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Magyar Onkologia Mar 2024Pineal region tumors account for less than 1% of adult supratentorial tumors. Their treatment requires a multimodality approach. Previously, the treatment of choice was... (Review)
Review
Pineal region tumors account for less than 1% of adult supratentorial tumors. Their treatment requires a multimodality approach. Previously, the treatment of choice was direct surgery, which is associated with high surgical risk. Advances in minimally invasive techniques and onco-radiotherapy offer a safe and multimodal personalized therapy. The aim of our study was to describe the practice of our Institute based on combined endoscopic and radiotherapy techniques. We performed a retrospective clinical study. We processed data from 23 adult patients who underwent endoscopic third ventricle fenestration and pineal tumor biopsy between 2014 and 2023. Descriptive statistics, t-test, Fisher's exact test and Kaplan-Meier analysis were performed. Clinical improvement with endoscopic intervention was achieved in 78.3% of cases. Significant increase in preoperative performance status was observed in the postoperative period (p=2.755e-5), and radiotherapy resulted in regression or stable disease. Our results suggest a safe treatment with good clinical outcome and an excellent alternative to direct surgery.
Topics: Adult; Humans; Brain Neoplasms; Combined Modality Therapy; Pineal Gland; Pinealoma; Retrospective Studies
PubMed: 38484373
DOI: No ID Found -
BMC Pediatrics Mar 2024Germline mutations of NSD1 are associated with Sotos syndrome, characterized by distinctive facial features, overgrowth, and developmental delay. Approximately 3% of...
Germline mutations of NSD1 are associated with Sotos syndrome, characterized by distinctive facial features, overgrowth, and developmental delay. Approximately 3% of individuals with Sotos syndrome develop tumors. In this study, we describe an infant in pineoblastoma with facial anomalies, learning disability and mild autism at 1 years diagnosed as Sotos syndrome owing to carrying a novel mutation de novo germline NSD1 likely pathogenic variant. This patient expands both the mutation and phenotype spectrum of the Sotos Syndrome and provides new clinical insights into the potential mechanism of underlying pinealoblastoma pathology.
Topics: Infant; Humans; Sotos Syndrome; Histone-Lysine N-Methyltransferase; Histone Methyltransferases; Germ-Line Mutation; Pinealoma; Mutation; Brain Neoplasms; Pineal Gland
PubMed: 38459438
DOI: 10.1186/s12887-024-04636-y -
Advances in Radiation Oncology Feb 2024Volumetric modulated arc therapy (VMAT) is a relatively new treatment technique in sub-Saharan Africa. Although craniospinal irradiation (CSI) in the pediatric...
PURPOSE
Volumetric modulated arc therapy (VMAT) is a relatively new treatment technique in sub-Saharan Africa. Although craniospinal irradiation (CSI) in the pediatric population has been practiced in Nigeria for many years, the use of VMAT to deliver this treatment is previously undocumented. We reviewed the first set of patients to undergo CSI at a cancer center in Nigeria, detailing the treatment technique, the progress experienced, dose statistics achieved, treatment toxicities, and cancer outcomes to date.
METHODS AND MATERIALS
This was a prospective case series of 5 children with histologically diagnosed cancers requiring CSI whose parents consented to the study. They were recruited at evaluation and followed through the process of their therapy. Toxicity was monitored at weekly review appointments using the Common Terminology Criteria for Adverse Events version 5.0. Follow-up of the children will continue in the long-term effects clinic.
RESULTS
Five patients with a median age of 6 were recruited. Diagnoses were intracranial germ cell tumor (n = 2), medulloblastoma (n = 1), pineoblastoma (n = 1), and ependymoma (n = 1). For all patients, a dose of 36.0 Gy in 1.8 Gy daily fractions was prescribed to the entire neuraxis. A subsequent boost of 18 Gy (n = 4) to 19.8 Gy (n = 1) in 10 daily fractions to the primary tumor bed (n = 2) and posterior fossa (n = 2) was delivered. Four patients had chemotherapy before, during, or after radiation therapy. No patient experienced grade 3 or greater toxicity.
CONCLUSIONS
Our results indicate great progress has been made in the delivery of CSI in Nigeria, demonstrating tolerable acute side effects using VMAT. This series suggests the feasibility of implementing VMAT technology in low- or middle-income countries. Additional follow-up will be needed to determine whether survival rates and chronic toxicity rates are similar to those reported in the literature.
PubMed: 38405304
DOI: 10.1016/j.adro.2023.101325 -
Asian Journal of Surgery Jun 2024
Topics: Humans; Ependymoma; Pineal Gland; Pinealoma; Brain Neoplasms; Male; Magnetic Resonance Imaging; Female
PubMed: 38388272
DOI: 10.1016/j.asjsur.2024.02.027 -
World Neurosurgery Apr 2024To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors.
METHODS
A systematic review and meta-analysis adhering to Cochrane Standards and PRISMA framework were conducted. PubMed, Embase, and Web Of Science databases were searched until December 2023. Outcomes included rate of histopathologic diagnosis success, ETV success, complications, required VPS, and mortality.
RESULTS
Seventeen studies (N = 388) met inclusion criteria. Histopathologic diagnosis success rate was 90% for general population (95% CI: 86%-95%; I = 42%) and 94% for pediatric patients (95% CI: 89%-98%; I = 19%). ETV Success rate was 93% (95% CI: 88%-97%; I = 60%). An estimated risk of postoperative ETV complications was found to be 16% for the general population (95% CI: 5%-28%; I = 90%) and 5% for pediatric patients (95% CI: 0%-13%; I = 51%). The risk of requiring VPS was estimated as 2% (95% CI: 0%-4%; I = 39%) and for the pediatric population it was 7% (95% CI: 0%-16%; I = 69%). Mortality risk was found to be 1% (95% CI: 0%-3%; I = 0%).
CONCLUSIONS
Simultaneous endoscopic biopsy and ETV demonstrated high diagnostic and therapeutic success rates. The procedure's safety profile, with low mortality and complications, supports its role in treating hydrocephalus associated to pineal region tumors. Subgroup analyses revealed higher diagnostic success rates and required VPS in the pediatric population, whilst it had lower complication rates.
Topics: Child; Humans; Ventriculostomy; Neuroendoscopy; Third Ventricle; Pinealoma; Biopsy; Postoperative Complications; Hydrocephalus; Brain Neoplasms; Pineal Gland; Treatment Outcome; Retrospective Studies
PubMed: 38340795
DOI: 10.1016/j.wneu.2024.02.015 -
Acta Neuropathologica Feb 2024
Topics: Humans; Pinealoma; Pineal Gland; Brain Neoplasms
PubMed: 38340193
DOI: 10.1007/s00401-024-02685-2 -
Acta Neuropathologica Feb 2024
Pineal parenchymal tumors of intermediate differentiation: in need of a stringent definition to avoid confusion. Scientific commentary on 'Genetical and epigenetical profiling identifies two subgroups of pineal parenchymal tumors of intermediate differentiation (PPTID) with distinct molecular,...
Topics: Humans; Pinealoma; Brain Neoplasms; Pineal Gland
PubMed: 38340187
DOI: 10.1007/s00401-024-02684-3 -
Cytopathology : Official Journal of the... May 2024A rare case of pineoblastoma on cerebrospinal fluid cytology was reported in a 15-year-old girl. In the current paper, a rare case of pienoblastoma on CSF cytology has...
A rare case of pineoblastoma on cerebrospinal fluid cytology was reported in a 15-year-old girl. In the current paper, a rare case of pienoblastoma on CSF cytology has been described.
Topics: Female; Humans; Adolescent; Pinealoma; Cytological Techniques; Cytodiagnosis; Brain Neoplasms; Pineal Gland
PubMed: 38308391
DOI: 10.1111/cyt.13364