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1.
Zhonghua Yi Xue Za Zhi ; 100(3): 178-181, 2020 Jan 21.
Artículo en Chino | MEDLINE | ID: mdl-32008282

RESUMEN

Objective: To evaluate the classification of the types of pediatric posterior fossa brain tumors based on routine MRI (T(1)WI, T(2)WI and ADC) using wavelet transformation analysis of whole tumor. Methods: MRI images of medulloblastoma (n=59), ependymoma (n=13) and pilocytic astrocytoma (n=27) confirmed by pathology before treatments in Children's Hospital of Nanjing Medical University from January 2014 to February 2019 were enrolled in this retrospective study as well as the clinical data of age, gender and symptoms. Registration was performed among the three sequences and wavelet features of ROI were acquired. Afterwards, the top ten features were ranked and trained among groups by using random forest classifier. Finally, the results were compared and analyzed according to the classification. Results: The top ten contribution three sequences and wavelet features of ROI were acquired from the ADC sequence. The random forest classifier achieved 100% accuracy on training data and was validated best accuracy (86.8%) when combined of first and third wavelet features. The sensitivity was 100%, 94.8%, 76.9%, and the specificity was 97.6%, 88.0%, 98.8% respectively. Conclusions: Features based on wavelet transformation of ADC sequence of entire tumor can provide more quantitative information, which could provide help in the differential diagnosis of pediatric posterior fossa brain tumors. The optimum combination to distinguish three pediatric posterior fossa brain tumors is sixth and twelfth wavelet features of ADC sequence.


Asunto(s)
Astrocitoma/clasificación , Neoplasias Cerebelosas/patología , Neoplasias Infratentoriales/patología , Imagen por Resonancia Magnética/métodos , Meduloblastoma/clasificación , Astrocitoma/patología , Neoplasias Encefálicas/patología , Niño , Humanos , Meduloblastoma/patología , Estudios Retrospectivos
2.
Zhonghua Bing Li Xue Za Zhi ; 49(1): 52-56, 2020 Jan 08.
Artículo en Chino | MEDLINE | ID: mdl-31914535

RESUMEN

Objective: To investigate the immunohistochemical staining of anaplastic lymphoma kinase (ALK; clone 1A4) in pediatric medulloblastoma (MB). Methods: Molecular subtyping was performed based on the NanoString and sequencing techniques for 44 pediatric MB cases at Children's Hospital, Zhejiang University School of Medicine from 2014 to 2017. ALK expression was detected with EnVision immunhistochemistry using ALK clone 1A4 on whole section. Statistical analyses were performed to evaluate the correlation of protein expression with molecular subgroups. Results: The age ranged from 0.5 to 13.0 years with an average age of 5.8 years. There were 28 males and 16 females, and 31 classic, 5 desmoplastic nodular, 3 extensive nodular and 5 large cell/anaplastic MBs. Except three cases was unable classified, 41 MBs were classified into the four molecular groups: 5 in WNT group, 12 in SHH group, 9 in Group 3 and 15 in Group 4. Thirteen of 44 MB cases were positive staining for ALK, and the positive rate was 29.5%. Six cases were strong reaction, and 7 cases were weak. The expression of ALK at the protein level was associated with the WNT group (P<0.01). The characteristic perinuclear dot-like staining was only showed in WNT group. Conclusions: The ALK immunhistochemistry using antibody clone 1A4 is a useful marker for the molecular subgroup detection of MB. The strong staining and perinuclear dot-like staining indicate as WNT group.


Asunto(s)
Neoplasias Cerebelosas , Meduloblastoma , Adolescente , Quinasa de Linfoma Anaplásico , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Proteínas Tirosina Quinasas Receptoras
3.
World Neurosurg ; 133: 99-103, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31574330

RESUMEN

BACKGROUND: Intracranial tumor growth associated with pregnancy is not an uncommon phenomenon. Pilocytic astrocytoma is typically considered to be an indolent tumor with little to no risk of progression to higher-grade lesion. We present a rare case of cerebellar pilocytic astrocytoma transformation to hemorrhagic high-grade glioma during pregnancy. CASE DESCRIPTION: Patient EK was a 32-year-old female with neurofibromatosis type 1 and known cerebellar pilocytic astrocytoma. For nearly a decade before her pregnancy, her cerebellar tumor was stable on imaging. Routine magnetic resonance imaging (MRI) of the head obtained at 20 weeks' gestation continued to demonstrate tumor stability. At 24 weeks' gestation, the patient had sudden, severe headaches. MRI of the head showed evidence of significant tumor expansion. The following day, the patient was found unresponsive. Computed tomography of the head demonstrated hemorrhage within the tumor and tonsillar herniation. Her neurologic examination revealed no brainstem reflexes; however, given her age and pregnancy, she underwent emergent decompression and tumor debulking. Unfortunately, she never improved neurologically. Final pathology identified the lesion as high-grade glioma with anaplastic changes and hemorrhagic conversion. CONCLUSIONS: This is a unique case of indolent cerebellar pilocytic astrocytoma that transformed to high-grade glioma during pregnancy, proven by tumor growth on MRI and anaplasia on pathology. We hypothesize that increased levels of pregnancy hormones (progesterone, vascular endothelial growth factor, placental growth factor, among others) likely contributed to tumor growth. We recommend that all glial tumors be monitored extremely closely throughout pregnancy, and perhaps one should consider surgical treatment (if possible) before patients become pregnant.


Asunto(s)
Astrocitoma/cirugía , Neoplasias Cerebelosas/cirugía , Neurofibromatosis 1/cirugía , Complicaciones Neoplásicas del Embarazo/cirugía , Adulto , Astrocitoma/diagnóstico por imagen , Proliferación Celular , Neoplasias Cerebelosas/diagnóstico por imagen , Craneotomía , Descompresión Quirúrgica , Progresión de la Enfermedad , Resultado Fatal , Femenino , Humanos , Neurofibromatosis 1/diagnóstico por imagen , Embarazo , Complicaciones Neoplásicas del Embarazo/diagnóstico por imagen
4.
Magy Onkol ; 63(4): 331-345, 2019 Dec 09.
Artículo en Húngaro | MEDLINE | ID: mdl-31821388

RESUMEN

Despite continuing advances in therapeutic strategies, survival of childhood medulloblastoma (MB) patients has reached a plateau in the past decade. Current clinical approaches divide patients into average- and high-risk treatment categories, although this categorization does not take patient heterogeneity into account. Advanced genomics has initiated an exciting transition that lead to a consensus of four distinct molecular entities within MBs (WNT-activated, SHH-activated, Group 3 MB, and Group 4 MB), each with distinct origins, demographics, molecular alterations and clinical outcomes. Within each of the four primary subgroups additional subtypes started to emerge with distinct biological backgrounds and clinical outcomes. Here we summarize subgroup-specific genomic alterations, affected signaling pathways and potential prognostic biomarkers. The poor prognosis associated with recurrent disease is responsible for the stagnant survival rates. Nevertheless, the mortality is unlikely to change without new biomarkers linked to different mechanisms of pathway activation.


Asunto(s)
Neoplasias Cerebelosas , Meduloblastoma , Niño , Perfilación de la Expresión Génica , Genómica , Humanos , Pronóstico
5.
No Shinkei Geka ; 47(11): 1173-1178, 2019 Nov.
Artículo en Japonés | MEDLINE | ID: mdl-31761779

RESUMEN

Intracranial epidermoid cysts are benign cystic lesions that typically exhibit slow growth. Their malignant transformation into squamous cell carcinoma is rare. We report a 77-year-old woman who was admitted to our hospital because of a near-drowning incident due to a seizure sustained in her bathtub. Magnetic resonance imaging(MRI)revealed an extra-axial tumor occupying the right cerebellopontine angle. The lesion appeared hyperintense in diffusion-weighted images and exhibited contrast enhancement after gadolinium injection. Cerebrospinal fluid examination revealed noninfectious meningitis, presumably due to the ruptured epidermoid cyst. Tumor resection was performed and histopathological examination revealed squamous cell carcinoma, which was indicative of malignant transformation of the cyst. The patient underwent adjuvant radiotherapy and has no signs of recurrence 9 months postsurgery. Rapid neurological deterioration and contrast enhancement on MRI are key signs of malignant transformation of epidermoid cysts.


Asunto(s)
Ángulo Pontocerebeloso/cirugía , Quiste Epidérmico , Anciano , Transformación Celular Neoplásica , Neoplasias Cerebelosas , Quiste Epidérmico/cirugía , Femenino , Humanos , Imagen por Resonancia Magnética , Recurrencia Local de Neoplasia
6.
Prague Med Rep ; 120(2-3): 95-102, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31586508

RESUMEN

Malignant transformation of an epidermoid tumour is a rare entity that in almost all patients occurs at the same site of the primary lesion. We report a case of an epidermoid tumour with malignant transformation to squamous cell carcinoma (SCC) at the adjacent site but without any relation to the primary site of the tumour. A 30-year-old patient with a history of cranial surgery and resection of cerebellopontine (CP) angle epidermoid cyst five years ago, presented with a headache, nausea, and vomiting. Physical examination showed no neurological deficit. The brain magnetic resonance imaging (MRI) demonstrated a well-defined lesion within left middle cerebellar peduncle with no relation to CP angle cistern (the previous tumour site). It was isointense on T1, isointense on T2 and had a rim enhancement on gadolinium (GD) injection. Via retrosigmoid and transcortical approach, total resection of the tumour was performed. During the surgery, there was no visible relationship between the current lesion and the previously resected lesion site. Histopathology revealed squamous cell carcinoma. The systemic survey to finding a probable origin of the tumour was negative and the patient referred for performing brain radiotherapy. We are reporting a case of malignant transformation of epidermoid cyst separate from primary location. Moreover, malignant transformation can occur years after index surgery even after gross total resection.


Asunto(s)
Carcinoma de Células Escamosas/patología , Transformación Celular Neoplásica , Neoplasias Cerebelosas/patología , Ángulo Pontocerebeloso/patología , Quiste Epidérmico/patología , Adulto , Humanos , Imagen por Resonancia Magnética
7.
World Neurosurg ; 132: 398, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31520755

RESUMEN

Intracranial epidermoid cysts are developmental central nervous system abnormalities that arise from erroneous ectodermal inclusion during neural tube closure. Epidermoids are relatively common, representing 1% of all intracranial masses, and are particularly predominant in the cerebellopontine angle (CPA), where they are the third most prevalent diagnosis after schwannomas and meningiomas. Although small, asymptomatic, or incidental lesions may be safely observed, in the presence of symptomatic mass effect, epidermoid cysts typically require surgical resection. As shown in Video 1, we demonstrate the operating room setup and endoscopic resection of a left CPA epidermoid cyst through a retrosigmoid craniotomy. By use of a 2-surgeon 4-hands technique, the mass was dissected from the surrounding neurovascular structures, and a gross total removal was achieved with no postoperative neurologic deficits. Our video highlights the critical nuances pertinent to the fully endoscopic resection technique, with special attention to the relative restrictions of a small working corridor parallel to the tentorium and the petrous bone.


Asunto(s)
Enfermedades Cerebelosas/cirugía , Ángulo Pontocerebeloso/cirugía , Endoscopía/métodos , Quiste Epidérmico/cirugía , Procedimientos Neuroquirúrgicos/métodos , Neoplasias Cerebelosas/cirugía , Humanos , Meningioma/cirugía , Neurocirujanos
9.
Zhonghua Bing Li Xue Za Zhi ; 48(9): 694-699, 2019 Sep 08.
Artículo en Chino | MEDLINE | ID: mdl-31495089

RESUMEN

Objective: To investigate the expression and potential role of heterogeneous nuclear ribonucleo-protein A2B1 (HNRNPA2B1) in mouse cerebellar development and the significance of HNRNPA2B1 in human medulloblastoma. Methods: The data of HNRNPA2B1 RNA expression in mouse and human cerebella were obtained from databases. Western blot and immunohistochemical staining were performed to detect the protein level of HNRNPA2B1 in mouse cerebella at different ages. The expression level of HNRNPA2B1 in control human cerebellum and medulloblastoma was detected by immunohistochemical staining. m6A-IP-qPCR method was applied to confirm whether HNRNPA2B1 RNA in Daoy cells was modified with m6A.Western blot was used to detect the effect of MG132 treatment on the HNRNPA2B1 protein level in Daoy cells. Results: The level of HNRNPA2B1 protein in postnatal mouse cerebella was higher than that in adult mouse cerebella, with weak HNRNPA2B1 staining in external granular cells while strong staining in mature Purkinje cells and molecular layer. Compared with control normal human cerebella, the RNA expression level of HNRNPA2B1 increased in medulloblastoma, while immunohistochemical staining showed that the mean intensity of HNRNPA2B1 decreased in medulloblastoma. HNRNPA2B1 RNA in medulloblastoma and Daoy cells was modified by m6A. The HNRNPA2B1 protein level in Daoy cells increased upon MG132 treatment. Conclusions: HNRNPA2B1 is dynamically expressed during mouse cerebellar development. Compared with normal human cerebella, HNRNPA2B1 is significantly up-regulated at transcriptional level but obviously down-regulated at translational level in medulloblastoma. These results indicate that HNRNPA2B1 may be involved in cerebellar development process and medulloblastoma tumorigenesis. The m6A methylation in HNRNPA2B1 transcript and protein ubiquitin-proteasome pathway may account for the down-regulation of HNRNPA2B1 at protein level.


Asunto(s)
Neoplasias Cerebelosas , Meduloblastoma , Animales , Línea Celular Tumoral , Cerebelo , Regulación hacia Abajo , Ribonucleoproteína Heterogénea-Nuclear Grupo A-B , Humanos , Ratones
10.
Pan Afr Med J ; 33: 118, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31489096

RESUMEN

Dysplastic gangliocytoma or Lhermitte-Duclos disease is a rare disorder characterized by a slowly progressive unilateral tumour mass of the cerebellar cortex. It is probably hamartomatous, although the exact pathogenesis remains unknown. Lhermitte-Duclos disease was recently encountered to be part of a multiple hamartoma-neoplasia complex (Cowden's syndrome). It typically presents in young adults, although it has been encountered at all ages. We present the case of bilateral cerebellar location of this pathology in a 50-year-old man presented with a progressive onset and worsening of headaches accompanied by nuchal rigidity, photophobia and nausea awakening each morning. Upon physical examination, the patient was awake with a discrete right vestibular syndrome made of positive Romberg without nystagmus. Magnetic Resonance Imaging (MRI) was performed and revealed salient "tiger stripe" appearance of the bilateral cerebellar cortex relevant to a Lhermitte-Duclos disease.


Asunto(s)
Neoplasias Cerebelosas/diagnóstico por imagen , Síndrome de Hamartoma Múltiple/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Neoplasias Cerebelosas/patología , Síndrome de Hamartoma Múltiple/patología , Cefalea/etiología , Humanos , Masculino , Persona de Mediana Edad , Rigidez Muscular/etiología , Náusea/etiología , Fotofobia/etiología
11.
Medicine (Baltimore) ; 98(32): e16756, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31393392

RESUMEN

RATIONALE: In some cases, surgery of cerebellopontine angle meningioma (CPAM) might result in multiple cranial nerve injury, which could bring serious impact on the patients, especially when it affects the function of facial muscles and eyeballs. This report describes a successful application of acupuncture for rehabilitation in a patient after surgery for CPAM. PATIENT CONCERNS: A 27-year-old patient presented with limitation of left eye abduction, accompanied with frontal and facial sensory disturbance on the left after resection of the pontocerebellar angle tumor. The patient also suffered from significant anxiety and depression as concomitant symptoms. DIAGNOSES: Based on medical history, clinical symptoms, and magnetic resonance imaging results, the patient was diagnosed with the fourth, fifth, sixth, and seventh cranial nerve injury after surgery for CPAM. INTERVENTIONS: Acupuncture treatment was applied for this patient. One acupuncture session was given every 2 days in 35 days, and the needles were retained for 30 minutes per session. OUTCOMES: After acupuncture treatment, the limitation of left eye abduction had totally recovered. The superficial sensory disturbance in the frontal and facial region was significantly relived. Besides, the scores of Hamilton Anxiety and Depression Scale showed a significant reduction. However, the superficial sensory of the alar and nasolabial groove on the left side still decreased mildly when compared with the right side. CONCLUSION: Acupuncture might be an option for rehabilitation after surgery for CPAM.


Asunto(s)
Terapia por Acupuntura/métodos , Neoplasias Cerebelosas/cirugía , Traumatismos del Nervio Craneal/rehabilitación , Meningioma/cirugía , Adulto , Neoplasias Cerebelosas/patología , Ángulo Pontocerebeloso/patología , Traumatismos del Nervio Craneal/etiología , Humanos , Imagen por Resonancia Magnética , Masculino
12.
Arkh Patol ; 81(4): 66-72, 2019.
Artículo en Ruso | MEDLINE | ID: mdl-31407721

RESUMEN

In most cases, oncogene amplification are prognostic and predictive markers for various tumors, therefore DNA probes are unable to reveal changes in the copy numbers should not be used to diagnose malignant tumors. OBJECTIVE: To comparatively analyze DNA probes from different manufacturers to detect MYC gene amplification in routine practice. MATERIAL AND METHODS: The study material was formalin-fixed paraffin-embedded medulloblastoma fragments from 4 patients, with discrepancies in the results in the detection of MYC gene amplification. RESULTS: MYC gene amplification was determined using DNA probes: Kreatech MYC (8q24)/SE 8, Vysis LSI MYC SO, Vysis CEP 8 (D8Z2) SG, and Zytolight SPEC MYC/CEN 8 Dual Color Probe. The use of the probes Kreatech TERC (3q26)/MYC (8q24)/SE7 Triple-Color probe failed to detect MYC gene amplification; this probe showed a balanced profile of chromosome 8. CONCLUSION: In routine practice, fluorescence in situ hybridization with the DNA probes Kreatech MYC (8q24)/SE 8, Vysis LSI MYC SO, Vysis CEP 8 (D8Z2) SG and Zytolight SPEC MYC/CEN 8 Dual Color Probe can be the method of choice for studying the copy number of the MYC gene. However, the authors strongly recommend that the Kreatech TERC (3q26)/MYC (8q24)/SE7 Triple-Color should not be used for this purpose. In addition, probes for fluorescence in situ hybridization must be necessarily tested in large reference laboratories dealing with one or another area of oncopathology.


Asunto(s)
Neoplasias Cerebelosas , Amplificación de Genes , Genes myc , Hibridación Fluorescente in Situ , Meduloblastoma , Neoplasias Cerebelosas/diagnóstico , Neoplasias Cerebelosas/genética , Humanos , Meduloblastoma/diagnóstico , Meduloblastoma/genética
13.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(8): 761-765, 2019 Aug.
Artículo en Chino | MEDLINE | ID: mdl-31416499

RESUMEN

OBJECTIVE: To investigate the risk factors for recurrence of medulloblastoma (MB) within 2 years and their influence on progression-free survival (PFS). METHODS: A retrospective analysis was performed for the clinical data of 123 children with MB who were admitted from January to December, 2017. According to the presence or absence of recurrence, they were divided into recurrence group with 30 children and non-recurrence group with 93 children. The risk factors for recurrence within 2 years were analyzed, and PFS was compared between the children with different risk factors. RESULTS: Large-cell/anaplastic type and M stage were risk factors for MB recurrence within 2 years. The risk of recurrence in the children with M+ MB was 3.525 times that in those with M0 MB, and the risk of recurrence in the children with large-cell/anaplastic MB was 3.358 times that in those with classic MB (P<0.05). The survival analysis showed that the median PFS time was 20 months in the children with M+ MB, and the 20-month PFS rate was 50% ±â€…11% in the children with M+ MB and 81% ±â€…5% in those with M0 MB (P<0.05). The 20-month PFS rate was 80% ±â€…5% in the children with classic MB, 65% ±â€…10% in those with desmoplastic/nodular MB, 86% ±â€…13% in those with MB with extensible nodularity, and 36% ±â€…20% in those with large-cell/anaplastic MB (P<0.05). CONCLUSIONS: Recurrence is an important influencing factor for the prognosis of MB, and M+ stage and large-cell/anaplastic MB are risk factors for recurrence. Children with such risk factors tend to have a low PFS rate.


Asunto(s)
Neoplasias Cerebelosas , Meduloblastoma , Niño , Humanos , Recurrencia Local de Neoplasia , Pronóstico , Recurrencia , Estudios Retrospectivos , Factores de Riesgo
15.
Cancer Radiother ; 23(5): 370-377, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31331843

RESUMEN

PUPOSE: Medulloblastoma is the most common primary malignant central nervous system tumor in childhood, accounting for 16-25% of cases (1). New treatment approaches have led to improved survival rates; however toxicities are still a major concern. PATIENTS AND METHODS: Participants were selected from the records of patients who were treated with craniospinal irradiation for medulloblastoma. Between January 2008 and December 2012, 62 patients were diagnosed with medulloblastoma at the national institute of oncology Rabat, 27 patients were still alive at the time of the study, of which n=16 patients were included in the study. The mean age of patients at the time of the study was 9.6 years. All children were treated with radiation therapy and chemotherapy, according to standard protocols. Median follow-up between treatment and evaluation was 4 years. All the children were assessed with the Wechsler Intelligence Scale for Children - fourth Edition (WISC-IV) three to five years after completion of radiotherapy. The test was administered by two well-trained psychologists in a distraction-free environment. The scoring was then reviewed by a psychologist from Brooklyn College. RESULTS: The mean standard score Full-Scale Intelligence Quotient (FSIQ) (M=63, SD=12.6) was found to be in the extremely low range and in the 1st percentile rank (PR), compared to the general population. All the measured primary index scales were below typical performance: verbal comprehension (M=67.7, SD=13.1), perceptual reasoning (M=63.5, SD=13.8) and processing speed (M=62.7, SD=15.5) were all found to be in the extremely low range, while xorking memory (M=75.5, SD=10.8) was found to be in the borderline range compared to the general population. To identify factors influencing the results, we performed both univariate and multivariate analyses. Age at the time of radiotherapy, initial clinical stage, total cranial radiotherapy dose, socioeconomic status, and the time of evaluation were identified as significantly impacting cognitive scores in the univariate analysis. In the multivariate analysis, only age at the time of radiotherapy and initial clinical stage remained factors significantly impacting cognitive outcomes with P=0.001 and P<0.001 respectively. CONCLUSION: Our study is evidence that tremendous efforts are still to be made in low-income countries to correctly measure neurocognitive dysfunction in medulloblastoma survivors and to prepare those patients to a typical life after the completion of treatment.


Asunto(s)
Neoplasias Cerebelosas/radioterapia , Irradiación Craneana/efectos adversos , Meduloblastoma/radioterapia , Trastornos Neurocognitivos/etiología , Factores de Edad , Antineoplásicos/uso terapéutico , Neoplasias Cerebelosas/tratamiento farmacológico , Niño , Preescolar , Terapia Combinada , Estudios de Seguimiento , Sustancia Gris/lesiones , Sustancia Gris/patología , Hipocampo/lesiones , Hipocampo/patología , Humanos , Meduloblastoma/tratamiento farmacológico , Trastornos de la Memoria/etiología , Trastornos de la Memoria/patología , Trastornos Neurocognitivos/patología , Tamaño de los Órganos , Modelos de Riesgos Proporcionales , Escalas de Wechsler , Sustancia Blanca/lesiones , Sustancia Blanca/patología
16.
BMJ Case Rep ; 12(7)2019 Jul 17.
Artículo en Inglés | MEDLINE | ID: mdl-31320371

RESUMEN

We report the first clinical case on the successful use of proton beam therapy in the management of malignant transformation of intracranial epidermoid cyst. A 43-year-old man was initially diagnosed as this disease with left facial paresis, hypesthesia and hypoalgesia in the territories of the trigeminal nerve. After failure of surgical interventions, he was referred to our radiation centre. We performed a postoperative proton beam therapy for treatment. We delivered a total dose of 57 GyE in 31 fractions. He tolerated the treatment well with mild acute toxicities and remained healthy and functional by 2-year follow-up postradiotherapy. No evidence of delayed radiation-induced neurotoxicity was observed.


Asunto(s)
Quistes del Sistema Nervioso Central/radioterapia , Neoplasias Cerebelosas/radioterapia , Quiste Epidérmico/radioterapia , Carcinoma de Células Escamosas de Cabeza y Cuello/radioterapia , Adulto , Transformación Celular Neoplásica , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Quistes del Sistema Nervioso Central/patología , Quistes del Sistema Nervioso Central/cirugía , Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/patología , Neoplasias Cerebelosas/cirugía , Ángulo Pontocerebeloso , Quiste Epidérmico/diagnóstico por imagen , Quiste Epidérmico/patología , Quiste Epidérmico/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Procedimientos Neuroquirúrgicos , Terapia de Protones , Carcinoma de Células Escamosas de Cabeza y Cuello/diagnóstico por imagen , Carcinoma de Células Escamosas de Cabeza y Cuello/patología , Carcinoma de Células Escamosas de Cabeza y Cuello/cirugía
17.
World Neurosurg ; 130: 378-379, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31306840

RESUMEN

Vestibular schwannomas (VSs) are rare in children and, when present, are usually part of neurofibromatosis 2 and bilateral. Sporadic unilateral VSs in the pediatric age group itself are rare in medical literature and giant sporadic unilateral pediatric VSs (>4 cm) are extremely rare. Herein, we describe the largest reported case of giant sporadic left-sided VS in a 10-year-old boy.


Asunto(s)
Neoplasias Cerebelosas/patología , Neuroma Acústico/patología , Ángulo Pontocerebeloso/patología , Niño , Humanos , Masculino , Tratamientos Conservadores del Órgano/métodos , Resultado del Tratamiento , Carga Tumoral
19.
Nat Commun ; 10(1): 2400, 2019 06 03.
Artículo en Inglés | MEDLINE | ID: mdl-31160565

RESUMEN

BET-bromodomain inhibition (BETi) has shown pre-clinical promise for MYC-amplified medulloblastoma. However, the mechanisms for its action, and ultimately for resistance, have not been fully defined. Here, using a combination of expression profiling, genome-scale CRISPR/Cas9-mediated loss of function and ORF/cDNA driven rescue screens, and cell-based models of spontaneous resistance, we identify bHLH/homeobox transcription factors and cell-cycle regulators as key genes mediating BETi's response and resistance. Cells that acquire drug tolerance exhibit a more neuronally differentiated cell-state and expression of lineage-specific bHLH/homeobox transcription factors. However, they do not terminally differentiate, maintain expression of CCND2, and continue to cycle through S-phase. Moreover, CDK4/CDK6 inhibition delays acquisition of resistance. Therefore, our data provide insights about the mechanisms underlying BETi effects and the appearance of resistance and support the therapeutic use of combined cell-cycle inhibitors with BETi in MYC-amplified medulloblastoma.


Asunto(s)
Azepinas/farmacología , Ciclo Celular/efectos de los fármacos , Neoplasias Cerebelosas/tratamiento farmacológico , Meduloblastoma/tratamiento farmacológico , Neurogénesis/efectos de los fármacos , Proteínas/antagonistas & inhibidores , Triazoles/farmacología , Animales , Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/efectos de los fármacos , Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/metabolismo , Sistemas CRISPR-Cas , Proteínas de Ciclo Celular/efectos de los fármacos , Proteínas de Ciclo Celular/metabolismo , Línea Celular Tumoral , Linaje de la Célula , Neoplasias Cerebelosas/genética , Ciclina D2/efectos de los fármacos , Ciclina D2/metabolismo , Quinasa 4 Dependiente de la Ciclina/antagonistas & inhibidores , Quinasa 6 Dependiente de la Ciclina/antagonistas & inhibidores , Resistencia a Antineoplásicos , Perfilación de la Expresión Génica , Humanos , Meduloblastoma/genética , Ratones , Células-Madre Neurales/efectos de los fármacos , Células-Madre Neurales/metabolismo , Proteínas Proto-Oncogénicas c-myc/genética , Fase S/efectos de los fármacos
20.
Nat Commun ; 10(1): 2410, 2019 06 03.
Artículo en Inglés | MEDLINE | ID: mdl-31160587

RESUMEN

Medulloblastoma, which is the most common malignant paediatric brain tumour, has a 70% survival rate, but standard treatments often lead to devastating life-long side effects and recurrence is fatal. One of the emerging strategies in the search for treatments is to determine the roles of tumour microenvironment cells in the growth and maintenance of tumours. The most attractive target is tumour-associated macrophages (TAMs), which are abundantly present in the Sonic Hedgehog (SHH) subgroup of medulloblastoma. Here, we report an unexpected beneficial role of TAMs in SHH medulloblastoma. In human patients, decreased macrophage number is correlated with significantly poorer outcome. We confirm macrophage anti-tumoural behaviour in both ex vivo and in vivo murine models of SHH medulloblastoma. Taken together, our findings suggest that macrophages play a positive role by impairing tumour growth in medulloblastoma, in contrast to the pro-tumoural role played by TAMs in glioblastoma, another common brain tumour.


Asunto(s)
Neoplasias Cerebelosas/inmunología , Macrófagos/inmunología , Meduloblastoma/inmunología , Microambiente Tumoral/inmunología , Animales , Antígenos CD/genética , Antígenos CD/metabolismo , Antígenos de Diferenciación Mielomonocítica/genética , Antígenos de Diferenciación Mielomonocítica/metabolismo , Antígeno CD11b/genética , Antígeno CD11b/metabolismo , Proteínas de Unión al Calcio , Neoplasias Cerebelosas/genética , Neoplasias Cerebelosas/metabolismo , Quimiocina CCL2/inmunología , Quimiocina CCL2/metabolismo , Proteínas de Unión al ADN/genética , Modelos Animales de Enfermedad , Proteínas Hedgehog/metabolismo , Humanos , Macrófagos/metabolismo , Meduloblastoma/genética , Meduloblastoma/metabolismo , Ratones , Proteínas de Microfilamentos , Microglía/inmunología , Células Mieloides/inmunología , Receptores CCR2/genética , Regulación hacia Arriba
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