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Intracranial germ cell tumors are rare brain tumors that are distinguished based on their histology and selected tumor markers. Non-germinomatous germ cell tumors are a diverse group of such tumors having the poorest prognosis. Most commonly, they are located in the suprasellar and pineal regions. Since the exact treatment protocol has not yet been established, there is currently no standardized modality of management. We present a case of intracranial multifocal non-germinomatous germ cell tumor in an 18-year-old male, along with relevant literature review. We describe initial diagnostic and treatment procedures in a young adult presented with diplopia and ataxic gait. Neuroradiological findings and elevated alpha fetoprotein and beta chain of the human chorionic gonadotropin tumor markers indicated the possible mixed germ cell tumor. Chemotherapy regimen was adjusted accordingly, biopsy was not performed. The patient's clinical condition improved significantly and his alpha fetoprotein values decreased remarkably after initiation of chemotherapy. In conclusion, initial evaluation with neuroimaging, tumor markers, and cytology from cerebrospinal fluid is important as guidance to further treatment and prognosis. In selected cases, biopsy may not be indicated to start adjuvant chemotherapy. We emphasize the importance of specific treatment modality selection based mainly on tumor markers, regardless of the precise histologic classification.
Assuntos
Neoplasias Encefálicas , Neoplasias Embrionárias de Células Germinativas , Masculino , Adulto Jovem , Humanos , Adolescente , alfa-Fetoproteínas/uso terapêutico , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/terapia , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/terapia , Prognóstico , Biomarcadores TumoraisRESUMO
INTRODUCTION: Adult hemangioblastomas are rare WHO central nervous system (CNS) Grade 1 tumors particularly affecting the posterior cranial fossa. They exhibit a gender bias, impacting men in their fifth and sixth decades of life and manifesting sporadically or as part of von Hippel Lindau (VHL) disease. Understanding the intricacies of CNS hemangioblastomas is crucial for clinical decision-making. METHODS: A systematic review of 576 articles was conducted following PRISMA guidelines. Eligibility criteria included 3189 adult cases of CNS hemangioblastomas. Data on patient demographics, tumor characteristics, symptoms, treatment modalities, complications, and outcomes were systematically extracted and synthesized. RESULTS: The review revealed a heterogeneous demographic distribution, with a male predominance. Median age at diagnosis was 44.7 years. Cranial hemangioblastomas were more commonly located in the infratentorial (73 %) than supratentorial (27%) compartments. Spinal hemangioblastomas were mostly located in the cervical spine (44.3 %), followed by thoracic (36.7 %) and lumbar spine (12 %). Clinical symptoms varied by location, emphasizing the importance of anatomical considerations. Surgical intervention-total resection (82% of cases)-was the preferred treatment modality, while radiotherapy was less common. Histological examination and immunohistochemistry aided in accurate diagnosis. Complications were location-specific, with intracranial complications more common in infratentorial tumors. Overall, favorable outcomes were prevalent (78% of cases), with low mortality rates. CONCLUSION: Adult CNS hemangioblastomas present with diverse characteristics and clinical manifestations. Surgical intervention remains the mainstay treatment; ongoing research into genetic and molecular mechanisms may enhance our understanding of tumor pathology and lead to improved management strategies in the future.
RESUMO
Introduction: Planning cranioplasty (CPL) in patients with suspected or proven post-traumatic hydrocephalus (PTH) poses a significant management challenge due to a lack of clear guidance. Research question: This project aims to create a European document to improve adherence and adapt to local protocols based on available resources and national health systems. Methods: After a thorough non-systematic review, a steering committee (SC) formed a European expert panel (EP) for a two-round questionnaire using the Delphi method. The questionnaire employed a 9-point Likert scale to assess the appropriateness of statements inherent to two sections: "Diagnostic criteria for PTH" and "Surgical strategies for PTH and cranial reconstruction." Results: The panel reached a consensus on 29 statements. In the "Diagnostic criteria for PTH" section, five statements were deemed "appropriate" (consensus 74.2-90.3 %), two were labeled "inappropriate," and seven were marked as "uncertain."In the "Surgical strategies for PTH and cranial reconstruction" section, four statements were considered "appropriate" (consensus 74.2-90.4 %), six were "inappropriate," and five were "uncertain." Discussion and conclusion: Planning a cranioplasty alongside hydrocephalus remains a significant challenge in neurosurgery. Our consensus conference suggests that, in patients with cranial decompression and suspected hydrocephalus, the most suitable diagnostic approach involves a combination of evolving clinical conditions and neuroradiological imaging. The recommended management sequence prioritizes cranial reconstruction, with the option of a ventriculoperitoneal shunt when needed, preferably with a programmable valve. We strongly recommend to adopt local protocols based on expert consensus, such as this, to guide patient care.