Neuroepithelial Neoplasms (Gliomatosis Cerebri)

Neoplasms composed of neuroepithelial cells, which have the capacity to differentiate into NEURONS, oligodendrocytes, and ASTROCYTES. The majority of craniospinal tumors are of neuroepithelial origin. (From Dev Biol 1998 Aug 1;200(1):1-5)

Also Known As:

Gliomatosis Cerebri; Neoplasms, Neuroepithelial; Neuroepithelial Tumors; Astroblastomas; Ependymoastrocytomas; Neoplasm, Neuroepithelial; Neuroepithelial Neoplasm; Neuroepithelial Tumor; Polar Spongioblastoma; Polar Spongioblastomas; Spongioblastomas, Polar; Tumor, Neuroepithelial; Tumors, Neuroepithelial; Astroblastoma; Ependymoastrocytoma; Spongioblastoma, Polar

Networked: 412 relevant articles (16 outcomes, 33 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neuroepithelial Neoplasms (Gliomatosis Cerebri)
2.	Glioma (Gliomas)
3.	Focal Cortical Dysplasia
4.	Seizures (Absence Seizure)
5.	Venous Thromboembolism

Experts

1.	Nobusawa, Sumihito: 6 articles (10/2021 - 12/2018)
2.	Schittenhelm, Jens: 5 articles (04/2022 - 08/2007)
3.	Harding, Brian N: 5 articles (12/2019 - 11/2003)
4.	Suh, Yeon-Lim: 5 articles (11/2015 - 11/2005)
5.	Chen, Li: 5 articles (03/2010 - 07/2005)
6.	Biegel, Jaclyn A: 4 articles (01/2021 - 07/2010)
7.	Santi, Mariarita: 4 articles (12/2019 - 07/2010)
8.	Capper, David: 4 articles (10/2019 - 01/2014)
9.	Kakita, Akiyoshi: 4 articles (05/2019 - 01/2013)
10.	Tominaga, Teiji: 4 articles (05/2019 - 08/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neuroepithelial Neoplasms:

1.	Temozolomide (Temodar)FDA LinkGeneric 11/27/2001 - "Possible efficacy of temozolomide in a patient with gliomatosis cerebri." 02/19/2008 - "Up-front temozolomide (TMZ) has been recently proposed as a treatment for gliomatosis cerebri (GC), but no predictive or prognostic markers have been identified so far. " 12/01/2003 - "Response to low dose temozolomide in radiation induced gliomatosis cerebri." 11/01/2003 - "A 46-year-old man with gliomatosis cerebri was treated with temozolomide (200 mg/m(2) per day for 5 days every 28 days). " 11/01/2003 - "Gliomatosis cerebri: clinical, neurochemical and neuroradiological response to temozolomide administration."
2.	2,6-dinitrotoluene (DNT)IBA 08/01/2012 - "To study the clinicopathologic features, radiologic findings, treatment options and prognosis of dysembryoplastic neuroepithelial tumor (DNT). " 08/01/2007 - "To study the clinicopathologic features, immunophenotype and histogenesis of dysembryoplastic neuroepithelial tumor (DNT). " 09/01/2005 - "To study the clinicopathologic features and radiologic findings of dysembryoplastic neuroepithelial tumor (DNT). " 09/01/2005 - "OBJECTIVE To study the clinicopathologic features, radiologic findings, treatment modalities and prognosis of dysembryoplastic neuroepithelial tumor (DNT). " 01/01/1999 - "Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases."
3.	DNA (Deoxyribonucleic Acid)IBA 05/01/2016 - "Thus, TMZ/DNA-NLCs could prove to be a superior co-delivery nanomedicine to achieve therapeutic efficacy and this report could be a new promising strategy for treatment in malignant gliomatosis cerebri." 05/01/2016 - "Also TMZ/DNA-NLCs transfer both drug and gene to the gliomatosis cerebri, enhance the antitumor capacity and gene transfection efficacy. " 03/01/1995 - "Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical, and deoxyribonucleic acid analyses in a pediatric series." 07/09/1991 - "We therefore suggest that the flow cytometric measurement of DNA-ploidy has relevant significance in predicting survival in patients treated for intracranial neuroepithelial tumors." 07/09/1991 - "Prognostic significance of nuclear DNA content in human neuroepithelial tumors."
4.	Procarbazine (Matulane)FDA Link 01/01/2008 - "Procarbazine and CCNU as initial treatment in gliomatosis cerebri." 09/01/2011 - "The NOA-05 multicenter trial was performed to analyze the efficacy of primary chemotherapy with procarbazine and lomustine (PC) in patients with gliomatosis cerebri (GC) and to define clinical, imaging, and molecular factors influencing outcome. " 09/01/2011 - "NOA-05 phase 2 trial of procarbazine and lomustine therapy in gliomatosis cerebri." 10/01/2014 - "Procarbazine, CCNU, and Vincristine Chemotherapy in Gliomatosis Cerebri." 10/01/2002 - "We conclude that (1) the molecular genetic alterations in gliomatosis cerebri resemble those in diffuse astrocytomas; (2) the prognosis of gliomatosis cerebri is variable but for at least 50% of patients as poor as for glioblastoma; and (3) some patients respond to radiotherapy and/or procarbazine, carmustine, vincristine chemotherapy."
5.	Lomustine (CCNU)FDA Link 01/01/2008 - "Procarbazine and CCNU as initial treatment in gliomatosis cerebri." 09/01/2011 - "The NOA-05 multicenter trial was performed to analyze the efficacy of primary chemotherapy with procarbazine and lomustine (PC) in patients with gliomatosis cerebri (GC) and to define clinical, imaging, and molecular factors influencing outcome. " 09/01/2011 - "NOA-05 phase 2 trial of procarbazine and lomustine therapy in gliomatosis cerebri." 10/01/2014 - "Procarbazine, CCNU, and Vincristine Chemotherapy in Gliomatosis Cerebri."
6.	Biomarkers (Surrogate Marker)IBA 01/01/2023 - "Identification of distinct genetic and epigenetic profiles in various neuroepithelial tumors has improved the classification and uncovered novel diagnostic, prognostic, and predictive molecular biomarkers for improved prediction of treatment response and outcome. " 11/01/2022 - "Latest impactful studies identifying new diagnostic or prognostic biomarkers in frequent tumors and describing new rare tumor types are summarized about adult and pediatric gliomas, rare neuroepithelial tumors, ependymomas, medulloblastomas and meningiomas. "
7.	Methionine (L-Methionine)FDA Link 01/01/2020 - "BRAF V600E mutation mediates FDG-methionine uptake mismatch in polymorphous low-grade neuroepithelial tumor of the young." 06/01/1998 - "18-FDG and 11C methionine PET scans were performed on two patients with gliomatosis cerebri. " 10/01/2014 - "Accuracy of distinguishing between dysembryoplastic neuroepithelial tumors and other epileptogenic brain neoplasms with [¹¹C]methionine PET." 01/01/2004 - "Usefulness of [11C]methionine PET in the diagnosis of dysembryoplastic neuroepithelial tumor with temporal lobe epilepsy." 01/01/2004 - "We assessed the diagnostic value of [11C]methionine (MET) positron emission tomography (PET) in the differential diagnosis of dysembryoplastic neuroepithelial tumors (DNETs) among benign tumors associated with temporal lobe epilepsy (TLE). "
8.	SteroidsIBA 12/01/1998 - "Steroid therapy may be an effective treatment for gliomatosis cerebri before the terminal stage." 12/01/1998 - "Secondary glioblastoma remarkably reduced by steroid administration after anaplastic transformation from gliomatosis cerebri--case report." 11/01/1990 - "Characteristics and biological role of steroid hormone receptors in neuroepithelial tumors." 07/01/1993 - "We studied the expression of ten genes (encoding the receptors for glucocorticoid, mineralocorticoid, progesterone, androgen, estrogen, thyroid, retinoid acid, vitamin D) belonging to the steroid-thyroid hormone receptor superfamily (STRS) in 12 neuroepithelial tumors, 12 meningiomas and 2 human glioblastoma cell lines. "
9.	Choline (Choline Chloride)IBA 02/01/2011 - "¹¹C-Methionine or ¹¹C-Choline PET is superior to MRI in the evaluation of gliomatosis cerebri." 01/01/2004 - "A patient with histologically proven gliomatosis cerebri presented with a normal choline level but a markedly abnormal elevated myo-inositol level on magnetic resonance (MR) spectroscopy. " 01/01/2004 - "Role of proton magnetic resonance spectroscopy in the diagnosis of gliomatosis cerebri: a unique pattern of normal choline but elevated Myo-inositol metabolite levels." 05/01/2003 - "Proton MR spectroscopy of gliomatosis cerebri: case report of elevated myoinositol with normal choline levels." 05/01/2003 - "The primary conclusion is that in suspected cases of gliomatosis cerebri, myo-inositol/creatine and myo-inositol/N-acetyl should be determined because they may provide evidence of tumor, even though choline/creatine is normal. "
10.	Antineoplastic Agents (Antineoplastics)IBA 11/01/2003 - "Temozolomide has shown antineoplastic activity against malignant gliomas and more recently was beneficial in one patient with gliomatosis cerebri. "

Therapies and Procedures

1.	Radiotherapy 09/01/2014 - "This study showed that gliomatosis cerebri could be effectively treated with radiotherapy and that initial response to radiotherapy was a significant prognostic factor affecting the survival." 05/01/2006 - "These cases suggest that radiation therapy is effective for gliomatosis cerebri." 09/01/2014 - "To evaluate the efficacy of radiotherapy and factors affecting the prognosis of gliomatosis cerebri. " 07/15/2003 - "Gliomatosis cerebri: improved outcome with radiotherapy." 01/01/2000 - "Radiation therapy in gliomatosis cerebri is associated with a temporary improvement in or stabilization of clinical symptoms in the majority of cases. "
2.	Drug Therapy (Chemotherapy) 10/01/2022 - "Patients with high-grade neuroepithelial tumor with BCOR alteration (HGNET-BCOR) had abysmal responses to upfront chemotherapy-only regimens (5-year EFS = 0%), but survival extended with salvage radiation after progression [5-year OS = 53.6% ± 20.1%]. " 01/01/2018 - "To evaluate the use of chemotherapy and radiation, and their outcomes for patients with astroblastoma. " 07/27/2004 - "Initial chemotherapy is useful for some patients with gliomatosis cerebri. " 07/27/2004 - "Initial chemotherapy in gliomatosis cerebri." 06/01/2004 - "Combined surgery, radiation, and chemotherapy for oligodendroglial gliomatosis cerebri."
3.	Therapeutics 06/15/2009 - "TWO TYPES OF GLIOMATOSIS CEREBRI EXIST: Type I and Type II. We report the results of a histological and genetic study of two cases of gliomatosis cerebri Type II, correlating these results with therapy and prognosis. " 01/01/2023 - "Awareness of the emerging entity of PATZ1 fusion neuroepithelial tumors is important not only for accurate diagnostic and prognostic purposes but also for predicting response to therapy." 07/01/2015 - "In patients harboring anaplastic astroblastoma, gross-total resection and adjuvant therapy after the initial surgery seems to be the best choice. " 10/01/2013 - "[Differential diagnosis of and therapy for anaplastic astroblastoma: case report and review of the literature]." 02/01/2008 - "In patients harboring anaplastic astroblastoma, gross-total resection and adjuvant therapy after the initial surgery seems to be the best choice. "
4.	Craniospinal Irradiation 10/01/1993 - "Surgery, hyperfractionated craniospinal irradiation, and adjuvant chemotherapy in the management of supratentorial embryonal neuroepithelial neoplasms in children." 06/01/2017 - "Between 1997 and 2015, we treated 41 pediatric patients (26 males, 15 females; age ranging from 3.3 to 15.7 years, median 9-year-old) of pediatric brain tumors [17 medulloblastomas, 7 primitive neuroectodermal tumors (PNET), 3 pineoblastomas, 6 non-germinomatous germ cell tumors (NGGCT), 8 gliomas (including 4 ependymomas, 1 anaplastic astrocytoma, 1 oligodendroglioma, 1 pilocytic astrocytoma, 1 astroblastoma)] with high-dose craniospinal irradiation. "
5.	Chemoradiotherapy 01/01/2023 - "High-grade adult-type diffuse gliomas are malignant neuroepithelial tumors with poor survival rates in combined chemoradiotherapy. " 12/01/1989 - "The data on 645 patients with anaplastic neuroepithelial tumors subjected to radiotherapy, irradiation with radiosensitizers (metronidazole) and chemoradiotherapy are presented. "