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1.
World Neurosurg ; 136: e310-e321, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31926359

RESUMO

OBJECTIVE: To study the natural growth dynamics of skull base chordomas. METHODS: A retrospective study of skull base chordomas was performed. Patients with ≥2 preoperative magnetic resonance (MR) images and with pathologically confirmed chordomas were enrolled. All clinical data and MR images were studied. RESULTS: Twenty-one patients with pathologically confirmed skull base chordomas were enrolled. The mean volume of the tumors at diagnosis was 19.9 ± 17.0 cm3, with a mean interval examination period of 22.4 ± 26.1 (range, 3-113) months. The mean tumor volume change was approximately 15.4 ± 16.3 cm3. The mean specific growth rate was 8% ± 9% per month, and the mean specific growth volume was 0.8 ± 0.7 cm3 per month. The tumor MR signal index grade, female gender, no dura mater breakthrough, endophytic type, small tumors, and soft tumor texture were related to a higher tumor growth rate (P < 0.05), and small tumors showed the greatest growth rate compared with the middle-sized and large tumors. Curve estimation was performed using a power function (R2 = 0.545). CONCLUSIONS: The skull base chordoma is a slow-growing tumor. The cases involving characteristics of female gender, endophytic type, small tumor size, and MR grade 3 showed a higher growth rate.


Assuntos
Cordoma/patologia , Neoplasias da Base do Crânio/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
2.
Br J Radiol ; 93(1107): 20190028, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31322969

RESUMO

OBJECTIVE: To assess the radiation-induced optic neuropathy (RION) prevalence, following high dose pencil beam scanning proton therapy (PBSPT) to skull base and head and neck (H&N) tumours. METHODS: Between 1999 and 2014, 216 adult patients, median age 47 years (range, 18-77), were treated with PBS PT for skull base or H&N malignancies, delivering ≥45 GyRBE to the optic nerve(s) (ON) and/or optic chiasma (OC). The median administered dose to the planning target volume was 74.0 GyRBE (range, 54.0-77.4). The median follow-up was 5.3 years (range, 0.8-15.9). RESULTS: RION was observed in 14 (6.5%) patients at a median time of 13.2 months (range, 4.8-42.6) following PBSPT. Most (92.9%) of RION were symptomatic. Most affected patients (11/14; 79%) developed unilateral toxicity. Grade 4, 3, 2 and 1 toxicity was observed in 10, 2, 1 and 1 patients, respectively. On univariate analyses, age (<70 vs ≥70 years; p < 0.0001), hypertension (p = 0.0007) and tumour abutting the optic apparatus (p = 0.012) were associated with RION. OC's V60 GyRBE was of border line significance (p = 0.06). None of the other evaluated OC-ON dose/volume metrics (Dmax, Dmean, V40-60) were significantly associated with this complication. CONCLUSION: These data suggest that high-dose PBS PT for skull base and H&N tumours is associated with a low prevalence of RION. Caution should be however exercised when treating elderly/hypertensive patients with tumours abutting the optic apparatus. ADVANCES IN KNOWLEDGE: This is the first study reporting the risk of developing RION following proton therapy with PBS technique, demonstrating the safety of this treatment.


Assuntos
Neoplasias de Cabeça e Pescoço/radioterapia , Doenças do Nervo Óptico/etiologia , Nervo Óptico/efeitos da radiação , Terapia com Prótons/efeitos adversos , Lesões por Radiação/complicações , Neoplasias da Base do Crânio/radioterapia , Adolescente , Adulto , Fatores Etários , Idoso , Análise de Variância , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Hipertensão/complicações , Pessoa de Meia-Idade , Quiasma Óptico/efeitos da radiação , Doenças do Nervo Óptico/epidemiologia , Doenças do Nervo Óptico/patologia , Prevalência , Terapia com Prótons/métodos , Dosagem Radioterapêutica , Radioterapia Adjuvante , Fatores de Risco , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/cirurgia , Adulto Jovem
3.
Pediatr Blood Cancer ; 67(2): e28080, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31736243

RESUMO

PURPOSE: Despite the dosimetric advantages of proton therapy, little data exist on patients who receive proton therapy for Ewing sarcoma of the cranium and skull base. This study reports local disease control and toxicity in such patients. MATERIALS/METHODS: We reviewed 25 patients (≤21 years old) with nonmetastatic Ewing sarcoma of the cranium and skull base treated between 2008 and 2018. Treatment toxicity was graded per the Common Terminology Criteria for Adverse Events v4.0. The Kaplan-Meier product limit method provided estimates of disease control and survival. RESULTS: Median patient age was 5.9 years (range, 1-21.7). Tumor subsites included the skull base (48%), non-skull-base calvarial bones (28%), paranasal sinuses (20%), and nasal cavity (4%). All patients underwent multiagent alkylator- and anthracycline-based chemotherapy; 16% underwent gross total resection (GTR) before radiation. Clinical target volume (CTV) 1 received 45 GyRBE and CTV2 received 50.4 GyRBE following GTR or 54-55.8 GyRBE following biopsy or subtotal resection. Median follow-up was 3.7 years (range, 0.26-8.3); no patients were lost. The 4-year local control, disease-free survival, and overall survival rates were 96%, 86%, and 92%, respectively. Two patients experienced in-field recurrences. One patient experienced bilateral conductive hearing loss requiring aids, two patients developed intracranial vasculopathy, and 6 patients required hormone replacement therapy for neuroendocrine deficits. None developed a secondary malignancy. CONCLUSION: Proton therapy is associated with a favorable therapeutic ratio in children with large Ewing tumors of the cranium and skull base. Despite its high conformality, we observed excellent local control and no marginal recurrences. Treatment dosimetry predicts limited long-term neurocognitive and neuroendocrine side effects.


Assuntos
Neoplasias Ósseas/mortalidade , Neoplasias dos Nervos Cranianos/mortalidade , Recidiva Local de Neoplasia/mortalidade , Terapia com Prótons/mortalidade , Sarcoma de Ewing/mortalidade , Neoplasias da Base do Crânio/mortalidade , Adolescente , Adulto , Neoplasias Ósseas/patologia , Neoplasias Ósseas/radioterapia , Criança , Pré-Escolar , Neoplasias dos Nervos Cranianos/patologia , Neoplasias dos Nervos Cranianos/radioterapia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/radioterapia , Prognóstico , Estudos Prospectivos , Dosagem Radioterapêutica , Sarcoma de Ewing/patologia , Sarcoma de Ewing/radioterapia , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/radioterapia , Taxa de Sobrevida , Adulto Jovem
4.
World Neurosurg ; 134: e1099-e1107, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31785435

RESUMO

BACKGROUND: Skull base chordomas (SBCs) are rare clinically aggressive neoplasms, developing local recurrences after surgical resection. Although SBCs have traditionally been resected by craniotomy or microscopic transsphenoidal surgery (TSS), the recent development of the endoscopic endonasal approach (EEA) has revolutionized treatment strategies through minimally invasive techniques. This study aimed to evaluate clinical outcomes after traditional microsurgeries or EEAs for SBCs. METHODS: The present retrospective study investigated 66 patients with primary SBCs who underwent surgery between 1977 and 2019. Resection was performed via EEA in 17 cases, craniotomy in 23, transoral approach in 8, TSS in 12, staged surgery in 4, and others in 2. The median follow-up period for progression-free survival (PFS) was 19.5 months. RESULTS: There were no significant differences in preoperative tumor volume or resection rate among these approaches. The incidence of postoperative cranial nerve palsy was significantly lower in EEA than that in craniotomy (P < 0.05). Although total resection was observed in 4 cases of EEA expanding into the superior and inferior part of the clivus, no cases of transoral approach or TSS achieved total resection for both parts. No significant difference in PFS was found among these approaches. Multivariate analysis showed that being female and the absence of radiotherapy were significantly associated with shorter PFS (P < 0.05 and P < 0.001, respectively). The resection rate was not associated with PFS. CONCLUSIONS: EEA is a less invasive surgical approach for SBCs. The development of surgical instruments and postoperative radiotherapy will further improve patients' outcomes.


Assuntos
Cordoma/cirurgia , Craniotomia/métodos , Microcirurgia/métodos , Neuroendoscopia/métodos , Neoplasias da Base do Crânio/cirurgia , Adolescente , Adulto , Idoso , Vazamento de Líquido Cefalorraquidiano/epidemiologia , Criança , Cordoma/patologia , Doenças dos Nervos Cranianos/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Boca , Cavidade Nasal , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/epidemiologia , Intervalo Livre de Progressão , Estudos Retrospectivos , Neoplasias da Base do Crânio/patologia , Carga Tumoral , Adulto Jovem
5.
J Neuropathol Exp Neurol ; 79(3): 314-324, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31841164

RESUMO

Chordomas are slow-growing rare malignant neoplasms. The aim of this study was to establish a primary model of chordoma in the lumbosacral orthotopic area, to compare the growth rate to the subcutaneous site, and to show that this new graft site optimizes tumor growth and bony invasion. Eleven chordoma samples were transplanted subcutaneously in the flank and/or in contact with the lumbosacral region and grown into nude mice. Engraftment rate was significantly more successful in the lumbosacral environment compared with the flank at P0. Two xenografts from 2 patients showed bone invasion. One tumor was maintained through multiple rounds of serial transplantation, creating a model for study. Histological and immunostaining analysis confirmed that tumor grafts recapitulated the primary tumor from which they were derived, consisting of a myxoid chordoma expressing brachyury, cytokeratin AE1, EMA, and VEGF. Clear destruction of the bone by the tumor cells could be demonstrated. Molecular studies revealed PIK3CA and PTEN mutations involved in PI3K signaling pathway and most of the frequently reported chromosomal alterations. We present a novel orthotopic primary xenograft model of chordoma implanted for the first time in the lumbosacral area showing bone invasion, PIK3CA, and PTEN mutations that will facilitate preclinical studies.


Assuntos
Cordoma/patologia , Cordoma/fisiopatologia , Modelos Animais de Doenças , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/fisiopatologia , Neoplasias da Medula Espinal/patologia , Neoplasias da Medula Espinal/fisiopatologia , Adulto , Idoso , Animais , Feminino , Xenoenxertos , Humanos , Região Lombossacral , Masculino , Pessoa de Meia-Idade , Adulto Jovem
6.
Medicina (B Aires) ; 79(6): 453-460, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31829947

RESUMO

Stereotactic radiosurgery has shown effectiveness in the multimodal treatment of skull base tumors, however it is suggested that in radiobiologically sensitive areas (eg. optic nerves and brainstem) the radiation dose should be reduced due to radiotoxic effects. To avoid the consequent therapeutic ineffectiveness, a recently implemented alternative is multisession radiosurgery (RCH). We retrospectively analyzed a series of patients with skull base tumors, in order to evaluate the treatment with RCH through tumor control in the post-treatment images. Age, sex, tumor histology, tumor volume, radiation protocol, pre-treatment Karnofsky performance status (KPS) previous neurosurgery and radiant treatment were also evaluated. Eighty-four patients were treated between January 2009 and January 2017. The median age was 51.5 years; females: 53.6%. There was a 92.7% non-progression after treatment, with a median follow-up time of 36 months. Treated tumors were mainly pituitary adenomas, acoustic schwannomas, and skull base meningiomas. Most of the patients received a 5-day fractionated treatment scheme, with a total dose of 25 Gy. No clinically manifest late radiotoxicity was observed. In the multivariate analysis, a high pre-treatment KPS was significantly associated with tumor non-progression. In our series, the high incidence of tumor non-progression would indicate that treatment with RCH could be a therapeutic option in some cases of skull-base tumors, mainly recurrences or tumor residuals of pituitary adenomas, neurinomas and meningiomas.


Assuntos
Radiocirurgia/métodos , Neoplasias da Base do Crânio/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Avaliação de Estado de Karnofsky , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Doses de Radiação , Reprodutibilidade dos Testes , Estudos Retrospectivos , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/patologia , Fatores de Tempo , Carga Tumoral
7.
BMJ Case Rep ; 12(12)2019 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-31796459

RESUMO

Chondrosarcomas of head and neck region are rare. Very few cases of chondrosarcomas arising in parotid gland have been reported and none with intracranial extension. We report a case of a female presenting with a parotid swelling and a mass in external auditory canal with extradural extension to posterior cranial fossa. With a preoperative fine needle aspiration diagnosis of pleomorphic adenoma, it was excised and the histopathology came out to be low-grade myxoid chondrosarcoma. She has not received any adjuvant chemoradiotherapy and there is no evidence of recurrence at months.


Assuntos
Condrossarcoma/patologia , Neoplasias Parotídeas/patologia , Neoplasias da Base do Crânio/patologia , Biópsia por Agulha Fina , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/cirurgia , Meato Acústico Externo/patologia , Feminino , Perda Auditiva Condutiva/etiologia , Humanos , Imagem por Ressonância Magnética , Mastoidectomia , Invasividade Neoplásica , Neoplasias Parotídeas/diagnóstico por imagem , Neoplasias Parotídeas/cirurgia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Tomografia Computadorizada por Raios X , Adulto Jovem
8.
Orv Hetil ; 160(40): 1584-1590, 2019 Oct.
Artigo em Húngaro | MEDLINE | ID: mdl-31565974

RESUMO

Introduction: Malignant tumours of the sinonasal region - including those with invasion of the skull base - necessitate surgical resection. The majority of the cases give an opportunity to perform the procedure via minimally invasive, endoscopic approach, without external, craniofacial surgery. Aim: To assess our clinical experience in treating anterior skull base malignancies, performing minimally invasive endoscopic transcribriform resection. Method: Between February 2015 and July 2017, four male and one female patient underwent minimally invasive, endoscopic skull base procedure. The mean age was 64.6 years (59-70, median: 66). Every surgery was performed via transnasal, endoscopic transcribriform approach. In two cases Kadish C esthesioneuroblastomas, while in one case a T3N0 sinonasal non-differentiated carcinoma, a T1N0 intestinal type adenocarcinoma and a T4N0 squamous cell carcinoma was the indication of surgery, respectively. Results: The mean follow-up time was 22.8 months, between 14 and 46 months. Intraoperative complications did not occur during the procedures. Regarding the postoperative period, liquorrhoea and pneumocephalus occurred in one case. Complications were solved with lumbar drainage. During follow-up, neither residual nor recurrent tumour was observed in our patients. Conclusion: Endoscopic transcribriform resection of the skull base malignancies is a safe and viable alternative to the traditional open approach. Orv Hetil. 2019; 160(40): 1584-1590.


Assuntos
Endoscopia , Procedimentos Cirúrgicos Minimamente Invasivos/instrumentação , Neuroendoscopia/métodos , Procedimentos Neurocirúrgicos/métodos , Neoplasias dos Seios Paranasais/cirurgia , Neoplasias da Base do Crânio/cirurgia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Recidiva Local de Neoplasia , Neuroendoscopia/efeitos adversos , Neoplasias dos Seios Paranasais/patologia , Base do Crânio/cirurgia , Neoplasias da Base do Crânio/patologia , Resultado do Tratamento
9.
Radiologe ; 59(12): 1064-1070, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31602499

RESUMO

Malignant skull base tumors consist of a heterogeneous group of malignancies that can be divided into primary and secondary (metastatic) skull base tumors. In addition, according their anatomical location, they can be further divided into tumors of the anterior, middle, or posterior cranial fossa. Although malignant skull base tumors do not rigorously respect anatomical borders, their anatomical occurrence can potentially be helpful for possible differential diagnosis. This article is focused on the most common malignant tumors of the skull base and their imaging and clinical presentations.


Assuntos
Neoplasias da Base do Crânio , Base do Crânio , Fossa Craniana Posterior , Diagnóstico Diferencial , Humanos , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/patologia
10.
Artigo em Chinês | MEDLINE | ID: mdl-31550754

RESUMO

Objective: To develop the Chinese version of anterior skull base questionnaire(ASBQ) and to verify its application in patients with anterior and middle skull base tumors. Methods: The following steps were finished including getting the permission from the author of the original English scale, translating and back-translating, tentative test, discussing the consequence and cultural debugging. From October 2016 to December 2018, 51 patients with skull base tumors from Xuanwu Hospital and China-Japan Friendship Hospital were enrolled as an experimental group, aged from 24 to 70 years old, with 19 males and 32 females, which included 27 patients with anterior skull base tumor and 24 patients with middle skull base tumor. From December 2016 to January 2018, 46 healthy volunteers were selected as a control group, aged from 18 to 36 years old, including 26 females and 20 males. The subjects in the test group and the control group were rigorously tested with official manuscripts and judged whether the manuscript was applicable. The SPSS 22.0 statistical software was used to analyze the data of the test group, the anterior skull base group, the middle skull base group and the control group to evaluate the performance of the scale. Results: Both the rate of the recovery and efficiency in experimental group, anterior skull base group and middle skull base group were 100%, with the average time of completion of (8.7±3.2), (11.2±4.0) and (7.3±2.1) min, respectively in each group. The r value of test-retest reliability was 0.96, 0.99 and 0.97 in experimental group, anterior skull base group and middle skull base group, with the split-half reliability coefficient of 0.91, 0.90 and 0.96, with the entire scale Cronbach's coefficient of 0.91, 0.95 and 0.93, respectively. The content validity and the construct validity of the scale were good enough, and the criteria validity was-0.483,-0.509 and -0.489 in experimental group, anterior skull base group and middle skull base group. The scale could well distinguish the difference of the quality of life between the preoperative and postoperative patients in experimental group and the middle skull base group. The difference of the quality of life in anterior skull base group was not found between preoperative and postoperative patients. Conclusion: The Chinese version of ASBQ has good reliability and validity, which is suitable for a wide range of Chinese patients with anterior and middle skull base tumors to assess their quality of life.


Assuntos
Neoplasias da Base do Crânio , Inquéritos e Questionários , Adolescente , Adulto , Idoso , China , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Reprodutibilidade dos Testes , Base do Crânio/patologia , Neoplasias da Base do Crânio/patologia , Adulto Jovem
11.
Eur J Radiol ; 118: 81-87, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31439263

RESUMO

PURPOSE: Patients with skull base chordoma and chondrosarcoma have different prognoses and are not readily differentiated preoperatively on imaging. Multiparametric magnetic resonance imaging (MRI) is a routine diagnostic tool that can noninvasively characterize the salient characteristics of tumors. In the present study, we developed and validated a preoperative multiparametric MRI-based radiomic signature for differentiating these tumors. METHOD: This retrospective study enrolled 210 patients and consecutively divided them into the primary and validation cohorts. A total of 1941 radiomic features were acquired from preoperative T1-weighted imaging, T2-weighted imaging and contrast-enhanced T1-weighted imaging for each patient. The most discriminative features were selected by minimum-redundancy maximum-relevancy and recursive feature elimination algorithms in the primary cohort. The multiparametric and single-sequence MRI signatures were constructed with the selected features using a support vector machine model in the primary cohort. The ability of the novel radiomic signatures to differentiate chordoma from chondrosarcoma were assessed using receiver operating characteristic curve analysis in the validation cohort. RESULTS: The multiparametric radiomic signature, which consisted of 11 selected features, reached an area under the receiver operating characteristic curve of 0.9745 and 0.8720 in the primary and validation cohorts, respectively. Moreover, compared with each single-sequence MRI signature, the multiparametric radiomic signature exhibited better classification performance with significant improvement (p <  0.05, Delong's test) in the primary cohorts. CONCLUSION: By combining features from three MRI sequences, the multiparametric radiomics signature can accurately and robustly differentiate skull base chordoma from chondrosarcoma.


Assuntos
Condrossarcoma/patologia , Cordoma/patologia , Neoplasias da Base do Crânio/patologia , Adolescente , Adulto , Idoso , Algoritmos , Criança , Pré-Escolar , Estudos de Coortes , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética Multiparamétrica/métodos , Prognóstico , Curva ROC , Estudos Retrospectivos , Máquina de Vetores de Suporte
12.
World Neurosurg ; 130: 512-515, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31279922

RESUMO

BACKGROUND: Hemangiomas are benign blood vessels tumors that represent less than 1% of all the bone neoplasms. Calvarial hemangiomas are mainly solitary lesions commonly located in the frontal and parietal bone; however, they may occur in any skull region. These tumors increase in size over a period of months to years before they start showing their first symptoms such as headache, bone deformity, and pathological fractures. Differential diagnosis with osteosarcoma should be considered. Surgical resection with a safety margin is a standard treatment of the cranial hemangioma. Furthermore, radiotherapy has proven to stop the tumor's growth but not its size. CASE DESCRIPTION: We treated an 11-year-old male who had a rare case of a capillary hemangioma located in the clivus bone. The patient underwent 2 endoscopic endonasal resection because of tumor recurrence. Surgical safety margins are highly recommended, but this procedure could not be performed because of the tumor's location. After the second relapse, the oncology team decided to initiate radiotherapy. At 6-month follow-up, the tumor reduced its size and remained unchanged. CONCLUSIONS: Surgical safety margins are highly important to prevent recurrence in this type of bone tumors. Skull base hemangiomas are a big challenge when you want to achieve these safety margins. We believe that a combined treatment of surgery and radiotherapy should be considered as the main treatment.


Assuntos
Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Hemangioma/diagnóstico , Hemangioma/patologia , Neoplasias da Base do Crânio/diagnóstico , Neoplasias da Base do Crânio/patologia , Criança , Fossa Craniana Posterior/irrigação sanguínea , Diagnóstico Diferencial , Humanos , Masculino , Osteossarcoma/diagnóstico , Osteossarcoma/patologia , Neoplasias da Base do Crânio/irrigação sanguínea
13.
World Neurosurg ; 130: e620-e626, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31265927

RESUMO

OBJECTIVE: The aim of this study was to discuss the relationship between Ki-67 values and the degree to which chordoma invade the clivus and to certify that the prognosis of chordoma is worse when it invades the middle and lower clivus than when it does not. METHODS: We collected 56 cases of first-time chordoma illness in which patients received no treatment before surgery. Patients underwent craniocerebral magnetic resonance imaging and skull-base 3-dimensional computed tomography scans before the operation. We divided patients into 2 groups depending on the extent to which the middle and lower clivus were invaded. We classified patients with chordoma that did not significantly invade the middle and lower clivuses into a "noninvasive" group and the others into an "invasive" group. Ki-67 values were extracted from the pathological report after surgery. We use an independent χ2 test to indicate that Ki-67 values for the invasive group were higher than those for the noninvasive group. RESULTS: We grouped the data and did a statistical analysis. We found that the Ki-67 values are >5% for most patients in whom chordoma have eroded the middle-lower clivus, whereas it is ≤5% for patients in whom the middle-lower clivus region has not been invaded. Therefore, there is a correlation between Ki-67 value and the region of chordoma invading the clivus. CONCLUSIONS: Statistical analysis revealed that Ki-67 values when the chordoma invaded the middle and lower clivus were significantly higher than when it did not. Thus, we can conclude that the prognosis is worse when chordoma invade the middle and lower clivus.


Assuntos
Cordoma/patologia , Fossa Craniana Posterior/patologia , Invasividade Neoplásica/patologia , Neoplasias da Base do Crânio/patologia , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/análise , Cordoma/diagnóstico por imagem , Fossa Craniana Posterior/diagnóstico por imagem , Feminino , Humanos , Antígeno Ki-67/análise , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica/diagnóstico por imagem , Prognóstico , Neoplasias da Base do Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto Jovem
14.
World Neurosurg ; 131: e265-e270, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31349076

RESUMO

OBJECTIVE: To investigate the expression characteristics and prognostic value of transforming growth factor ß1 (TGF-ß1) in primary skull base chordomas (SBCs). METHODS: The mRNA expression levels of TGF-ß1 were measured in 57 frozen samples from patients with primary SBCs. Clinical data collection, follow-up, correlations, and survival analyses were performed. RESULTS: In the series of 57 patients (29 men and 28 women) with primary SBCs, the mean value of TGF-ß1 mRNA was 1.713 with a median of 0.904. Twenty-four SBCs were soft type and 33 were hard type. The Mann-Whitney U test revealed that the expression level of TGF-ß1 mRNA in hard type SBCs was significantly higher than the expression level found in the soft type (P = 0.03). The independent-samples median test suggested that the expression level of TGF-ß1 mRNA in female patients' SBCs was significantly higher than that in male patients' SBCs (P = 0.01). Expression differences of TGF-ß1 were not seen among different pathological subtypes, tumor blood supply, or degree of resection. The Spearman rank correlation coefficient clarified that TGF-ß1 mRNA levels were not correlated with tumor diameter, preoperative Karnofsky Performance Status (KPS), postoperative KPS, follow-up KPS, age, or intraoperative blood loss. The multivariate Cox analysis revealed that pathological subtype (P = 0.008), expression level of TGF-ß1 mRNA (P = 0.01), and tumor texture (P = 0.03) were all independent prognostic factors for tumor progression. CONCLUSIONS: SBCs in female patients and SBCs with hard texture were prone to have high TGF-ß1 mRNA expression. High expression of TGF-ß1, hard tumor texture, and conventional subtype were all independent risk factors for tumor progression.


Assuntos
Cordoma/genética , Neoplasias da Base do Crânio/genética , Fator de Crescimento Transformador beta1/genética , Adolescente , Adulto , Criança , Cordoma/irrigação sanguínea , Cordoma/patologia , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , RNA Mensageiro/metabolismo , Fatores Sexuais , Neoplasias da Base do Crânio/irrigação sanguínea , Neoplasias da Base do Crânio/patologia , Carga Tumoral , Adulto Jovem
15.
Neuroradiology ; 61(12): 1355-1364, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31324948

RESUMO

PURPOSE: A subset of skull base meningiomas (SBM) may show early progression/recurrence (P/R) as a result of incomplete resection. The purpose of this study is the implementation of MR radiomics to predict P/R in SBM. METHODS: From October 2006 to December 2017, 60 patients diagnosed with pathologically confirmed SBM (WHO grade I, 56; grade II, 3; grade III, 1) were included in this study. Preoperative MRI including T2WI, diffusion-weighted imaging (DWI), and contrast-enhanced T1WI were analyzed. On each imaging modality, 13 histogram parameters and 20 textural gray level co-occurrence matrix (GLCM) features were extracted. Random forest algorithms were utilized to evaluate the importance of these parameters, and the most significant three parameters were selected to build a decision tree for prediction of P/R in SBM. Furthermore, ADC values obtained from manually placed ROI in tumor were also used to predict P/R in SBM for comparison. RESULTS: Gross-total resection (Simpson Grades I-III) was performed in 33 (33/60, 55%) patients, and 27 patients received subtotal resection. Twenty-one patients had P/R (21/60, 35%) after a postoperative follow-up period of at least 12 months. The three most significant parameters included in the final radiomics model were T1 max probability, T1 cluster shade, and ADC correlation. In the radiomics model, the accuracy for prediction of P/R was 90%; by comparison, the accuracy was 83% using ADC values measured from manually placed tumor ROI. CONCLUSIONS: The results show that the radiomics approach in preoperative MRI offer objective and valuable clinical information for treatment planning in SBM.


Assuntos
Imagem por Ressonância Magnética/métodos , Meningioma/diagnóstico por imagem , Recidiva Local de Neoplasia/diagnóstico por imagem , Neoplasias da Base do Crânio/diagnóstico por imagem , Adulto , Algoritmos , Meios de Contraste , Árvores de Decisões , Progressão da Doença , Feminino , Humanos , Interpretação de Imagem Assistida por Computador , Masculino , Meningioma/patologia , Meningioma/cirurgia , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Valor Preditivo dos Testes , Estudos Retrospectivos , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/cirurgia
16.
J Laryngol Otol ; 133(6): 501-507, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31271349

RESUMO

OBJECTIVE: Prompted by a recurring skull base multidisciplinary team debate on the necessity of securing a definitive tissue diagnosis before initiating treatment for lesions of the orbital apex, a review of anterior skull base procedures over an 11-year period was undertaken. METHODS: Data collected prospectively on cases from 2006 to 2017 were analysed. Presenting symptoms, imaging and histology findings, outcomes, complications, and impact on treatment were evaluated. All surgery was carried out endoscopically with the aid of image guidance. RESULTS: Twenty-one patients undergoing endoscopic orbital apex and/or optic canal biopsy were included. The mean patient age was 49 years. Five malignant tumours were identified, five benign tumours, seven infective cases (two tuberculosis and five fungal) and two cases of immunoglobulin G4 related disease. Two patients had non-diagnostic biopsies (one lesional) and were treated successfully as Tolosa-Hunt syndrome cases. CONCLUSION: A successful diagnosis was achieved in nearly all cases without adverse impact, other than one cerebrospinal fluid leakage case. Management was directly influenced by the outcome in all cases.


Assuntos
Biópsia Guiada por Imagem/métodos , Imageamento Tridimensional , Órbita/cirurgia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia , Adulto , Fatores Etários , Idoso , Endoscopia/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Cavidade Nasal/diagnóstico por imagem , Cavidade Nasal/patologia , Cavidade Nasal/cirurgia , Órbita/diagnóstico por imagem , Órbita/patologia , Medição da Dor , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Medição de Risco , Fatores Sexuais , Neoplasias da Base do Crânio/patologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
17.
J Neurooncol ; 144(1): 65-77, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31240525

RESUMO

BACKGROUND: Chordoma is a rare refractory neoplasm that arises from the embryological remnants of the notochord, which is incurable using any multimodality therapy. Vascular endothelial growth factor (VEGF) is a potent activator of angiogenesis that is strongly associated with the tumor-immune microenvironment. These factors have not been elucidated for chordomas. METHODS: To evaluate the characteristics of vascular and tumor cells in chordoma, we first analyzed the expression of VEGF receptor (VEGFR) 1, VEGFR2, CD34, and Brachyury in a cell line and 54 tumor tissues. Patients with primary skull base chordomas were divided into the following two groups as per the tumor growth rate: patients with slow progression (SP: < 3 mm/year) and those with rapid progression (RP: ≥ 3 mm/year). Thus, the expressions of VEGF-A, VEGFR 1, and VEGFR2 on tumor cells; tumor infiltrative immune cells, including regulatory T cells (Tregs) and tumor-associated macrophages (TAMs); and immune-checkpoint molecules (PD-1/PD-L1) were analyzed with the clinical courses, especially in a comparison between the two groups. RESULTS: In chordomas, both VEGFR1 and VEGFR2 were strongly expressed not only on vascular endothelial cells, but also on tumor cells. The recurrent cases showed significantly higher VEGFR1 expressions on tumor cells than the primary cases. The expression of VEGF-A was significantly higher in RP than that in SP group. The numbers of CD163+ TAMs and Foxp3+ Tregs were higher in RP than that in SP group. CONCLUSIONS: Expression of VEGFR1 and VEGFR2 on tumor cells and immunosuppressive tumor-microenvironment were related to tumor growth in patients with chordomas.


Assuntos
Biomarcadores Tumorais/metabolismo , Cordoma/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias da Base do Crânio/patologia , Receptor 1 de Fatores de Crescimento do Endotélio Vascular/metabolismo , Receptor 2 de Fatores de Crescimento do Endotélio Vascular/metabolismo , Adolescente , Adulto , Idoso , Antígenos CD34/metabolismo , Cordoma/metabolismo , Cordoma/cirurgia , Feminino , Proteínas Fetais/metabolismo , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/metabolismo , Recidiva Local de Neoplasia/cirurgia , Prognóstico , Neoplasias da Base do Crânio/metabolismo , Neoplasias da Base do Crânio/cirurgia , Proteínas com Domínio T/metabolismo , Adulto Jovem
18.
World Neurosurg ; 130: e356-e361, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31233929

RESUMO

BACKGROUND: Maffucci syndrome (MS) and Ollier disease (OD) are rare diseases characterized by multiple benign enchondromas. The incidence of skull base (SB) enchondromas and the risk of malignant transformation remain unknown. Most SB lesions are asymptomatic, and surgical resection carries significant morbidity. Observation may be a reasonable strategy. We report our experience with observation of probable SB enchondromas in MS/OD patients. METHODS: Retrospective review of OD/MS patients with cranial imaging between 1995 and 2018 at 1 institution. RESULTS: 14 patients were included: 3 with MS (21.4%) and 11 with OD (78.6%). The median age was 28 years (range, 11-74 years) and 57.1% were female. Extracranial chondrosarcoma was reported in 3 (21.4%) patients. Seven (50%) patients with SB enchondroma or chondrosarcoma were identified on initial imaging. In patients with SB lesions, the indications for imaging were headache (n=3), seizure (n=1), and diplopia (n=1); 2 cases were incidental findings. The most commonly involved structures were petroclival fissure (86%) and clivus (71%). Treatment included observation (6/7) and resection (1/7). Follow-up imaging was available for all SB lesions, with a mean interval of 50.7 months (range, 5-225 months) and was negative for progression in all patients. CONCLUSIONS: Primary SB lesions in OD/MS patients frequently present in the petroclival junction. Cranial screening and close observation should be considered in MS/OD patients, given the increased risk of intra-axial intracranial tumors, de novo chondrosarcomas, or malignant degeneration of previously known lesions. In asymptomatic patients, observation appears to be a safe strategy in this cohort. Further case accumulation and follow-up are required to better understand the long-term outcomes.


Assuntos
Neoplasias Ósseas/epidemiologia , Condroma/epidemiologia , Condrossarcoma/epidemiologia , Encondromatose/epidemiologia , Neoplasias da Base do Crânio/epidemiologia , Adolescente , Adulto , Idoso , Neoplasias Ósseas/complicações , Neoplasias Ósseas/patologia , Criança , Condroma/complicações , Condroma/patologia , Condrossarcoma/complicações , Condrossarcoma/patologia , Encondromatose/complicações , Encondromatose/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias da Base do Crânio/complicações , Neoplasias da Base do Crânio/patologia , Adulto Jovem
19.
Eur Arch Otorhinolaryngol ; 276(9): 2465-2473, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31203383

RESUMO

INTRODUCTION: Inflammatory pseudotumor (IPT) in the sinonasal cavity and skull base region is benign non-neoplastic inflammatory process. However, IPT can mimic malignant tumor or infectious disease and there are difficulties in confirmation of diagnosis. The aim of study is to evaluate the clinical significance of immunoglobulin G4 (IgG4) in IPT in terms of steroid response and differential diagnosis with other skull base infiltrative lesions. METHODS: Medical records were reviewed retrospectively from 1998 to 2016. Subjects diagnosed with IPT by surgical biopsy were enrolled. IgG4 positivity was defined as IgG4/IgG ratio > 0.4. Additionally, IgG4/IgG ratio was calculated in eight skull base osteomyelitis (SBO) patients. RESULTS: Twenty-six IPT patients were included and the average age was 52.3 years, and 57.7% were male and 42.3% were female. Most lesions were involved in the sinuses (88.5%) and the incidence of extension beyond the sinuses itself was as follows: the cheek/hard palate/parapharynx (15.4%), orbit (61.5%), skull base (57.7%), and dura or brain (23.1%). All IPT cases revealed IgG4 + plasma cells and IgG4/IgG ratio over 0.4 was detected in 42.3% (11/26) of cases. In case of SBO, no patients had IgG4/IgG ratio exceed 0.4. Main treatment modality was systemic steroids (61.5%) and other modalities were used: surgery (3.8%), immunosuppressant (7.7%), radiotherapy (30.8%), or a combination of these modalities (15.4%). Steroid responses were not significantly different, but IgG4-positive group tended to have better response to steroid therapy. CONCLUSIONS: IgG4-positive and IgG4-negative IPT patients revealed no differences in involvement sites, clinical course, and steroid responses. However, IgG4/IgG ratio and IgG4 + plasma cell count can provide a diagnostic clue for infiltrative skull base lesions such as IPT and a differential diagnosis of SBO.


Assuntos
Granuloma de Células Plasmáticas/imunologia , Imunoglobulina G/sangue , Neoplasias Nasais/imunologia , Neoplasias da Base do Crânio/imunologia , Adulto , Idoso , Biópsia , Contagem de Células Sanguíneas , Criança , Diagnóstico Diferencial , Feminino , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/patologia , Estudos Retrospectivos , Base do Crânio/patologia , Neoplasias da Base do Crânio/diagnóstico , Neoplasias da Base do Crânio/patologia
20.
J Neurooncol ; 144(1): 11-20, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31177425

RESUMO

AIMS: Skull base meningiomas represent approximately 25% of all meningiomas, nearly 20% of which are atypical or anaplastic. To date, effective medical treatments for meningiomas are still lacking. Genetic aberrations (TRAF7, KLF4, AKT1, and SMO) and the effects of genetic aberrations on the expression of inhibitory immune checkpoint molecules (PD-L1, IDO, and TDO2) in skull base meningiomas are still unclear. METHODS: Genetic alterations in the four genes were identified in 92 skull base meningiomas by Sanger sequencing. The expression differences in immune checkpoints between mutant and wild-type (WT) tumors were determined by immunohistochemistry (IHC) and Western blot (WB). RESULTS: The four mutations were not concurrently detected in the patients with skull base meningiomas. Among the tumors from the KLF4-mutated group, almost half were petroclival meningiomas. KLF4- and TRAF7-mutated tumors were predominantly secretory meningiomas. SMO-mutated tumors exhibited higher calcification, and half of these tumors were observed in the brain midline. Receiver operating characteristic curve analysis indicated that tumor volume can predict KLF4 and TRAF7 mutation status with high sensitivity and specificity, respectively. The IHC and WB analyses indicated that PD-L1, IDO, and TDO2 levels in tumors with TRAF7 mutations were significantly higher than those in WT tumors. Meanwhile, there was a significant difference in TDO2 between tumors with AKT1 mutations and WT tumors. Specifically, TRAF7 mutations could play a key role in skull base meningiomas by regulating the expression of inhibitory immune checkpoints and thus suppressing immune responses. CONCLUSIONS: Checkpoint inhibitors may be potential strategies for targeted immunotherapies of these mutant meningiomas.


Assuntos
Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Regulação Neoplásica da Expressão Gênica , Neoplasias Meníngeas/patologia , Meningioma/patologia , Mutação , Neoplasias da Base do Crânio/patologia , Adolescente , Adulto , Idoso , Antígeno B7-H1/genética , Antígeno B7-H1/metabolismo , Feminino , Seguimentos , Humanos , Indolamina-Pirrol 2,3,-Dioxigenase/genética , Indolamina-Pirrol 2,3,-Dioxigenase/metabolismo , Fatores de Transcrição Kruppel-Like/genética , Fatores de Transcrição Kruppel-Like/metabolismo , Masculino , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/imunologia , Neoplasias Meníngeas/metabolismo , Meningioma/genética , Meningioma/imunologia , Meningioma/metabolismo , Pessoa de Meia-Idade , Prognóstico , Proteínas Proto-Oncogênicas c-akt/genética , Proteínas Proto-Oncogênicas c-akt/metabolismo , Curva ROC , Neoplasias da Base do Crânio/genética , Neoplasias da Base do Crânio/imunologia , Neoplasias da Base do Crânio/metabolismo , Receptor Smoothened/genética , Receptor Smoothened/metabolismo , Triptofano Oxigenase/genética , Triptofano Oxigenase/metabolismo , Peptídeos e Proteínas Associados a Receptores de Fatores de Necrose Tumoral/genética , Peptídeos e Proteínas Associados a Receptores de Fatores de Necrose Tumoral/metabolismo , Adulto Jovem
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