scholarly journals Intracranial pilocytic astrocytoma with spinal drop metastasis: A case review, discussion of its current management and related literature

2022 ◽  
Vol 7 (4) ◽  
pp. 129-132
Author(s):  
Shekhar Kumar Keshri ◽  
Satyendra Narayan Sinha
Keyword(s):  
Pilocytic Astrocytoma ◽  
Benign Tumor ◽  
Obstructive Hydrocephalus ◽  
Primary Site ◽  
Low Grade ◽  
Case Review ◽  
Current Management ◽  
Hypothalamic Region ◽  
Drop Metastasis ◽  
Ventriculo Peritoneal Shunt

Pilocytic astrocytoma (PA) is a low grade benign tumor, commonly occurs in Cerebellum (42-60%), Optic & hypothalamic region (9-30%), Brain Stem (9%). They rarely spread. It rarely occurs within the ventricle. In this report, we described our experience with cranial pilocytic astrocytoma in a 14-year-old child, who initially treated with near total decompression of tumor. Four and half years later, recurrence of primary tumor with obstructive hydrocephalus occurred for which he underwent ventriculo-peritoneal shunt followed by re-excision. As further treatment was being evaluated for radiotherapy in view of recurrence at the primary site, he got detected to have significant spinal drop metastasis seen on MRI Spine. Finally, he underwent craniospinal irradiation (CSI).

scholarly journals LGG-23. EXCELLENT CLINICAL / RADIOLOGICAL RESPONSE TO BRAF INHIBITION IN A YOUNG CHILD WITH IN-OPERABLE SUPRA-SELLAR PILOCYTIC ASTROCYTOMA

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii370-iii371
Author(s):  
Stacy Chapman ◽  
Demitre Serletis ◽  
Colin Kazina ◽  
Mubeen Rafay ◽  
Sherry Krawitz ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Pilocytic Astrocytoma ◽  
Clinical Symptoms ◽  
Obstructive Hydrocephalus ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
Low Grade ◽  
Dramatic Improvement ◽  
Chemotherapeutic Regimen ◽  
Response To Chemotherapy

Abstract In-operable low grade gliomas (LGG) in the pediatric population continue to present a treatment dilemma. Due to the low-grade nature of these tumors, and variable response to chemotherapy / radiation, the choice of adjuvant treatment is difficult. Overall survival is directly related to the degree of surgical resection, adding complexity to these inoperable tumors. Current chemotherapeutic regimen for these inoperable tumors includes vincristine (VCR) and carboplatin (Carbo). With advancements in the molecular characterization of gliomas, the role of targeted therapy has come into question. We present a 2-year-old female with biopsy proven Pilocytic Astrocytoma (positive BRAF-V600E mutation) involving the hypothalamic/optic chiasm region. She presented with ataxic gait, bi-temporal hemianopia, obstructive hydrocephalus and central hypothyroidism, which progressed to altered consciousness, and right hemiparesis due to location/mass effect of the tumor. She was initially treated with chemotherapy (VCR/Carbo) but her tumor progressed at 6 weeks of treatment. As her tumor was positive for BRAF-V600E mutation, she was started on Dabrafenib monotherapy, resulting in dramatic improvement in her clinical symptoms (able to stand, improved vision), and a 60% reduction in tumor size at 3-months. At 6-months, follow up MRI showed slight increase in the solid portion of the tumor, with no clinical symptoms. We plan to add MEK inhibitor (Trametinib) and continue with Dabrafenib. Our experience and literature review suggests that LGG with BRAF-V600E mutations may benefit from upfront targeted therapy. Prospective clinical trials comparing the efficacy of BRAF inhibitors versus standard chemotherapy in LGG with BRAF mutations are urgently needed.

scholarly journals Neuroendoscopic followed by microsurgical approach for the treatment of pilocytic astrocytoma of the pineal region

2021 ◽  
Vol 3 (3(September-December)) ◽  
pp. e882021
Author(s):  
Leopoldo Mandic Furtado ◽  
José Aloysio Da Costa Val Filho ◽  
François Dantas ◽  
Camila Moura De Sousa
Keyword(s):  
Pilocytic Astrocytoma ◽  
Obstructive Hydrocephalus ◽  
Pineal Region ◽  
Third Ventriculostomy ◽  
Low Grade ◽  
Anatomical Landmarks ◽  
Tumor Biopsy ◽  
Life Threatening ◽  
Microsurgical Approach ◽  
Supracerebellar Infratentorial Approach

Pilocytic astrocytoma is a low-grade tumor that can affect the pineal region and has a potential life-threatening presentation with obstructive hydrocephalus. This video presents the case of a child who underwent an endoscopic third ventriculostomy with tumor biopsy and for whom a supracerebellar infratentorial approach was chosen after confirmation of the diagnosis of a pilocytic astrocytoma. Herein, we discuss the technical details of both procedures, such as the single burr hole in the neuroendoscopic approach, the ventricular landmarks and identification of the venous sinus through neuronavigation, the anatomical landmarks during the supracerebellar infratentorial approach, and the use of ultrasonographic aspiration.

scholarly journals Pilomyxoid astrocytoma: diagnosis, prognosis, and management

2005 ◽  
Vol 18 (6) ◽  
pp. 1-4 ◽  
Author(s):  
Ricardo J. Komotar ◽  
J Mocco ◽  
Jess E. Jones ◽  
Brad E. Zacharia ◽  
Tarik Tihan ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Pilocytic Astrocytoma ◽  
Pediatric Brain Tumor ◽  
Low Grade ◽  
Current Management ◽  
Pilomyxoid Astrocytoma ◽  
Histological Features ◽  
Unusual Variant ◽  
Prognostic Implications ◽  
Pediatric Brain

Pilomyxoid astrocytoma (PMA) is a recently defined pediatric brain tumor; PMAs were previously classified within the pilocytic astrocytoma (PA) category. Nevertheless, PMA has different histological features and has been shown to behave more aggressively than PA. These findings indicate that PMA may be a unique entity that is distinct from PA, or it may be an unusual variant. To increase awareness of PMA within the neurosurgical community, the authors review the diagnostic criteria, prognostic implications, and current management of this recently described pediatric low-grade astrocytoma.

scholarly journals RADT-29. EFFECT OF RADIATION THERAPY ON OVERALL SURVIVAL FOLLOWING SUBTOTAL RESECTION OF ADULT PILOCYTIC ASTROCYTOMA

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii187-ii188
Author(s):  
Adham Khalafallah ◽  
Adrian Jimenez ◽  
Henry Brem ◽  
Debraj Mukherjee
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Pilocytic Astrocytoma ◽  
Radiation Treatment ◽  
Cox Proportional Hazards Model ◽  
Adult Patients ◽  
Subtotal Resection ◽  
Adjuvant Radiation ◽  
Low Grade ◽  
Risk Of Death

Abstract BACKGROUND Pilocytic astrocytoma (PCA) is a low-grade glioma common in children but also rarely diagnosed in adults. The role of adjuvant radiation therapy (RT) in treating these tumors remains unclear. OBJECTIVE We investigated the effect of RT on overall survival, specifically among adult patients who had undergone subtotal PCA resection. METHODS Information on adult patients (age 18 years old) who had undergone subtotal PCA resection between 2004 and 2016 was collected from the National Cancer Database (NCDB). A multivariate Cox proportional hazards model was utilized to determine factors independently associated with overall survival. RESULTS A total of 451 patients were identified. The mean age of our patient cohort was 36.8 years old, and the majority of patients (83.4%) did not receive radiation treatment following subtotal PCA resection. Overall median survival was 93.8 months. Survival was longer (p < 0.001) in the patients who did not receive post-surgical RT (median: 98.3 months) compared to patients who did (median: 54.8 months). Patients who had older age at diagnosis (hazard ratio [HR]=1.05, 95% confidence interval [CI]=1.03-1.07, p < 0.01), were Black or African American (HR=2.76, CI=1.12-6.46, p=0.019), received radiation during their initial treatment (HR=4.53, CI=2.08-9.89, p < 0.01), or had a Charlson/Deyo score of > 1 (HR=3.68, CI=1.55, p=0.003) had a significantly higher risk of death following subtotal PCA resection. CONCLUSION Postoperative RT is independently associated with a significantly higher risk of death among adults who underwent subtotal PCA resection. Our findings provide a rationale for further investigation into the efficacy and safety of RT within this patient population.

scholarly journals LGG-28. NOVEL BRAF INTRAGENIC DELETION IN A GLIOMA: A CASE REPORT OF A PEDIATRIC PATIENT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii371-iii372
Author(s):  
Valerie Cruz Flores ◽  
Maxine Sutcliffe ◽  
Thomas Geller ◽  
Ignacio Gonzalez Gomez ◽  
Stephanie Smith ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Pilocytic Astrocytoma ◽  
Pediatric Population ◽  
Binding Pocket ◽  
Genetic Alterations ◽  
Low Grade ◽  
Braf Gene ◽  
Intragenic Deletion ◽  
Whole Genome Microarray ◽  
Intragenic Deletions

Abstract BACKGROUND Numerous variant BRAF genetic alterations have been associated with malignancies. BRAF activating fusions/mutations are frequently present in low grade gliomas. BRAF intragenic deletions have been reported in melanoma, but have not previously been reported in gliomas. OBJECTIVE To report a BRAF intragenic deletion in a pediatric patient with recurrent low-grade glioma. RESULTS A 3-year-old female underwent a complete resection of a posterior fossa pilocytic astrocytoma. She had recurrences at age 4, and then at age 9; pathology was consistent with pilocytic astrocytoma. Microarray analysis on sample from the first recurrence showed one region of loss encompassing 86 Kbp within the BRAF gene. The deletion breakpoints are within intron 1 and 9, resulting in loss of exons 2 through 9, inclusive. This has been previously described melanoma, but appears to be a novel finding in glioma. It is hypothesized that, since the loss retains the kinase and ATP binding pocket domains but deletes the N-terminal conserved region 1 and 2 (CR1, CR2) of the BRAF gene, it is likely functionally similar to the loss and activation resulting from the more usually described KIAA1549 and BRAF gene fusion. CONCLUSION This is the first BRAF intragenic deletion involving exons 2–9 reported in a glioma. Although 86kbp is small using whole genome microarray technology, it is large using sequencing strategies, and a targeted sequencing approach to investigate the BRAF gene would not readily identify this deletion. It is speculated that the deletion may be under ascertained in the pediatric population.

scholarly journals Anaplastic pilocytic astrocytoma of the cerebellum presenting with conus medullaris drop metastasis

2021 ◽  
Vol 8 (1) ◽  
pp. 10
Author(s):  
Jared F. Sweeney ◽  
Vaibhav Chumbalkar ◽  
Michael D. Staudt ◽  
Pouya Entezami ◽  
Jiang Qian ◽  
...  
Keyword(s):  
Pilocytic Astrocytoma ◽  
Cauda Equina ◽  
Conus Medullaris ◽  
Complete Understanding ◽  
First Case ◽  
Spine Imaging ◽  
Drop Metastasis ◽  
Cerebellar Mass ◽  
Definition Of ◽  
Staged Operations

Due to its rarity, a complete understanding of the clinical behavior, pathogenesis, and diagnostic definition of anaplastic pilocytic astrocytoma (APA) is currently lacking. The optimal clinical management and use of adjuvant therapies has yet to be defined. We present a 64 year-old-female with progressive headaches, dysarthria, and ataxia, who was found to have right cerebellar mass. A gross total resection was achieved through two staged operations. Pathology demonstrated focal areas of necrosis, tumor infiltration, and increased mitotic activity most consistent with APA. Adjuvant chemotherapy and stereotactic radiosurgery were administered. Approximately two years later, the patient presented with symptoms of cauda equina syndrome, and lumbar spine imaging demonstrated a large intradural mass at the conus medullaris with diffuse leptomeningeal enhancement. A biopsy was performed and was consistent with metastatic APA. APA may rarely progress to metastatic disease, most frequently involving the leptomeninges of the posterior fossa and cervical spine. This report represents the first case of metastases distal to the cervicomedullary junction.

scholarly journals Low-grade fibromyxoid sarcoma: A rare case in an unusual location

2020 ◽  
Vol 8 ◽  
pp. 2050313X2094431
Author(s):  
Diandra Perez ◽  
Ola El-Zammar ◽  
Brando Cobanov ◽  
Rana Naous
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Rare Case ◽  
Primary Site ◽  
Low Grade ◽  
Deep Soft Tissue ◽  
Unusual Location ◽  
Fibromyxoid Sarcoma ◽  
Intrathoracic Mass

Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.

scholarly journals Neurotrophic Receptor Tyrosine Kinase 2 (NTRK2) Alterations in Low-Grade Gliomas: Report of a Novel Gene Fusion Partner in a Pilocytic Astrocytoma and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Siobhan S. Pattwell ◽  
Eric Q. Konnick ◽  
Yajuan J. Liu ◽  
Rebecca A. Yoda ◽  
Laligam N. Sekhar ◽  
...  
Keyword(s):  
Pilocytic Astrocytoma ◽  
Gene Fusion ◽  
Pediatric Population ◽  
Neurofibromatosis Type ◽  
Genetic Alterations ◽  
Neurotrophin Receptor ◽  
Low Grade ◽  
Fusion Partner ◽  
Tyrosine Kinase 2 ◽  
Cns Neoplasms

Pilocytic astrocytoma is a low-grade glial neoplasm of the central nervous system (CNS) that tends to occur in the pediatric population and less commonly presents in adults. Hereditary pilocytic astrocytoma is often associated with germline genetic alterations in the tumor suppressor NF1, the gene responsible for the syndrome neurofibromatosis type 1. Sporadic pilocytic astrocytoma frequently harbors somatic alterations in BRAF, with rare pilocytic astrocytomas containing alterations in FGFR1 and NTRK2. NTRK2 encodes for the protein tropomyosin receptor kinase B (TrkB), which is a neurotrophin receptor with high affinity for Brain-Derived Neurotrophic Factor (BDNF), and plays a role in several physiological functions of neurons, including cell survival and differentiation. In this report, we describe a novel PML-NTRK2 gene fusion occurring in an adult sporadic pilocytic astrocytoma and review the biology and implications of specific NTRK2 mutations occurring in CNS neoplasms.

P3 Outcomes following diagnostic brain biopsy: a 1 year case review

2019 ◽  
Vol 90 (3) ◽  
pp. e27.3-e27
Author(s):  
R Ingleton ◽  
B Gorgun ◽  
N Gorgun ◽  
M Hayder ◽  
RR Vindlacheruvu
Keyword(s):  
Brain Biopsy ◽  
Low Grade ◽  
Case Review ◽  
Electronic Patient Records ◽  
Stereotactic Brain Biopsy ◽  
Neurosurgical Procedure ◽  
Tissue Diagnosis ◽  
Radiology Reports ◽  
Crucial Component ◽  
Management Plans

ObjectivesStereotactic brain biopsy is an established neurosurgical procedure utilised in times of diagnostic or therapeutic uncertainty. Achieving a definitive tissue diagnosis allows tailored surgical, medical or oncological management. We present a 1 year case review of brain biopsies and compare suspected pre-operative diagnosis with final tissue outcome.DesignRetrospective analysis of all brain biopsies performed in 2017 in an adult-only neurosurgical unit.SubjectsAll patients undergoing brain biopsy.MethodsTheatre records were retrospectively analysed to identify patients undergoing brain biopsy in 2017. Further information was obtained using electronic patient records and radiology reports.Results41 patients underwent brain biopsy in 2017. Only 1 proved inconclusive and required repeat procedure. 26 patients had a suspected diagnosis documented pre-operatively based on radiological/clinical evidence. The most commonly suspected pathologies were low grade and high grade gliomas (9 and 15 patients). Of 25 patients with both a pre-operative differential and subsequent conclusive tissue diagnosis, 10 (40%) were ultimately given diagnoses which did not match the suspected pathology. Significantly, 1 biopsy diagnosed cerebral lymphoma.ConclusionsBrain biopsy is a relatively safe and simple neurosurgical procedure which can reliably provide a definitive tissue diagnosis. We have demonstrated how it remains a crucial component in formally establishing diagnosis prior to determining optimum management plans.

scholarly journals Neuroendoscopic lavage and continuous drainage of ventricles: Treatment of pyocephalus in a newborn

2019 ◽  
Vol 1 (1) ◽  
pp. 55-59
Author(s):  
Ram Babu Joshi ◽  
Rupendra Bahadur Adhikari ◽  
Amit Thapa
Keyword(s):  
Clinical Cure ◽  
Obstructive Hydrocephalus ◽  
Primary Treatment ◽  
Direct Visualization ◽  
Pyogenic Meningitis ◽  
Continuous Drainage ◽  
Life Threatening ◽  
Life Threatening Complication ◽  
Ventriculo Peritoneal Shunt

Pyocephalus/Cerebral ventricular empyema is a serious life threatening complication of acute pyogenic meningitis. The primary treatment of ventriculitis is administration of antibiotics. With recent advances, neuroendoscopic lavage (NEL) of ventricles through direct visualization has helped save lives when multipronged approaches including intravenous (IV) antibiotics, intrathecal antibiotics and continuous drainage of cerebrospinal fluid (CSF) fails. We report a case of a 23-day old neonate who developed pyocephalus as a complication of pyogenic meningitis. He did not respond favorably to initial IV antibiotic treatment for two weeks. NEL of ventricles was performed. Thick pus/flakes inside the ventricles had caused obstructive hydrocephalus. Continuous CSF drainage was done through strategically placed multiple external ventricular drains. Interval ventriculo-peritoneal shunt was done bilaterally after the CSF was macroscopically/ microscopically clear of visible debris, and sterile. Microbiological and clinical cure was achieved and the child survived and is thriving well at last follow-up at the age of 6 months.

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