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[PMID]: 29506496
[Au] Autor:Yon DK; Ahn TK; Shin DE; Kim GI; Kim MK
[Ad] Address:Department of Pediatrics, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Republic of Korea.
[Ti] Title:Extragonadal germ cell tumor of the posterior mediastinum in a child complicated with spinal cord compression: a case report.
[So] Source:BMC Pediatr;18(1):97, 2018 Mar 05.
[Is] ISSN:1471-2431
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Germ cell tumors (GCTs) in children are rare neoplasms with diverse pathological findings according to the site and age of presentation. The most common symptoms in children with mediastinal GCTs, which are nonspecific, are dyspnea, chest pain, cough, hemoptysis, vena cava occlusion syndrome, and fatigue/weakness. Because of these nonspecific symptoms, it is difficult to suspect a mediastinal mass. A posterior mediastinal tumor causing spinal cord compression is an important example of an oncologic emergency arising from a neurogenic tumor. CASE PRESENTATION: Children with posterior mediastinum GCTs can be easily mistaken as having a neurogenic tumor because of site of tumor origin. We treated our 7-year-old patient with emergency decompression surgery and high-dose steroid pulse therapy to prevent secondary injury to the spinal cord. Primary injury was a result of spinal cord compression due to the initial manifestation of GCT in the posterior mediastinum. Cisplatin-based chemotherapy was also administered. The patient was followed up regularly for 3 years and is undergoing rehabilitation without any signs of recurrence. CONCLUSIONS: We present an extremely rare case of a child with paraparesis caused by extradural spinal cord compression as the initial manifestation of GCT in the posterior mediastinum. The child was treated with emergency decompression surgery and high-dose pulse steroid therapy to prevent secondary injury to the spinal cord.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:In-Data-Review
[do] DOI:10.1186/s12887-018-1070-6

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[PMID]: 29454505
[Au] Autor:Bowzyk Al-Naeeb A; Murray M; Horan G; Harris F; Kortmann RD; Nicholson J; Ajithkumar T
[Ad] Address:Department of Oncology, Cambridge University Hospitals, Cambridge, UK. Electronic address: anna.bowzykalnaeeb@addenbrookes.nhs.uk.
[Ti] Title:Current Management of Intracranial Germ Cell Tumours.
[So] Source:Clin Oncol (R Coll Radiol);30(4):204-214, 2018 Apr.
[Is] ISSN:1433-2981
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Intracranial germ cell tumours (icGCTs) are uncommon tumours occurring in children and young adults. They are usually segregated into germinomas and non-germinomatous tumours (NGGCTs) in most classifications. Germinomas are highly curable tumours with multimodality treatment, but NGGCTs are associated with poorer survival outcomes. There are some differences in the approach to the management of icGCTs globally. Current research generally focuses on reducing treatment intensity, particularly the dose and volume of radiotherapy, in order to minimise the risks of late sequelae while maintaining high cure rates in icGCTs.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[St] Status:In-Data-Review

  3 / 3616 MEDLINE  
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[PMID]: 29177595
[Au] Autor:Wang WG; Ye H; Chinnaiyan P
[Ad] Address:Department of Radiation Oncology, Beaumont Health, Oakland University William Beaumont School of Medicine, 3601 West 13 Mile Road, Royal Oak, MI, 48073, USA.
[Ti] Title:Practice patterns and survival outcomes of intracranial germinoma: an analysis of the National Cancer Database.
[So] Source:J Neurooncol;137(1):77-82, 2018 Mar.
[Is] ISSN:1573-7373
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The goal of the study is to examine the practice pattern and survival outcome of adult and pediatric patients with intracranial germinoma. Patients from the National Cancer Database (NCDB) brain tumor registry between the years 2004-2014 with intracranial germinoma were extracted for analysis. Patients who had distant metastasis, received no treatments, or only surgery/chemotherapy alone were excluded. An age cutoff of > 21 years old was used to define the pediatric population. Patients were stratified by the treatments radiation therapy alone (RT) and chemotherapy followed by radiation therapy (C + RT). 445 patients with intracranial germinoma meeting our inclusion criteria were identified. Of the adult patients, 65.7% received RT and 34.3% received C + RT, compared to the pediatric patients, where 31.8% received RT and 68.2% received C + RT. Those patients who received C + RT had a lower radiation dose compared to the RT group (p < 0.001). The 5 and 10 year overall survival (OS) for the entire cohort was 92.6 and 87.9%, respectively. Univariate analysis demonstrated improved OS with younger age, private insurance, C + RT treatment, and pediatric patients. Only age and insurance type remained significant on multivariate analysis. The 5 year OS was 92.6% (RT) versus 97.2% (C + RT) (p = 0.307) and 83.4% (RT) versus 95.4% (C + RT) (p = 0.122) in the pediatric and adult patients, respectively. There is a higher use of C + RT with an accompanied reduction in RT dose in the treatment of intracranial germinoma. There is no difference in survival between the treatment approaches of RT or C + RT in the NCDB patient cohort.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 180225
[Lr] Last revision date:180225
[St] Status:In-Data-Review
[do] DOI:10.1007/s11060-017-2696-1

  4 / 3616 MEDLINE  
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[PMID]: 29274449
[Au] Autor:Yamasaki F; Kinoshita Y; Takayasu T; Usui S; Kolakshyapati M; Takano M; Tsuyuguchi S; Amatya VJ; Akiyama Y; Takeshima Y; Sugiyama K; Kurisu K
[Ad] Address:Department of Neurosurgery, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan. Electronic address: fyama@hiroshima-u.ac.jp.
[Ti] Title:Proton Magnetic Resonance Spectroscopy Detection of High Lipid Levels and Low Apparent Diffusion Coefficient Is Characteristic of Germinomas.
[So] Source:World Neurosurg;, 2017 Dec 20.
[Is] ISSN:1878-8769
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: The differentiation of germinoma from other tumors by conventional magnetic resonance imaging (MRI) can be very difficult. The purpose of our study was to determine whether diffusion-weighted imaging (DWI) and single-voxel proton magnetic resonance spectroscopy (1H-MRS) could provide additional useful information for a definitive diagnosis of germinomas. METHODS: Our hospital's Institutional Review Board approved this retrospective study. We reviewed imaging studies of 26 patients with histologically confirmed germinomas who were treated at our hospital between 2003 and 2016. We also studied 25 patients with pineal tumors, which included 14 nongerminomatous germ cell tumors (NGGCTs), 9 pineal parenchymal tumors (PPTs; including 3 pineocytomas, 4 PPTs of intermediate differentiation [PPTID], and 2 pineoblastomas) and 2 meningiomas. Patients underwent conventional MRI and advanced MRI, including DWI and/or 1H-MRS. RESULTS: The germinoma group comprised 24 males and 2 females, ranging in age from 9.1 to 37.7 years (average, 17.5 years; median, 14.6 years). The NGGCT group was all male, ranging in age from 5.4 to 51.9 years (mean, 20.1 years; median, 14.1 years). In the PPT group, patient age ranged from 4.5 to 64.7 years (mean, 29.9 years; median, 30.7 years). High lipid peaks detected on 1H-MRS were observed in 16 of 16 examined germinomas. In contrast, in the pineocytomas, PPTID, pineoblastomas, and meningiomas, lipid peaks were small or absent in 10 of 10 examined tumors. In the NGGCT group, high lipid peaks on 1H-MRS were observed in 11 of 12 examined tumors; however, no tumors showed high intensity on DWI, with all low to high mixed intensity. ADC was statistically lower in the germinoma group compared with the NGGCT group (P = 0.0002). CONCLUSIONS: Lower ADC values and high lipid peaks detected on 1H-MRS are characteristics of germinomas.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180217
[Lr] Last revision date:180217
[St] Status:Publisher

  5 / 3616 MEDLINE  
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[PMID]: 29415126
[Au] Autor:Wildeman ME; Shepard MJ; Oldfield EH; Lopes MBS
[Ad] Address:Department of Pathology (Neuropathology).
[Ti] Title:Central Nervous System Germinomas Express Programmed Death Ligand 1.
[So] Source:J Neuropathol Exp Neurol;, 2018 Feb 05.
[Is] ISSN:1554-6578
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Immunomodulation and tumor-induced tolerance is one of the central mechanisms in the oncogenesis of malignant and benign neoplasms. While numerous pathways have been described, signaling through the programmed death receptor 1 (PD-1) on T lymphocytes, via activation through its ligand, programmed death ligand 1 (PD-L1) expressed on tumor cells is one of the central pathways involved in tumor-induced tolerance. While the neoplastic component of germinomas of the CNS is the germ cell, these tumors also exhibit an abundance of quiescent tumor-infiltrating lymphocytes. We therefore investigated whether PD-L1 expression may be responsible for germinoma-induced T cell anergy, and if these tumors may be susceptible to immunotherapy. Pathologic specimens obtained from 21 cases of CNS germinomas between 2000 and 2016 were analyzed for the presence of PD-L1 and PD-1 expression by immunohistochemistry. Nineteen of 21 germinomas (90%) harbored germ cell components that stained positively for PD-L1. Positive lymphocyte staining for PD-L1 was evident in 16 cases. PD-1 expression was largely confined to lymphocytes; PD-L1 therefore may contribute to lymphocyte quiescence observed in these tumors. These results raise the possibility that immune checkpoint inhibitors such as nivolumab may have a therapeutic role in future treatment of germinomas.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180207
[Lr] Last revision date:180207
[St] Status:Publisher
[do] DOI:10.1093/jnen/nly008

  6 / 3616 MEDLINE  
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[PMID]: 28463397
[Au] Autor:Murray MJ; Bailey S; Heinemann K; Mann J; Göbel UK; Saran F; Hale JP; Calaminus G; Nicholson JC
[Ad] Address:Department of Paediatric Haematology and Oncology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, CB2 0QQ, United Kingdom.
[Ti] Title:Treatment and outcomes of UK and German patients with relapsed intracranial germ cell tumors following uniform first-line therapy.
[So] Source:Int J Cancer;141(3):621-635, 2017 08 01.
[Is] ISSN:1097-0215
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:We aimed to retrospectively assess treatments/outcomes, including the value of high-dose-chemotherapy and autologous-stem-cell-rescue (HDC + AuSCR) and re-irradiation, in a large, European patient-cohort with relapsed intracranial germ-cell-tumors (GCTs) receiving uniform first-line therapy, including radiotherapy as standard-of-care. Fifty-eight UK/German patients (48 male/10 female) with relapsed intracranial-GCTs [13 germinoma/45 non-germinomatous GCT (NGGCT)] treated 1996-2010 as per the SIOP-CNS-GCT-96 protocol were evaluated. For germinoma, six patients relapsed with germinoma and five with NGGCT (one palliative, one teratoma patient excluded). Five-year overall-survival (OS) for the whole-group (n = 11) was 55%. Four of six germinoma relapses and two of five relapsing with NGGCT were salvaged; patients were salvaged with either standard-dose-chemotherapy (SDC) and re-irradiation or HDC + AuSCR with/without re-irradiation. Of 45 relapsed NGGCT patients, 13 were excluded (three non-protocol adherence, five teratoma, five palliation). Five-year OS for the remaining 32 relapsed malignant NGGCT patients treated with curative intent was 9% (95%CI: 2-26%). By treatment received, 5-year OS for the 10 patients receiving SDC and 22 patients treated with intention for HDC + AuSCR was 0% (0-0%) and 14% (3-36%), respectively. The three relapsed NGGCT survivors had raised HCG markers alone; two received additional irradiation. Patients with relapsed germinoma had better 5-year OS than those with relapsed NGGCT (55 vs. 9%; p = 0.007). Patients with relapsed germinoma were salvaged both with SDC and re-irradiation or HDC + AuSCR with/without re-irradiation; both represent valid treatment options. Outcomes for malignant relapse following initial diagnosis of NGGCT were exceptionally poor; the few survivors received thiotepa-based HDC + AuSCR, which is a treatment option at first malignant relapse for such patients, with further surgery/irradiation where feasible.
[Mh] MeSH terms primary: Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Brain Neoplasms/therapy
Germinoma/therapy
Neoplasm Recurrence, Local/therapy
Neoplasms, Germ Cell and Embryonal/therapy
[Mh] MeSH terms secundary: Adolescent
Adult
Brain Neoplasms/pathology
Child
Child, Preschool
Combined Modality Therapy
Female
Follow-Up Studies
Germany
Germinoma/pathology
Humans
Infant
Male
Neoplasm Recurrence, Local/pathology
Neoplasm Staging
Neoplasms, Germ Cell and Embryonal/pathology
Prognosis
Retrospective Studies
Survival Rate
United Kingdom
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 180208
[Lr] Last revision date:180208
[Js] Journal subset:IM
[Da] Date of entry for processing:170503
[St] Status:MEDLINE
[do] DOI:10.1002/ijc.30755

  7 / 3616 MEDLINE  
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[PMID]: 29378909
[Au] Autor:Guedes BF; Souza MNP; Barbosa BJAP; Frassetto FP; Lucato LT; Ono CR; Castro LHM; Nitrini R; Simabukuro MM
[Ad] Address:Neurology Department, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
[Ti] Title:Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism.
[So] Source:Pract Neurol;, 2018 Jan 29.
[Is] ISSN:1474-7766
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:A young woman presented with primary amenorrhoea, progressive haemiparesis, visual disturbance, dementia and focal motor seizures. Investigations showed hypopituitarism, unilateral cerebral atrophy and inflamed cerebrospinal fluid. A trans-sphenoidal biopsy gave a unifying diagnosis of a pituitary germinoma.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180130
[Lr] Last revision date:180130
[St] Status:Publisher

  8 / 3616 MEDLINE  
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[PMID]: 29335082
[Au] Autor:Zhang JZ; Chen Q; Zheng P; Xie LN; Yi XL; Ren HT; Yang J
[Ad] Address:Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020, China. yangjian1306@sina.com.
[Ti] Title:[A comparative analysis of anti-N-methyl-D-aspartate receptor encephalitis with or without abnormal findings on cranial magnetic resonance imaging].
[So] Source:Zhongguo Dang Dai Er Ke Za Zhi;20(1):48-51, 2018 Jan.
[Is] ISSN:1008-8830
[Cp] Country of publication:China
[La] Language:chi
[Ab] Abstract:OBJECTIVE: To investigate the clinical features of children with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis with normal or abnormal cranial magnetic resonance imaging (MRI) findings via a comparative analysis. METHODS: A retrospective analysis was performed for the clinical data of 33 children with anti-NMDAR encephalitis. The clinical features and prognosis were compared between the children with normal and abnormal cranial MRI findings. RESULTS: In the 33 children with anti-NMDAR encephalitis, the most common initial symptoms were seizures (61%) and involuntary movement (61%), followed by language disorder (54%), mental and behavioral abnormalities (52%), and disturbance of consciousness (30%). All children had positive anti-NMDAR antibody in the cerebrospinal fluid, and 29 children (88%) had positive serum antibody. Of all the children, 15 (46%) had increased leukocytes in the cerebrospinal fluid, 3 (9%) had an increase in protein, and 29 (88%) had positive oligoclonal band; 26 children (79%) had electroencephalographic abnormalities (epileptic wave, slow wave, or a combination of these two types of waves). One child experienced respiratory failure. One child was found to have germinoma in the sellar region during follow-up. Of all the 33 children, 13 (39%) had abnormal cranial MRI findings, with hypointensity or isointensity on T1W1 and hyperintensity on T2WI and T2-FLAIR; 2 children had dural enhancement. As for the location of lesion, 5 children (38%) had lesions in the temporal lobe, 3 (23%) in the frontal lobe, 3 (23%) in the basal ganglia, 2 (15%) in the parietal lobe, 2 (15%) in the occipital lobe, 2 (15%) in the brainstem, 1 (8%) in the thalamus, and 1 (8%) in the cerebellum. Among the 13 children with abnormal cranial MRI findings, 5 (38%) had lesions mainly in the grey matter and 8 (62%) had lesions mainly in the white matter. Compared with the children with normal cranial MRI findings, the children with abnormal cranial MRI findings had significantly higher proportion of children with prodromal infection, incidence rate of disturbance of consciousness, probability of recurrence, Glasgow score, incidence rate of increased leukocytes in the cerebrospinal fluid, and application rate of second-line treatment (P<0.05). CONCLUSIONS: Children with anti-NMDAR encephalitis and abnormal cranial MRI findings have certain clinical features, which may provide guidance for the evaluation of disease conditions and the selection of diagnostic and treatment measures.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180116
[Lr] Last revision date:180116
[St] Status:In-Data-Review

  9 / 3616 MEDLINE  
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[PMID]: 29237087
[Au] Autor:Zapka P; Dörner E; Dreschmann V; Sakamato N; Kristiansen G; Calaminus G; Vokuhl C; Leuschner I; Pietsch T
[Ad] Address:Department of Neuropathology and Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.
[Ti] Title:Type, Frequency, and Spatial Distribution of Immune Cell Infiltrates in CNS Germinomas: Evidence for Inflammatory and Immunosuppressive Mechanisms.
[So] Source:J Neuropathol Exp Neurol;77(2):119-127, 2018 Feb 01.
[Is] ISSN:1554-6578
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Central nervous system germinomas are characterized by a massive immune cell infiltrate. We systematically characterized these immune cells in 28 germinomas by immunophenotyping and image analysis. mRNA expression was analyzed by Nanostring technology and in situ RNA hybridization. Tumor infiltrating lymphocytes (TILs) were composed of 61.8% ± 3.1% (mean ± SE) CD3-positive T cells, including 45.2% ± 3.5% of CD4-positive T-helper cells, 23.4% ± 1.5% of CD8-positive cytotoxic T cells, 5.5% ± 0.9% of FoxP3-positive regulatory T cells, and 11.9% ±1.3% PD-1-positive TILs. B cells accounted for 35.8% ± 2.9% of TILs and plasma cells for 9.3% ± 1.6%. Tumor-associated macrophages consisted of clusters of activated PD-L1-positive macrophages and interspersed anti-inflammatory macrophages expressing CD163. Germinoma cells did not express PD-L1. Expression of genes encoding immune cell markers and cytokines was high and comparable to mRNA levels in lymph node tissue. IFNG and IL10 mRNA was detected in subfractions of TILs and in PD-L1-positive macrophages. Taken together, the strong immune reaction observed in germinomas involves inflammatory as well as various suppressive mechanisms. Expression of PD-1 and PD-L1 and infiltration of cytotoxic T cells are biomarkers predictive of response to anti-PD-1/PD-L1 therapies, constituting a rationale for possible novel treatment approaches.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180111
[Lr] Last revision date:180111
[St] Status:In-Data-Review
[do] DOI:10.1093/jnen/nlx106

  10 / 3616 MEDLINE  
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[PMID]: 29232188
[Au] Autor:Van Lierde L; Clapuyt P; Nassogne MC; Oriot P
[Ad] Address:Universite Catholique de Louvain, Cliniques Universitaires Saint-Luc, Brussels, Belgium
[Ti] Title:Cerebral germinoma revealed through a polydipsic polyuric syndrome in a 10-year-old girl: case report.
[So] Source:Endocr Regul;51(4):216-219, 2017 10 26.
[Is] ISSN:1210-0668
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Cerebral germinoma is rare. Although the imaging of the germinoma is very evocative, it's very polymorphic clinical expression is at the origin of misguided diagnosis, as illustrated in our case. We report the case of a 10-year-old girl with diabetes insipidus evolving for 12 months associated with a decrease in visual acuity. Brain MRI (Magnetic Resonance Imaging) revealed a tumor process in the suprasellar region. The stereotaxic biopsy of the tumor confirmed the diagnosis of the hypothalamic germinoma, which allowed the patient to be treated by radiotherapy and chemotherapy. The incidence of cerebral germinoma, its clinical (principally diabetes insipidus) and radiological features as well as therapeutic strategies are discussed hereby.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180111
[Lr] Last revision date:180111
[St] Status:In-Data-Review


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