Long-term survival in a child with a central nervous system medulloepithelioma

2008 ◽  
Vol 2 (5) ◽  
pp. 339-345 ◽  
Author(s):  
Parham Moftakhar ◽  
Xuemo Fan ◽  
Carole H. Hurvitz ◽  
Keith L. Black ◽  
Moise Danielpour
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Treatment Strategies ◽  
High Dose Chemotherapy ◽  
World Health ◽  
High Dose ◽  
Term Survival ◽  
Stem Cell Rescue ◽  
Dismal Prognosis ◽  
Health Organization

Central nervous system medulloepitheliomas are extremely rare and malignant (World Health Organization Grade IV) primitive neuroectodermal tumors (PNETs) that arise in childhood. Unlike other PNETs, medulloepitheliomas have a dismal prognosis, with only 2 reported cases in the literature in which the patient survived beyond 5 years after treatment. The authors report on the third known case of a child who survived longer than 5 years. A review of all the published cases of medulloepithelioma is also presented, and alternative treatment strategies for PNET tumors, including high-dose chemotherapy with stem-cell rescue, are discussed.

scholarly journals Primary Central Nervous System Lymphoma in Elderly Patients: Management and Perspectives

Cancers ◽  
2021 ◽  
Vol 13 (14) ◽  
pp. 3479
Author(s):  
Andrea Morales-Martinez ◽  
Fernando Lozano-Sanchez ◽  
Alberto Duran-Peña ◽  
Khe Hoang-Xuan ◽  
Caroline Houillier
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Elderly Patients ◽  
Treatment Strategies ◽  
Central Nervous System Lymphoma ◽  
High Dose Chemotherapy ◽  
Current Treatment ◽  
Cns Lymphoma ◽  
High Dose ◽  
Consolidation Phase

The management of elderly patients suffering from primary central nervous system (CNS) lymphoma, who represent a rapidly growing population, is challenging. Despite the advances made in PCNSL treatment, the prognosis in older patients remains unsatisfactory. The high risk of systemic and CNS toxicity induced by a high-dose chemotherapy regimen and radiation therapy, respectively, limits the use of consolidation phase treatments in elderly patients and contributes to the poor outcome of these patients. Here, we review the current treatment strategies and ongoing trials proposed for elderly PCNSL patients.

Glial Central Nervous System Tumours of Childhood

2020 ◽  
pp. 179-187
Author(s):  
Dannis van Vuurden ◽  
Darren Hargrave ◽  
Dominik Sturm ◽  
David T.W. Jones
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health ◽  
High Grade ◽  
Dismal Prognosis ◽  
Central Nervous System Tumours ◽  
Current Therapies ◽  
Cns Tumours ◽  
Health Organization ◽  
Future Outcomes

Gliomas are the largest group of paediatric central nervous system (CNS) tumours and can affect any age and location. Currently, they are divided into low and high grade using the World Health Organization (WHO) classification but, increasingly, biology is being used to classify and stratify therapy, and this division may be challenged in the future. Outcomes vary greatly with some low-grade tumours being very indolent, requiring nothing more than careful observation, compared to aggressive high-grade gliomas of the brainstem that have a dismal prognosis. Current therapies are based on surgery, radiotherapy, and cytotoxic chemotherapy but, increasingly, biologically targeted therapies are being explored in an attempt to increase survival and decrease late effects and the burden of treatment.

Primary Central Nervous System Lymphoma

2018 ◽  
Vol 38 (01) ◽  
pp. 086-094 ◽  
Author(s):  
Tracy Batchelor ◽  
Sarah Löw
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Mammalian Target Of Rapamycin ◽  
Treatment Strategies ◽  
Central Nervous System Lymphoma ◽  
High Dose Chemotherapy ◽  
Genetic Alterations ◽  
High Dose ◽  
Non Hodgkin Lymphoma ◽  
Whole Brain Radiation

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma limited to the brain, spinal cord, leptomeninges, and eyes. The majority of patients are immunocompetent, with a median age of 65 years at diagnosis. Historically, whole-brain radiation therapy (WBRT) was the first and sole treatment for PCNSL. Today, due to the recognized neurotoxicity of WBRT, this modality is usually avoided in the treatment. Most chemotherapy regimens are based on high-dose methotrexate plus the anti-CD20 monoclonal antibody rituximab, leading to high response rates, but 5-year survival is still poor at approximately 30% compared with other extranodal lymphomas. New treatment strategies including high-dose chemotherapy/autologous stem cell transplantation, targeted therapies focusing on, for example, genetic alterations in B cells or mammalian target of rapamycin signaling, and immunotherapy with inhibitors of the programmed cell death 1 receptor are only a few options to improve the armamentarium against PCNSL.

scholarly journals Challenges in the Treatment of Newly Diagnosed and Recurrent Primary Central Nervous System Lymphoma

2020 ◽  
Vol 18 (11) ◽  
pp. 1571-1578
Author(s):  
Matthias Holdhoff ◽  
Maciej M. Mrugala ◽  
Christian Grommes ◽  
Thomas J. Kaley ◽  
Lode J. Swinnen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Central Nervous System Lymphoma ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Newly Diagnosed ◽  
Phase Ii Trials ◽  
Whole Brain Radiation ◽  
The Central Nervous System

Primary central nervous system lymphomas (PCNSLs) are rare cancers of the central nervous system (CNS) and are predominantly diffuse large B-cell lymphomas of the activated B-cell (ABC) subtype. They typically present in the sixth and seventh decade of life, with the highest incidence among patients aged >75 years. Although many different regimens have demonstrated efficacy in newly diagnosed and relapsed or refractory PCNSL, there have been few randomized prospective trials, and most recommendations and treatment decisions are based on single-arm phase II trials or even retrospective studies. High-dose methotrexate (HD-MTX; 3–8 g/m2) is the backbone of preferred standard induction regimens. Various effective regimens with different toxicity profiles can be considered that combine other chemotherapies and/or rituximab with HD-MTX, but there is currently no consensus for a single preferred regimen. There is controversy about the role of various consolidation therapies for patients who respond to HD-MTX–based induction therapy. For patients with relapsed or refractory PCNSL who previously experienced response to HD-MTX, repeat treatment with HD-MTX–based therapy can be considered depending on the timing of recurrence. Other more novel and less toxic regimens have been developed that show efficacy in recurrent disease, including ibrutinib, or lenalidomide ± rituximab. There is uniform agreement to delay or avoid whole-brain radiation therapy due to concerns for significant neurotoxicity if a reasonable systemic treatment option exists. This article aims to provide a clinically practical approach to PCNSL, including special considerations for older patients and those with impaired renal function. The benefits and risks of HD-MTX or high-dose chemotherapy with autologous stem cell transplantation versus other, better tolerated strategies are also discussed. In all settings, the preferred treatment is always enrollment in a clinical trial if one is available.

scholarly journals Pilomyxoid Astrocytoma Occurring in the Third Ventricle

2015 ◽  
Vol 5 ◽  
pp. 41
Author(s):  
Sanghyeon Kim ◽  
Myongjin Kang ◽  
Sunseob Choi ◽  
Dae Cheol Kim
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
World Health Organization ◽  
Third Ventricle ◽  
World Health ◽  
Central Nervous System Tumor ◽  
Pilomyxoid Astrocytoma ◽  
The Third ◽  
The Central Nervous System ◽  
Health Organization

Pilomyxoid astrocytoma (PMA) is a rare central nervous system tumor that has been included in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. Due to its more aggressive behavior, PMA is classified as Grade II neoplasm by the World Health Organization. PMA predominantly affects the hypothalamic/chiasmatic region and occurs in children (mean age of occurrence = 10 months). We report a case of a 24-year-old man who presented with headache, nausea, and vomiting. Brain CT and MRI revealed a mass occupying only the third ventricle. We performed partial resection. Histological findings, including monophasic growth with a myxoid background, and absence of Rosenthal fibers or eosinophilic granular bodies, as well as the strong positivity for glial fibrillary acidic protein were consistent with PMA.

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