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1.
Surg Pathol Clin ; 14(4): 619-643, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34742484

RESUMO

This review provides an overview of the spectrum of tumors showing notochordal differentiation. This spectrum encompasses benign entities that are mostly discovered incidentally on imaging, reported as benign notochordal cell tumor, usually not requiring surgical intervention; slowly growing and histologically low-grade tumors referred to as conventional chordoma but associated with a significant metastatic potential and mortality; and more aggressive disease represented by histologically higher-grade tumors including dedifferentiated chordoma, a high-grade biphasic tumor characterized by a conventional chordoma juxtaposed to a high-grade sarcoma, usually with a spindle or pleomorphic cell morphology, and associated with a poor prognosis and poorly differentiated chordoma.


Assuntos
Neoplasias Ósseas , Cordoma , Neoplasias Embrionárias de Células Germinativas , Neoplasias de Tecidos Moles , Diferenciação Celular , Cordoma/diagnóstico , Humanos
2.
Phys Med ; 90: 123-133, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34628271

RESUMO

PURPOSE: Carbon ion radiotherapy (CIRT) is sensitive to anatomical density variations. We examined the dosimetric effect of variable intestinal filling condition during CIRT to ten sacral chordoma patients. METHODS: For each patient, eight virtual computed tomography scans (vCTs) were generated by varying the density distribution within the rectum and the sigmoid in the planning computed tomography (pCT) with a density override approach mimicking a heterogeneous combination of gas and feces. Totally full and empty intestinal preparations were modelled. In addition, five different intestinal filling conditions were modelled by a mixed density pattern derived from two combined and weighted Gaussian distributions simulating gas and feces respectively. Finally, a patient-specific mixing proportion was estimated by evaluating the daily amount of gas detected in the cone beam computed tomography (CBCT). Dose distribution was recalculated on each vCT and dose volume histograms (DVHs) were examined. RESULTS: No target coverage degradation was observed at different vCTs. Rectum and sigma dose degradation ranged respectively between: [-6.7; 21.6]GyE and [-0.7; 15.4]GyE for D50%; [-377.4; 1197.9] and [-95.2; 1027.5] for AUC; [-1.2; 10.7]GyE and [-2.6; 21.5]GyE for D1%. CONCLUSIONS: Variation of intestinal density can greatly influence the penetration depth of charged particle and might compromise dose distribution. In particular cases, with large clinical target volume in very close proximity to rectum and sigmoid colon, it is appropriate to evaluate the amount of gas present in the daily CBCT images even if it is totally included in the reference planning structures.


Assuntos
Cordoma , Radioterapia com Íons Pesados , Cordoma/diagnóstico por imagem , Cordoma/radioterapia , Colo Sigmoide/diagnóstico por imagem , Tomografia Computadorizada de Feixe Cônico , Humanos , Dosagem Radioterapêutica , Planejamento da Radioterapia Assistida por Computador , Reto/diagnóstico por imagem
3.
Klin Lab Diagn ; 66(9): 533-538, 2021 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-34543531

RESUMO

The data of a comparative enzyme-linked immunosorbent assay of the content of the soluble form of the immunity checkpoint VISTA in the blood serum of 30 healthy donors (control group), 79 patients with primary malignant (osteosarcoma - 30, chondrosarcoma - 31, chordoma - 14) and 14 borderline (giant cell tumor) bone neoplasms are presented. In the general group of patients with malignant neoplasms of bones, the median sVISTA content in blood serum is statistically significant lower than in the control (p = 0.040). In patients with bone tumors and healthy donors over 18 years of age, there was a decrease with age in serum sVISTA levels. There were no significant differences in sVISTA concentration between patients with osteosarcoma, chondrosarcoma and healthy donors. Only in patients with chordoma were sVISTA levels statistically significant lower than in controls (p = 0.013). In the groups of patients with chondrosarcoma and osteosarcoma of the bone, there were no significant associations between the serum sVISTA content and the main clinical and morphological characteristics of the disease. In patients with osteosarcoma, no relationship was found between sVISTA levels and overall survival rates, while in patients with bone chondrosarcoma, there was a tendency towards a favorable prognosis with a high content of the marker in the blood serum.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Cordoma , Osteossarcoma , Adolescente , Adulto , Humanos , Prognóstico
4.
Eur Spine J ; 30(10): 2915-2924, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34338872

RESUMO

PURPOSE: Chordomas are rare tumors with an annual incidence of approximately one per million. Chordomas rarely metastasize but show a high local recurrence rate. Therefore, these patients present a major clinical challenge, and there is a paucity of the literature regarding the outcome after revision surgery of cervical spine chordomas. Available studies suggest a significantly worse outcome in revision scenarios. The purpose of this study is to analyze the survival rate, and complications of patients that underwent revision surgery for local recurrence or incomplete resection of chordoma at the craniocervical junction or at the cervical spine. METHODS: 24 consecutive patients that underwent revision surgery for cervical spine chordoma remnants or recurrence at a single center were reviewed retrospectively. We analyzed patient-specific surgical treatment strategies, complications, and outcome. Kaplan-Meier estimator was used to analyze five-year overall survival. RESULTS: Gross total resection was achieved in 17 cases. Seven patients developed dehiscence of the pharyngeal wall, being the most common long-term complication. No instability was observed. Postoperatively, four patients received proton beam radiotherapy and 12 patients had combined photon and proton beam radiotherapy. The five-year overall survival rate was 72.6%. CONCLUSION: With thorough preoperative planning, appropriate surgical techniques, and the addition of adjuvant radiotherapy, results similar to those in primary surgery can be achieved.


Assuntos
Cordoma , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Humanos , Recidiva Local de Neoplasia/cirurgia , Reoperação , Estudos Retrospectivos , Resultado do Tratamento
5.
Oper Neurosurg (Hagerstown) ; 21(5): E421-E426, 2021 10 13.
Artigo em Inglês | MEDLINE | ID: mdl-34392369

RESUMO

BACKGROUND: Chiari I malformations secondary to other causes represent a small subset of presenting symptomatic cases. Typically, the primary cause of the malformation is addressed first and results in resolution of the malformation and symptoms. However, in some cases, a patient may present with both a primary Chiari I malformation and another unrelated neurosurgical lesions. OBJECTIVE: To present a unique case in which resection of a ventral tumor allowed for spontaneous resolution of a simultaneously noted dorsal Chiari I malformation. METHODS: Pertinent data, including presenting symptoms, hospital course, surgical notes, preoperative images, and postoperative images, were collected using the electronic medical record. RESULTS: We present a case of a 46-yr-old man with a Chiari I malformation in conjunction with a ventral cranio-cervical junction chordoma. Endoscopic endonasal resection of the chordoma and ventral foramen magnum decompression resulted in radiographic resolution of the Chiari malformation and resolution of his symptoms. Our report represents a rare case of ventral foramen magnum decompression as a treatment for Chiari I malformation. CONCLUSION: It is felt that the chordoma mass effect was not the source of the Chiari I malformation. Thus, both ventral and dorsal decompressions of the posterior fossa may be considered for Chiari I decompression in select circumstances.


Assuntos
Malformação de Arnold-Chiari , Cordoma , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Descompressão Cirúrgica , Forame Magno/diagnóstico por imagem , Forame Magno/cirurgia , Humanos , Masculino
7.
Neuro Oncol ; 23(10): 1709-1722, 2021 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-34214167

RESUMO

BACKGROUND: Chordoma is a rare mesenchymal malignancy, with a high recurrence rate and unclear tumorigenic mechanism. Genetic alterations, epigenetic regulators, and chromatin spatial organization play crucial roles in the initiation and progression of chordoma. In the current study, we aim to uncover the novel therapeutical targets for chordoma via using integrated multi-omics analysis. METHODS: The RNA-sequencing (RNA-seq), assay for transposable accessible chromatin by high-throughput sequencing (ATAC-seq), and Hi-C were performed between chordoma and human nucleus pulposus (HNP), along with imageological examination and clinical information. The expressions of identified targets were validated by clinical samples and their functions were further evaluated by cell and animal experiments via gene knockdown and inhibitors. RESULTS: The integrated multi-omics analysis revealed the important roles of bone microenvironment in chordoma tumorigenesis. By comparing the hierarchical structures, CA2 (carbonic anhydrase II) and THNSL2 (threonine synthase-like 2) were identified in the switched compartments, cell-specific boundaries, and loops. Additionally, CA2 was highly expressed in chordoma but barely found in HNP. The cell growth and migration of chordoma cells were dramatically suppressed via inhibition of CA2 either with genetic deletion or pharmaceutical treatment with Dorzolamide HCl. Furthermore, Dorzolamide HCl also regulated the bone microenvironment by blocking the osteoclast differentiation of bone marrow monocytes. CONCLUSION: This study uncovers the roles of bone microenvironment in the chordoma tumorigenesis and identifies CA2 as a novel therapeutic target for chordoma. Besides, our findings suggest Dorzolamide HCl as a promising therapeutic option for chordoma.


Assuntos
Cordoma , Animais , Carcinogênese , Ciclo Celular , Proliferação de Células , Transformação Celular Neoplásica , Cordoma/tratamento farmacológico , Cordoma/genética , Humanos , Microambiente Tumoral
8.
J Radiol Case Rep ; 15(3): 1-8, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34267865

RESUMO

Mass lesions presenting at the craniocervical junction often present a unique challenge due to the complex anatomic arrangement limiting access for tissue diagnosis. The transoral approach has predominantly been used for percutaneous vertebroplasty of high cervical vertebrae with limited literature describing image guided biopsy for bony lesions in this region in the pediatric patient. We describe a technique of computed tomography guided transoral biopsy of a poorly differentiated chordoma located at the C1-C2 level in a 5-year-old child, and review this diagnosis.


Assuntos
Cordoma/patologia , Biópsia Guiada por Imagem/métodos , Tomografia Computadorizada por Raios X , Pré-Escolar , Feminino , Humanos
9.
Cochrane Database Syst Rev ; 7: CD013224, 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-34196007

RESUMO

BACKGROUND: Chordoma is a rare primary bone tumour with a high propensity for local recurrence. Surgical resection is the mainstay of treatment, but complete resection is often morbid due to tumour location. Similarly, the dose of radiotherapy (RT) that surrounding healthy organs can tolerate is frequently below that required to provide effective tumour control. Therefore, clinicians have investigated different radiation delivery techniques, often in combination with surgery, aimed to improve the therapeutic ratio. OBJECTIVES: To assess the effects and toxicity of proton and photon adjuvant radiotherapy (RT) in people with biopsy-confirmed chordoma. SEARCH METHODS: We searched CENTRAL (2021, Issue 4); MEDLINE Ovid (1946 to April 2021); Embase Ovid (1980 to April 2021) and online registers of clinical trials, and abstracts of scientific meetings up until April 2021. SELECTION CRITERIA: We included adults with pathologically confirmed primary chordoma, who were irradiated with curative intent, with protons or photons in the form of fractionated RT, SRS (stereotactic radiosurgery), SBRT (stereotactic body radiotherapy), or IMRT (intensity modulated radiation therapy). We limited analysis to studies that included outcomes of participants treated with both protons and photons. DATA COLLECTION AND ANALYSIS: The primary outcomes were local control, mortality, recurrence, and treatment-related toxicity. We followed current standard Cochrane methodological procedures for data extraction, management, and analysis. We used the ROBINS-I tool to assess risk of bias, and GRADE to assess the certainty of the evidence. MAIN RESULTS: We included six observational studies with 187 adult participants. We judged all studies to be at high risk of bias. Four studies were included in meta-analysis. We are uncertain if proton compared to photon therapy worsens or has no effect on local control (hazard ratio (HR) 5.34, 95% confidence interval (CI) 0.66 to 43.43; 2 observational studies, 39 participants; very low-certainty evidence). Median survival time ranged between 45.5 months and 66 months. We are uncertain if proton compared to photon therapy reduces or has no effect on mortality (HR 0.44, 95% CI 0.13 to 1.57; 4 observational studies, 65 participants; very low-certainty evidence). Median recurrence-free survival ranged between 3 and 10 years. We are uncertain whether proton compared to photon therapy reduces or has no effect on recurrence (HR 0.34, 95% CI 0.10 to 1.17; 4 observational studies, 94 participants; very low-certainty evidence). One study assessed treatment-related toxicity and reported that four participants on proton therapy developed radiation-induced necrosis in the temporal bone, radiation-induced damage to the brainstem, and chronic mastoiditis; one participant on photon therapy developed hearing loss, worsening of the seventh cranial nerve paresis, and ulcerative keratitis (risk ratio (RR) 1.28, 95% CI 0.17 to 9.86; 1 observational study, 33 participants; very low-certainty evidence). There is no evidence that protons led to reduced toxicity. There is very low-certainty evidence to show an advantage for proton therapy in comparison to photon therapy with respect to local control, mortality, recurrence, and treatment related toxicity. AUTHORS' CONCLUSIONS: There is a lack of published evidence to confirm a clinical difference in effect with either proton or photon therapy for the treatment of chordoma. As radiation techniques evolve, multi-institutional data should be collected prospectively and published, to help identify persons that would most benefit from the available radiation treatment techniques.


Assuntos
Neoplasias Ósseas/radioterapia , Cordoma/radioterapia , Fótons/uso terapêutico , Terapia com Prótons/métodos , Adulto , Viés , Neoplasias Ósseas/mortalidade , Cordoma/mortalidade , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/prevenção & controle , Estudos Observacionais como Assunto , Fótons/efeitos adversos , Intervalo Livre de Progressão , Terapia com Prótons/efeitos adversos , Radiocirurgia/métodos , Radioterapia Adjuvante , Radioterapia de Intensidade Modulada/métodos , Fatores de Tempo
10.
Front Immunol ; 12: 682262, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34305915

RESUMO

Immunotherapy begins to be widely used due to the increasing exploration and gratifying effects in multiple cancers. Chordoma, as a rare bone malignant tumor, often recurs and metastasizes after undergoing surgery and radiotherapy. Therefore, immunotherapy can be explored as an emerging, potentially effective treatment to improve the survival rate and clinical benefit of patients. However, a variety of immune-related adverse events (irAEs) cannot be avoided completely. And the immunotherapy-induced myocarditis, as a rare but fatal irAE, has been increasingly reported. Understanding the mechanism involved in irAEs can inform best practices for side effects management. Here, we firstly reported a case of immune myocarditis and subsequent myasthenia gravis (MG) following anti-PD-1 treatment for chordoma.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Cordoma/complicações , Miastenia Gravis/complicações , Miocardite/diagnóstico , Miocardite/etiologia , Idoso , Antineoplásicos Imunológicos/uso terapêutico , Biomarcadores , Cordoma/diagnóstico , Cordoma/tratamento farmacológico , Testes de Função Cardíaca , Humanos , Imunossupressores/uso terapêutico , Testes de Função Renal , Testes de Função Hepática , Imageamento por Ressonância Magnética , Masculino , Terapia de Alvo Molecular/efeitos adversos , Miocardite/tratamento farmacológico , Tomografia por Emissão de Pósitrons , Receptor de Morte Celular Programada 1/antagonistas & inibidores
11.
Clin Orthop Surg ; 13(2): 278-285, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34094020

RESUMO

Background: Bone tumors can cause severe pain and poor quality of life due to recurrence and non-achievement of complete remission after surgery, chemotherapy, or radiotherapy. Radiofrequency ablation (RFA) can be considered for minimally invasive treatment of bone tumors that are difficult to radically excise. In this study, RFA was performed for bone tumors that were difficult to radically excise and did not respond to surgery, chemotherapy, or radiotherapy due to their large sizes and/or locations. The purpose of this study was to retrospectively analyze the clinical characteristics and survival rates of bone tumors after RFA and provide one more treatment option for the future. Methods: There were 43 patients with bone tumors who underwent percutaneous RFA at our hospital from April 2007 to October 2017. The median age of the patients was 59 years (range, 31-75 years), and the median follow-up duration was 67.2 months (range, 10.2-130.5 months). Of the 43 patients, 26 were male and 17 were female. Thirty-four cases were metastatic bone tumors, 5 were chordomas, 3 were osteosarcomas, and 1 was a giant cell tumor. Pain and functional ability of the patients were evaluated using a visual analog scale (VAS) and the Musculoskeletal Tumor Society (MSTS) functional scoring system, respectively. Scores were recorded preoperatively, 1 week postoperatively, and 4 weeks postoperatively. The 1-year, 2-year, and 5-year survival rates were evaluated using the Kaplan-Meier method. Results: The mean VAS score was 8.21 preoperatively. The mean VAS score at 1 week, 4 weeks, 12 weeks, and 24 weeks postoperatively were 3.91, 3.67, 3.31, and 3.12, respectively. The mean preoperative MSTS score was 64.0% (range, 32%-87%). The mean postoperative MSTS score was 71.0% (range, 40%-90%). The 1-year, 2-year, and 5-year survival rates were 95.3%, 69.8%, and 30.2%, respectively. Conclusions: As per our study findings, RFA was effective in reducing pain and improving functional ability of patients with bone tumors that were difficult to radically excise.


Assuntos
Neoplasias Ósseas/terapia , Cordoma/terapia , Tumores de Células Gigantes/terapia , Osteossarcoma/terapia , Ablação por Radiofrequência/métodos , Adulto , Idoso , Neoplasias Ósseas/mortalidade , Cordoma/mortalidade , Feminino , Tumores de Células Gigantes/mortalidade , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Osteossarcoma/mortalidade , Medição da Dor , Estudos Retrospectivos , Inquéritos e Questionários
12.
Cancer Radiother ; 25(6-7): 545-549, 2021 Oct.
Artigo em Francês | MEDLINE | ID: mdl-34175224

RESUMO

Considering intracranial tumours, only few indications of protontherapy, such as chordoma, chondrosarcoma or uveal melanoma, are uniformly approved in the world. Other indications, excluding paediatric pathologies, are still debated. The aim of this article is to describe the rationale for the use of protonbeam irradiation for meningioma, pituitary adenoma, craniopharyngioma, paraganglioma, glioma, and schwannoma, and to inform the radiation oncologists if prospective studies or randomized studies are opened for inclusions. This article deals only with indications for adults.


Assuntos
Neoplasias Encefálicas/radioterapia , Neoplasias Meníngeas/radioterapia , Meningioma/radioterapia , Neoplasias Hipofisárias/radioterapia , Adenoma/radioterapia , Adulto , Cordoma/radioterapia , Craniofaringioma/radioterapia , Glioma/radioterapia , Humanos , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Neurilemoma/radioterapia , Paraganglioma/radioterapia , Estudos Prospectivos , Dosagem Radioterapêutica , Ensaios Clínicos Controlados Aleatórios como Assunto
13.
Sci Rep ; 11(1): 12466, 2021 06 14.
Artigo em Inglês | MEDLINE | ID: mdl-34127734

RESUMO

Chordomas are rare slow growing, malignant bone tumors of the axial skeleton with no approved medical treatment. As the majority of chordomas express cMET and its ligand, HGF, and crosstalks between EGFR and MET-signaling exist, we aimed to explore cMET activity in chordoma cell lines and clinical samples. We investigated nine chordoma patients and four chordoma cell lines for cMET expression. Two clival and two sacral chordoma cell lines were tested for chromosomal abnormalities of the MET gene locus; we studied the influence of HGF on the autocrine secretion and migration behavior, as well as protein expression and phosphorylation. Two MET/ALK inhibitors were investigated for their effects on cell viability, cell cycle, cyclin alterations, apoptosis, and downstream signaling pathways. Moderate and strong expression of membrane and cytoplasmic cMET in chordoma patients and cell lines used, as well as concentration-dependent increase in phospho cMET expression after HGF stimulation in all four chordoma cell lines was shown. U-CH2, MUG-Chor1, and UM-Chor1 are polysomic for MET. Chordoma cell lines secreted EGF, VEGF, IL-6, and MMP9 upon HGF-stimulation. Sacral cell lines showed a distinct HGF-induced migration. Both inhibitors dose-dependently inhibited cell growth, induce apoptosis and cell-cycle arrest, and suppress downstream pathways. Heterogeneous responses obtained in our in vitro setting indicate that cMET inhibitors alone or in combination with other drugs might particularly benefit patients with sacral chordomas.


Assuntos
Cordoma/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Proteínas Proto-Oncogênicas c-met/antagonistas & inibidores , Sacro/patologia , Neoplasias da Base do Crânio/tratamento farmacológico , Quinase do Linfoma Anaplásico/antagonistas & inibidores , Quinase do Linfoma Anaplásico/metabolismo , Anilidas/farmacologia , Anilidas/uso terapêutico , Apoptose/efeitos dos fármacos , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Sobrevivência Celular/genética , Cordoma/genética , Cordoma/patologia , Fossa Craniana Posterior , Crizotinibe/farmacologia , Crizotinibe/uso terapêutico , Variações do Número de Cópias de DNA , Regulação Neoplásica da Expressão Gênica , Fator de Crescimento de Hepatócito/genética , Fator de Crescimento de Hepatócito/metabolismo , Humanos , Inibidores de Proteínas Quinases/farmacologia , Proteínas Proto-Oncogênicas c-met/genética , Proteínas Proto-Oncogênicas c-met/metabolismo , Piridinas/farmacologia , Piridinas/uso terapêutico , Transdução de Sinais/efeitos dos fármacos , Neoplasias da Base do Crânio/patologia
14.
Photodiagnosis Photodyn Ther ; 35: 102346, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34038764

RESUMO

BACKGROUND: Chordoma are uncommon aggressive tumors of the skeleton. Surgical resection is often subtotal and adjuvant treatment possibilities are limited as chordomas are highly chemo- and radioresistant. In the present study we examined the impact of 5-ALA PDT on different human chordoma cell lines. Furthermore, we investigated the variation of two parameters: (1.) 5-ALA incubation time and (2.) supplemental use of ciprofloxacin as iron chelator. METHODS: Experiments were realized in vitro with three different human chordoma cell lines: U-CH2, U-CH2B and U-CH14. After pre-incubation for 24 h with various concentrations of ciprofloxacin (1.5 - 5.0 µg/ml), different amounts of 5-ALA (15 - 50 µg/ml) were applied to the cells either for a brief (4 h) or a long (6 h) incubation time. Subsequently cells were exposed to photodynamic radiation. Cell viability was exploited by WST-1 assay. Thus, for each of the three cell lines, two drug combinations (ciprofloxacin plus 5-ALA and 5-ALA only) and two incubation times (short, 4 h and long, 6 h) were tested. Negative control groups were also examined. RESULTS: Supplemental use of ciprofloxacin led to increased cell death in each of the cell lines. Different 5-ALA incubation times (4 h vs. 6 h) showed no significant differences in cell viability except for U-CH2. CONCLUSION: Ciprofloxacin as an ordinary applied antibiotic, enhanced the effect of 5-ALA PDT on different human chordoma cell lines in vitro. The impact was dependent on the dose of ciprofloxacin-5-ALA. There were no notable differences for the tested 5-ALA incubation times. In human chordoma cell lines 5-ALA PDT may effectively be amended by ciprofloxacin.


Assuntos
Cordoma , Fotoquimioterapia , Ácido Aminolevulínico/farmacologia , Linhagem Celular Tumoral , Sobrevivência Celular , Cordoma/tratamento farmacológico , Ciprofloxacina/farmacologia , Humanos , Fotoquimioterapia/métodos , Fármacos Fotossensibilizantes/farmacologia
15.
Neurosurg Focus ; 50(5): E17, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33932924

RESUMO

OBJECTIVE: Long-term local control in patients with primary chordoma and sarcoma of the spine and sacrum is increasingly reliant upon en bloc resection with negative margins. At many institutions, adjuvant radiation is recommended; definitive radiation is also recommended for the treatment of unresectable tumors. Because of the high off-target radiation toxicities associated with conventional radiotherapy, there has been growing interest in the use of proton and heavy-ion therapies. The aim of this study was to systematically review the literature regarding these therapies. METHODS: The PubMed, OVID, Embase, and Web of Science databases were queried for articles describing the use of proton, combined proton/photon, or heavy-ion therapies for adjuvant or definitive radiotherapy in patients with primary sarcoma or chordoma of the mobile spine and sacrum. A qualitative synthesis of the results was performed, focusing on overall survival (OS), progression-free survival (PFS), disease-free survival (DFS), and disease-specific survival (DSS); local control; and postradiation toxicities. RESULTS: Of 595 unique articles, 64 underwent full-text screening and 38 were included in the final synthesis. All studies were level III or IV evidence with a high risk of bias; there was also significant overlap in the reported populations, with six centers accounting for roughly three-fourths of all reports. Five-year therapy outcomes were as follows: proton-only therapies, OS 67%-82%, PFS 31%-57%, and DFS 52%-62%; metastases occurred in 17%-18% and acute toxicities in 3%-100% of cases; combined proton/photon therapy, local control 62%-85%, OS 78%-87%, PFS 90%, and DFS 61%-72%; metastases occurred in 12%-14% and acute toxicities in 84%-100% of cases; and carbon ion therapy, local control 53%-100%, OS 52%-86%, PFS (only reported for 3 years) 48%-76%, and DFS 50%-53%; metastases occurred in 2%-39% and acute toxicities in 26%-48%. There were no studies directly comparing outcomes between photon and charged-particle therapies or comparing outcomes between radiation and surgical groups. CONCLUSIONS: The current evidence for charged-particle therapies in the management of sarcomas of the spine and sacrum is limited. Preliminary evidence suggests that with these therapies local control and OS at 5 years are comparable among various charged-particle options and may be similar between those treated with definitive charged-particle therapy and historical surgical cohorts. Further research directly comparing charged-particle and photon-based therapies is necessary.


Assuntos
Cordoma , Sarcoma , Neoplasias da Coluna Vertebral , Cordoma/radioterapia , Cordoma/cirurgia , Humanos , Estudos Retrospectivos , Sacro/cirurgia , Sarcoma/radioterapia , Sarcoma/cirurgia , Neoplasias da Coluna Vertebral/radioterapia , Neoplasias da Coluna Vertebral/cirurgia , Resultado do Tratamento
16.
Oper Neurosurg (Hagerstown) ; 21(3): 150-159, 2021 08 16.
Artigo em Inglês | MEDLINE | ID: mdl-34038940

RESUMO

BACKGROUND: Extended endoscopic endonasal approaches (EEAs) have progressively widened the armamentarium of skull base surgeons. In order to reduce approach-related morbidity of EEAs and closure techniques, the development of alternative strategies that minimize the resection of normal tissue and alleviate the use of naso-septal flap (NSF) is needed. We report on a novel targeted approach to the clivus, with incision and closure of the mucosa of the rostrum, as the initial and final step of the approach. OBJECTIVE: To present an alternative minimally invasive approach and reconstruction technique for selected clival chordomas. METHODS: Three cases of clival chordomas illustrating this technique are provided, together with an operative video. RESULTS: The mucosa of the rostrum is incised and elevated from the underlying bone, as first step of surgery. Following tumor resection with angled scope and instruments, the mucosa of the sphenoid sinus (SS) is removed and the tumor cavity and SS are filled with abdominal fat. The mucosal incision of the rostrum is then sutured. A hangman knot is prepared outside the nasal cavity and tightened after the first stitch and a running suture is performed. CONCLUSION: We propose, in this preliminary report, a new targeted approach and reconstruction strategy, applying to EEAs the classic concept of skin incision and closure for transcranial approaches. With further development in the instrumentations and visualization tools, this technique may become a valuable minimally invasive endonasal approach for selected lesions.


Assuntos
Cordoma , Neoplasias da Base do Crânio , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Fossa Craniana Posterior/cirurgia , Humanos , Membrana Mucosa , Base do Crânio/cirurgia , Neoplasias da Base do Crânio/diagnóstico por imagem , Neoplasias da Base do Crânio/cirurgia
17.
Carcinogenesis ; 42(7): 913-923, 2021 07 16.
Artigo em Inglês | MEDLINE | ID: mdl-34057989

RESUMO

Chordoma is a rare bone tumor arising from notochordal remnants, but the underlying mechanism remains elusive. By integrated mRNA and microRNA analyses, we found significant downregulation of TGFB3 along with upregulation of its inhibitor, miR-29 family in chordoma comparing with notochord. Somatic copy number gains of miR-29 loci in chordoma highlighted a mechanism of inactivation of TGFB3 signaling in tumor formation. In zebrafish, knockout and knockdown homologous tgfb3 resulted in a chordoma-like neoplasm. On the other hand, Smad7 negative feedback regulation of transforming growth factor-ß (TGF-ß) signaling is retentive in chordoma cell UM-Chor1 despite its disruption in most cancer cells (e.g. A549). Therefore, contrary to other cancers, exogenous TGF-ß activated Smad7 by downregulating miR-182 and inhibited cell migration and invasion in UM-Chor1. Meanwhile, TGF-ß decreased chordoma characteristic protein Brachyury. Altogether, downregulation of TGFB3 causes chordomagenesis, showing a feasible target for therapies. The retention of Smad7 negative regulation may maintain the suppressor role of TGF-ß in chordoma.


Assuntos
Biomarcadores Tumorais/metabolismo , Cordoma/patologia , Transição Epitelial-Mesenquimal , Regulação Neoplásica da Expressão Gênica , Proteína Smad7/metabolismo , Fator de Crescimento Transformador beta3/antagonistas & inibidores , Apoptose , Biomarcadores Tumorais/genética , Proliferação de Células , Cordoma/genética , Cordoma/metabolismo , Humanos , Prognóstico , Proteína Smad7/genética , Fator de Crescimento Transformador beta3/genética , Fator de Crescimento Transformador beta3/metabolismo , Células Tumorais Cultivadas
18.
BMC Neurol ; 21(1): 207, 2021 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-34022822

RESUMO

BACKGROUND: Treating chordoma through surgery alone is often ineffective. Thus, surgery often performed with irradiation, with a reported 5-year survival rate of 60-75%. The clinical course varies, and disease rarity prevents larger number of clinical investigations. METHODS: In total, 19 patients with clival chordomas were retrospectively extracted from our institutional database. They were initially treated with maximal tumor removal using the extended transsphenoidal approach between March 2006 and January 2021. When total tumor removal was achieved, prophylactic irradiation was not performed. If tumor remnants or recurrence were confirmed, Gamma Knife (GK) radiosurgery was performed. The mean follow-up period was 106.7 months (ranged 27-224 months). The clinical course and prognostic factors were investigated. RESULTS: Total removal was achieved in 10 patients, whereas 4 patients suffered recurrence and required GK. GK was applied to 11 patients with a 50% isodose of 13-18 Gy (mean: 15.4 Gy), and eight patients remained progression free, whereas three patients suffered repeated local recurrence and died of tumor-related complications. The mean overall progression-free interval was 57.2 months (range: 6-169 months). One male patient died of tumor un-related lung cancer 36 months after the initial treatment, and other patients survived throughout the observational periods. The mean overall survival was 106.7 months (range: 27-224 months). Thus, the 5-year survival rate was 94.7%. Statistical analysis indicated that sex (men), > 15 Gy of 50% isodose by GK, and screening brain examinations as prophylactic medicine were significant favorable prognostic factors. CONCLUSIONS: The favorable outcomes in this investigation suggest the importance of early detection and treatment. Surgery may enable better conditions for sufficient GK doses.


Assuntos
Cordoma/cirurgia , Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias da Base do Crânio/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Prognóstico , Radiocirurgia/métodos , Estudos Retrospectivos , Taxa de Sobrevida
19.
Acta Neurochir (Wien) ; 163(8): 2141-2154, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33847826

RESUMO

BACKGROUND: Surgical access to the ventral pontomedullary junction (PMJ) can be achieved through various corridors depending on the location and extension of the lesion. The jugular tubercle (JT), a surgically challenging obstacle to access the PMJ, typically needs to be addressed in transcranial exposures. We describe the endoscopic endonasal transclival approach (EETCA) and its inferolateral transtubercular extension to assess the intradural surgical field gained through JT removal. We also complement the dissections with an illustrative case. METHODS: EETCA was surgically simulated, and the anatomical landmarks were assessed in eight cadaveric heads. Microsurgical dissections were additionally performed along the endoscopic surgical path. Lastly, we present an intraoperative video of the trans-JT approach in a patient with lower clival chordoma. RESULTS: The EETCA allowed adequate extracranial visualization and removal of the JT. The surgical bony window-obtained along the clivus and centered at the JT via the EETCA-measured 11 × 9 × 7 mm. Removal of the JT provided an improved intradural field within the lower third of the cerebellopontine cistern to expose an area bordered by the cranial nerves VII/VIII and flocculus superior and anterior margin of the lateral recess of the fourth ventricle and cranial nerves IX-XI inferiorly, centered on the foramen of Luschka. CONCLUSIONS: Removal of the JT via EETCA improves exposure along the lower third of the cerebellopontine and upper cerebellomedullary cisterns. The inferolateral transtubercular extension of the EETCA provides access to the lateral recess of the fourth ventricle, in combination with the ventral midline pontomedullary region.


Assuntos
Procedimentos Neurocirúrgicos , Cadáver , Cordoma , Fossa Craniana Posterior/cirurgia , Humanos , Nariz , Neoplasias da Base do Crânio/cirurgia
20.
Indian J Pathol Microbiol ; 64(2): 410-412, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33851648

RESUMO

We present a case of a 48-year-old man's unexpected death affected by a relapsed clivalchordoma. After partial excision surgery of the neoplasm, he manifested 5 days later, in conditions of well-being, a sudden lethal extracranial hemorrhage from nose and mouth. The autopsy examination and the subsequent histological investigations did not allow us to clarify the exact origin of the bleeding. Based on the negativity of the accurate examinations performed, the extent of the bleeding, and the findings highlighted by the means of the nuclear magnetic resonance (NMR) carried out a few days before death, we have considered reasonable to localize the source of hemorrhage in the intrapetrous tract of the left internal carotid artery. Since this is a unique event, never previously documented, we believe that our report may be of interest to the scientific community.


Assuntos
Cordoma/cirurgia , Morte Súbita , Hemorragia/mortalidade , Choque Hemorrágico/mortalidade , Autopsia , Artéria Carótida Interna/patologia , Cordoma/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Notocorda/patologia , Base do Crânio/patologia , Base do Crânio/cirurgia
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