe a case of DNT with rapid re-growth and features of pilocytic astrocytoma. A 19-year-old girl presented with a 3-year history of complex partial seizure, which was presumably because of DNT located in the left parietal lobe. The tumor was resected, although a tiny residual lesion with cystic space enlarged with ring enhancement identified on MRI 14 days after surgery resulted in re-excision. The recurrent tumor was eventually proved to be predominantly composed of pilocytic astrocytoma. DNT is known to recur only rarely as a tumor with morphologically different components, and no cases of recurrent DNT with rapidly-growing pilocytic astrocytoma have hitherto been reported in the literature. This phenomenon may be histological "replacement" at recurrence.
Topics: Adult; Astrocytoma; Brain Neoplasms; Epilepsies, Partial; Female; Humans; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Neoplasms, Neuroepithelial; Parietal Lobe; Reoperation; Young Adult
PubMed: 23881357
DOI: 10.1007/s10014-013-0155-z -
Child's Nervous System : ChNS :... Jul 2014Pilocytic astrocytomas in the supratentorial compartment make up 20 % of all brain tumours in children with only 5 % of these arising in the suprasellar region. Optic...
INTRODUCTION
Pilocytic astrocytomas in the supratentorial compartment make up 20 % of all brain tumours in children with only 5 % of these arising in the suprasellar region. Optic pathway gliomas or suprasellar gliomas are often seen in neurofibromatosis type 1 (NF1) patients. Given their location, suprasellar pilocytic astrocytomas are challenging to manage surgically with high morbidity rates from surgical resection. We assess our cohort of patients with suprasellar pilocytic astrocytoma and document our experience.
METHOD
A retrospective review of patients diagnosed with suprasellar glioma between 2000-October 2012. We included patients diagnosed with optic pathway glioma based on radiological features (with or without biopsy) and those who had a biopsy confirming pilocytic astrocytoma.
RESULTS
Fifty-three patients included (sporadic tumours 24 and NF1 related 29). Fifteen sporadic and four NF1 patients were biopsied. Twelve sporadic and 13 NF1 patients were initially treated with chemotherapy while only 1 patient had radiotherapy initially. Progression was noted in 58 % of the sporadic group and 24 % of the NF1 group. The only significant factor for progression was NF1 status (p = 0.026).
CONCLUSION
Management should be guided by individual patient circumstance. In our cohort, chemotherapy did not significantly improve progression free survival; however, NF1 status significantly correlated with the decreased progression.
Topics: Adolescent; Antineoplastic Agents; Astrocytoma; Brain Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Disease Progression; Humans; Male; Neurosurgical Procedures; Radiotherapy; Retrospective Studies; Treatment Outcome
PubMed: 24566674
DOI: 10.1007/s00381-014-2374-y -
Child's Nervous System : ChNS :... Jul 2019Juvenile pilocytic astrocytoma (JPA) is a World Health Organization (WHO) grade I tumor that is the commonest to occur in the 0-19 age group, with an excellent prognosis... (Review)
Review
Juvenile pilocytic astrocytoma (JPA) is a World Health Organization (WHO) grade I tumor that is the commonest to occur in the 0-19 age group, with an excellent prognosis of 96% 10-year survival in pediatric patients. Complete resection is the treatment of choice for JPAs. However, this is not always feasible due to the location of certain tumors, and the management following subtotal resection is controversial. Fractionated radiotherapy, chemotherapy, radiosurgery, and observation have all been used to treat tumor remnants. We report a young patient with good tumor control 23 years following low-dose Gamma Knife surgery (GKS) of a subtotally resected brainstem JPA and recommend that GKS may be a feasible treatment option to achieve long-term tumor control when subtotal resection cannot be achieved, even if the GKS prescription dose must be significantly reduced due to large tumor volume or proximity to critical structures sensitive to radiation.
Topics: Astrocytoma; Brain Stem; Brain Stem Neoplasms; Follow-Up Studies; Humans; Infant; Male; Radiosurgery; Treatment Outcome; Young Adult
PubMed: 30997566
DOI: 10.1007/s00381-019-04147-7 -
Neuropathology : Official Journal of... Oct 2019Pilocytic astrocytomas are the commonest childhood brain tumor. They are typically benign and usually are solitary neoplasms. To our knowledge, only one report of a...
Pilocytic astrocytomas are the commonest childhood brain tumor. They are typically benign and usually are solitary neoplasms. To our knowledge, only one report of a pilocytic astrocytoma with leukemia has been previously issued. We herein describe the first case with documented histopathology of a 2-year-old boy who had a cerebellar pilocytic astrocytoma co-localized with an acute B-lymphoblastic leukemia. We speculate that chemotactic migration of leukemic cells to the pilocytic astrocytoma may be partly mediated through vascular endothelial growth factor (VEGF) and VEGF receptors.
Topics: Astrocytoma; Cerebellar Neoplasms; Child, Preschool; Humans; Male; Neoplasms, Multiple Primary; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma
PubMed: 31408921
DOI: 10.1111/neup.12587 -
Journal of Neuroradiology = Journal de... Oct 2015Pilocytic astrocytoma, which is classified as a grade I astrocytic tumor by the World Health Organization, is the most common type of glioma in children and young...
Pilocytic astrocytoma, which is classified as a grade I astrocytic tumor by the World Health Organization, is the most common type of glioma in children and young adults. Pilocytic astrocytoma generally appears as a well-circumscribed, contrast-enhancing lesion, frequently with cystic components on magnetic resonance imaging (MRI). However, it has been reported that the MRI appearance of pilocytic astrocytoma may be similar to that of high-grade gliomas in some cases. We here report on 6 cases of pilocytic astrocytoma with atypical MRI findings, including small cyst formation, heterogeneously enhancing tumor nodules, irregularly enhancing tumor nodules, and enhancing tumor nodules with internal hemorrhage. All tumors were successfully resected, and the histological diagnoses were pilocytic astrocytoma. When the tumor is located near a cerebral cistern or ventricle, the risk of leptomeningeal dissemination is increased. Furthermore, partial resection has also been associated with a higher risk of recurrence and leptomeningeal dissemination. To date, all but one patient are alive and recurrence-free. Because the preoperative diagnosis influences the decision on the extent of resection and because of the high risk of leptomeningeal dissemination associated with these tumors, careful and correct diagnosis by MRI is important.
Topics: Adolescent; Adult; Aged; Astrocytoma; Brain; Brain Neoplasms; Diagnosis, Differential; Diagnostic Errors; Female; Humans; Magnetic Resonance Imaging
PubMed: 25454397
DOI: 10.1016/j.neurad.2014.09.001