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1.
No Shinkei Geka ; 47(8): 901-907, 2019 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-31477634

RESUMO

OBJECTIVES: Intracranial chordomas are thought to arise from remnants of the notochord and usually occur at the parasellar region. We present a case of a primary intrasellar chondroid chordoma in a patient who was initially diagnosed with a pituitary adenoma. CASE: A 77-year-old woman had a history of two surgeries for a tumor in the sella turcica(17 months after the 1st surgery). On initial MRI, the intrasellar mass showed low signal intensity on T1WI, very high signal intensity on T2WI, and inhomogeneous enhancement. On bone reconstructive CT, the sellar floor was thin, and no abnormalities were observed at the top of the clivus. Transsphenoidal surgery was performed. The pathological diagnosis was pituitary adenoma in both cases. Seventy-two months after the 1st(31 months after the 2nd)surgery, she developed right-sided oculomotor and abducent nerve palsies again. Since recurrence occurred during the relatively short period, the surgical specimens obtained from the 1st and 2nd surgeries were reexamined. Reexamination of the previously obtained specimen demonstrated areas of chondroid tissue that were embedded in a mucoid stroma and tumor cells that were composed of round or pleomorphic nuclei with vacuolated cytoplasm(physaliphorus cells)that were compatible with chondroid chordoma. The third surgery was performed. Postoperatively, her symptoms improved, and cyber knife therapy was administered for the residual part of the tumor. CONCLUSIONS: Although intrasellar chondroid chordomas are extremely rare, they should be considered in the differential diagnosis of tumors located in the sella turcica.


Assuntos
Adenoma , Cordoma , Neoplasias Hipofisárias , Adenoma/diagnóstico , Adenoma/cirurgia , Idoso , Cordoma/diagnóstico , Cordoma/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Recidiva Local de Neoplasia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/cirurgia , Sela Túrcica/patologia
2.
J Craniofac Surg ; 30(8): e755-e757, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31449204

RESUMO

A 78-year-old woman with progressive worsening vertigo and new onset of diplopia. Neurological examination revealed bilateral abducens nerve paralysis and gait disturbance with truncal ataxia. Neuroimaging revealed a mass lesion within the clivus with brain stem compression. The patient was operated with provisional diagnosis of clivus chordoma by neuro-navigation-guided extended endoscopic endonasal approach. Subtotal resection of the tumor was achieved with no intra- or postoperative complications. Histopathologic examination revealed intraosseous meningioma (WHO grade I). To the authors' best knowledge this is the first case reporting clivus site of intraosseous meningioma.


Assuntos
Cordoma/cirurgia , Fossa Craniana Posterior/diagnóstico por imagem , Diagnóstico Diferencial , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Neoplasias da Base do Crânio/cirurgia , Idoso , Cordoma/complicações , Cordoma/diagnóstico , Fossa Craniana Posterior/patologia , Diplopia/complicações , Feminino , Neoplasias de Cabeça e Pescoço , Humanos , Neuroimagem , Complicações Pós-Operatórias , Neoplasias da Base do Crânio/complicações , Neoplasias da Base do Crânio/diagnóstico
3.
Medicine (Baltimore) ; 98(35): e16990, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31464948

RESUMO

RATIONALE: Giant thoracic chordoma is a highly unusual disease with no standard curative managements yet. The objective of this study is to report a very rare case of giant thoracic chordoma successfully operated by combination of thoracoscopic surgery together with posterior spinal surgery. The management of these unique cases has yet to be well-documented. PATIENT CONCERNS: A 64-year-old man presented with a 4-month history of continuous and progressive back pain. The patient, who had been diagnosed of sacral chordoma for 2 years, received surgical treatment of posterior sacral tumor resection and instrumentation. A lytic, expanding lesion of the T5 and T6 vertebral and paraspinal region with mild epidural spinal cord compression was identified. DIAGNOSIS: MRI of spine and PET/CT showed spinal cord compression secondary to the epidural component of the T5 and T6 mass, with increased metastatic marrow infiltration of the left T5 and T6 vertebral and paravertebral region, which presented as a solid tumor. Postoperative pathology confirmed the diagnosis of thoracic chordoma. INTERVENTIONS: The patient underwent 1-stage thoracoscopic release of vertebral and paravertebral tumors, posterior resection of T5-T6 vertebral and paravertebral tumors, T4-T7 spinal canal decompression, and T2-T9 pedicle screw fixation procedure via a posterior approach. OUTCOMES: The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 3-month and 6-month follow-up visit. There were no other complications associated with the operation during the follow-up period. LESSONS: Taken together, the lesion's clinical features, imaging results, and pathological characteristics are unique. Combined efforts of specialists from orthopedics, thoracic surgery, neurosurgery, and medical oncology led to the successful diagnosis and management of this patient. Giant thoracic chordoma, although rare, should be part of the differential diagnosis when the patient has a history of sacral chordoma and presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the giant thoracic chordoma when the tumor has caused neurological deficits. One-stage thoracoscopic release or resection of vertebral and paravertebral tumor is also a good choice for surgical treatment.


Assuntos
Cordoma/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Procedimentos Cirúrgicos Operatórios/métodos , Vértebras Torácicas/cirurgia , Cordoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Procedimentos Ortopédicos/métodos , Neoplasias da Coluna Vertebral/patologia , Toracoscopia/métodos
4.
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
5.
Am J Case Rep ; 20: 773-775, 2019 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-31152144

RESUMO

BACKGROUND Chordoma is a primary bone tumor that most commonly arises in the sacrococcygeal vertebrae and the spheno-occipital areas. Chordoma is a malignant tumor that should be distinguished from benign notochordal cell tumor (BNCT) of the spine. This report is of a rare case of axial chordoma and describes the challenging approach to diagnosis and management. CASE REPORT A 50-year-old woman presented with a one-year history of a slowly growing swelling in the sacral region. She complained of numbness and progressive weakness in both lower limbs, and urinary incontinence. Computed tomography (CT) imaging showed a large destructive lesion involving the sacrum and coccyx with cranial extension to level S2 and invasion of the right and left S2-S3 neural foramina, sacral nerves, left gluteus maximums muscle, and adjacent subcutaneous tissue. The management approach included the early involvement of a multidisciplinary clinical team. En bloc resection of the tumor through an anterior and posterior approach was performed, and the defect was reconstructed later using bilateral rotational gluteal fascial flaps. CONCLUSIONS Axial chordoma is a very rare, locally aggressive, and highly recurrent primary tumor of bone. The clinical management is challenging and requires early involvement of a multidisciplinary team. Following surgical resection, careful selection from limited available reconstructive surgical options is necessary to ensure that the surgical defect is repaired.


Assuntos
Cordoma/diagnóstico , Cóccix , Sacro , Neoplasias da Coluna Vertebral/diagnóstico , Cordoma/complicações , Cordoma/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias da Coluna Vertebral/complicações , Neoplasias da Coluna Vertebral/cirurgia , Tomografia Computadorizada por Raios X
6.
Cir. plást. ibero-latinoam ; 45(2): 169-173, abr.-jun. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-184224

RESUMO

Los cordomas son tumores malignos de crecimiento lento derivados de la notocorda. Habitualmente se localizan en el clivus y se presentan en mujeres en la tercera o cuarta décadas de la vida. La resección quirúrgica es su tratamiento de elección, pudiendo asociarse en casos selectos a radioterapia. En cordomas pequeños el tratamiento de elección es el abordaje endoscópico transnasal, transesfenoidal y para las lesiones extensas se prefiere el abordaje máxilo-mandibular con glosotomía. Para la reconstrucción, al igual que en otras áreas anatómicas, las ventajas de los tejidos óseos vascularizados sobre los no vascularizados son: la rápida consolidación, la resistencia a la infección, la reacción hipertrófica originada por la carga mecánica y la tolerancia a niveles terapéuticos de radiación. Presentamos el caso de una mujer de 24 años de edad con diagnóstico de cordoma en C2-C3, a quien se le realizó corpectomía C2-C3 y exéresis marginal de la lesión, seguida de reconstrucción con colgajo libre de peroné


Chordoma neoplasms are malignant tumors with a generally slow growth. They are usually located in the clivus. The most common presentation is in young women (third and fourth decades of life). Surgical resection is the main line of treatment, occasionally radiotherapy may be needed. As for small chordomas, transnasal endoscopical resection can be performed, however, for larger tumors a maxillo-mandibular surgical approach may be needed. For reconstruction purposes, the main advantages of using vascularized tissues are: rapid consoloditation, higher infection resistance and a hypertrophic reaction secondary to the mechanical stress. We present the case of a 24 years old female patient with a C2-C3 chordom; corpectomy and marginal resection of the tumor were performed, as well as reconstruction with a free fibula flap


Assuntos
Humanos , Feminino , Adulto , Cordoma/cirurgia , Cordoma/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Microcirurgia/instrumentação , Retalhos de Tecido Biológico , Faringe/patologia , Faringe/cirurgia , Fíbula/cirurgia
7.
Medicine (Baltimore) ; 98(21): e15713, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31124949

RESUMO

RATIONALE: Chordomas are rare malignant neoplasms arised from residual embryonic notochordal tissue, mostly located in the axial midline. Tumors along extra-axial locations in the head and neck are rare. Chordomas located in the jugular foramen are extremely rare, with a low incidence of 0.2%. PATIENT CONCERNS: A 64-year-old male with 20 years of dizziness history complaining of 6 months of severe dizziness: significant with the changing of the body posture, vertigo which can be self-remissioned within 1 minute and hearing loss of both ears, without headache, nausea, dysphagia, or otalgia. Computed tomography and magnetic resonance imaging (MRI) were performed before surgery which suggests various possibilities. Immunohistochemistry helped to confirm the final diagnosis. DIAGNOSES: Immunohistochemistry demonstrated diffuse positivity for S100 (+++), positivity for D2-40 (focal +), EMA (+), and PR (+). Ki-67 labeling index was estimated at 2% focally. The final diagnosis was chordoma. INTERVENTIONS: The tumor was excised via retro-sigmoid approach without postoperative radiotherapy. OUTCOMES: Facial paralysis occurred in this case. House-Brackmann facial nerve grading system was used to evaluate the facial paralysis of this patient. It is considered as H-B grade IV. The patient was followed up regularly every month after operation, totally for 9 months. An MRI of the brain was performed 6 months after surgery which shows a small range of abnormal signals similar to the previous MRI in the jugular foramen, suggesting that there may be residual or recurrent tumor. And facial paralysis stays at H-B grade IV without any recovery. LESSONS: It is a big challenge for us to remove giant tumors located in the jugular foramen because of its unique anatomy. Access should be combined with retro-sigmoid or infra-temporal fossa approach to remove such tumors. Chordomas is a malignant neoplasm which may need radiotherapy after surgery, particularly those with subtotal and partial resection.


Assuntos
Cordoma/cirurgia , Paralisia Facial/patologia , Complicações Pós-Operatórias/patologia , Neoplasias da Base do Crânio/cirurgia , Cordoma/diagnóstico , Nervo Facial/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Neoplasias da Base do Crânio/diagnóstico , Tomografia Computadorizada por Raios X
8.
G Chir ; 40(2): 132-136, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31131813

RESUMO

INTRODUCTION: Brain notochordal cell tumors (BTCN) are lesions arising from notochordal differentiation which affect the axial skeleton. PRESENTATION OF CASE: We report a case of a patient treated in our General Surgery Unit of the University Hospital of Bari, Italy, with occasional finding of sacral chordoma at the histological examination. DISCUSSION: Because of their location, sacral chordomas can affect bowel and bladder with organ specific symptoms. Radiotherapy may be used as a palliative treatment or for recurrence in those patients who cannot be submitted to surgery. CONCLUSIONS: Due to the high local recurrence rate radiation therapy should be considered mandatory after any type of chordoma resection. Multidisciplinary management of the disease is mandatory and improve patient outcomes. Patients should have maximal tumor debulking with adjuvant radiotherapy when possible.


Assuntos
Cordoma/cirurgia , Sacro , Neoplasias da Coluna Vertebral/cirurgia , Idoso , Cordoma/diagnóstico por imagem , Cordoma/patologia , Humanos , Achados Incidentais , Masculino , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/patologia
9.
World Neurosurg ; 129: e199-e206, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31125781

RESUMO

OBJECTIVE: We aimed to characterize the expression of transforming growth factor-α (TGF-α) and Ki-67 and to assess the relationship between TGF-α and Ki-67 expression and prognostic factors in skull base chordoma. METHODS: We retrospectively analyzed the data from 46 patients with skull base chordoma. The follow-up duration ranged from 1 to 168 months (mean, 74.1). The survival data were statistically analyzed using the Kaplan-Meier method and multivariate Cox regression analysis. The expression of TGF-α and Ki-67 were detected by immunohistochemical staining of paraffin-embedded patient tissue specimens. RESULTS: The total resection (TR) group had longer overall survival compared with the non-TR group (P = 0.042). The TR group also had longer progression-free survival (PFS) than did the non-TR group (P = 0.046). The group with a high Ki-67 labeling index (Ki-67LI) had shorter overall survival than did the group with a low Ki-67LI (P = 0.039). Also, the group with a high Ki-67LI had significantly shorter PFS than did the group with a low Ki-67LI (P = 0.016). Moreover, the group with high TGF-α expression had significantly shorter PFS compared with the group with low TGF-α expression (P = 0.005). CONCLUSIONS: Our results have shown that high levels of TGF-α and Ki-67 are associated with shorter PFS in patients with chordoma. We have confirmed the role of Ki-67 as a functional molecular marker of poor prognosis. We also identified TGF-α as a potential novel biomarker for predicting prognosis for patients with skull base chordoma.


Assuntos
Cordoma/mortalidade , Antígeno Ki-67/metabolismo , Neoplasias da Base do Crânio/mortalidade , Fator de Crescimento Transformador alfa/metabolismo , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/metabolismo , Cordoma/metabolismo , Cordoma/patologia , Cordoma/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Intervalo Livre de Progressão , Estudos Retrospectivos , Neoplasias da Base do Crânio/metabolismo , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/cirurgia , Taxa de Sobrevida , Adulto Jovem
10.
Prog Neurol Surg ; 34: 207-214, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31096236

RESUMO

Chordomas and chondrosarcomas are rare locally aggressive skull base tumors with high progression or recurrence rates. Ultimately, they have high mortality rates unless they respond to multimodality management options that include one or more surgical resections, fractionated radiation therapy, and stereotactic radiosurgery (SRS). SRS has become a standard management option for recurrent or residual chordomas and chondrosarcomas after failed surgical resection and fractionated radiation therapy. This report examines the role of SRS in these skull base tumors.


Assuntos
Condrossarcoma/radioterapia , Cordoma/radioterapia , Recidiva Local de Neoplasia/radioterapia , Neoplasia Residual/radioterapia , Radiocirurgia/métodos , Condrossarcoma/cirurgia , Cordoma/cirurgia , Humanos , Recidiva Local de Neoplasia/cirurgia , Neoplasia Residual/cirurgia , Neoplasias da Base do Crânio
11.
Indian J Pathol Microbiol ; 62(2): 287-289, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30971557

RESUMO

A unique case of eyelid metastasis from nasopharyngeal chondroid chordoma in a 63-year-old woman was reported. Chordomas are rare tumors of the bone deriving from remnants of the embryonic notochord. Histologically, the tumor showed lobulated structure and concludes two types of cells: liquid drop cell and small round/cubic cell. Immunohistochemically, AE1/AE3, epithelial membrane antigene (EMA), and S100 showed a uniform and strong positivity. It has a great capacity for recurrence and malignant transformation, despite their slow-growing nature. The most common sites of metastases are liver, lungs, and bones. The eyelid metastasis from chordoma is an extremely rare finding, which may suggest a poor prognosis for the patient. Its significant clinicopathological characteristic could prompt us to take it into consideration when assessing the patient's prognosis.


Assuntos
Cordoma/patologia , Neoplasias Palpebrais/secundário , Neoplasias Nasofaríngeas/patologia , Metástase Neoplásica/diagnóstico , Cordoma/cirurgia , Pálpebras/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Nasofaríngeas/cirurgia , Prognóstico
12.
World Neurosurg ; 128: e185-e194, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31003024

RESUMO

BACKGROUND: Skull base chordoma can be a challenging surgical entity because of its invasive nature. OBJECTIVE: In this study, the role of intraoperative magnetic resonance imaging (iMRI) to optimize the resection of skull base chordomas is evaluated. METHODS: We performed a retrospective analysis of operated patients with skull base chordomas in the setting of iMRI. The clinical records, operative notes, radiologic images, tumor volumetry, location of the residual tumor, and surgical outcome were evaluated. RESULTS: Fifteen patients were operated on for resection of skull base chordomas between 2010 and 2017 in our institution. Gross total resection was planned and achieved in 8 patients and partial resection in 7 patients. In 8 patients (53.3%), the preoperatively planned volume of resection was achieved and confirmed in the first iMRI control. In 7 patients, repeated iMRI controls were required to achieve the surgical target. In 3 patients, the tumor residual requiring further resection was located in the clivus and in 4 patients in the intradural space. The improvement of the preoperative deficits showed a significant statistical association with the resection of the intradural component and decompression of the brainstem. CONCLUSIONS: This study shows that iMRI is a safe method for intraoperative assessment of the degree of resection and the volume and location of residual tumor when resecting skull base chordomas. When gross total resection of the tumor is not feasible, iMRI can be a useful tool for targeted tumor resection.


Assuntos
Cordoma/cirurgia , Cuidados Intraoperatórios/métodos , Imagem por Ressonância Magnética/métodos , Neoplasias da Base do Crânio/cirurgia , Adolescente , Adulto , Cordoma/diagnóstico por imagem , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/cirurgia , Feminino , Humanos , Avaliação de Estado de Karnofsky , Masculino , Pessoa de Meia-Idade , Neoplasia Residual , Estudos Retrospectivos , Neoplasias da Base do Crânio/diagnóstico por imagem , Adulto Jovem
13.
J Clin Neurosci ; 64: 44-46, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30902435

RESUMO

INTRODUCTION: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement. CASE DESCRIPTION: We present a 33-year-old woman previously submitted to five brain surgeries to treat a posterior fossa intradural chordoma since December 2012. Currently, she presented almost with right and left hemiplegia (grade 2 bilaterally) and also left oculomotor, adbucent and facial nerve paresis. We performed neuroaxis magnetic resonance MR which disclosed stability of posterior fossa tumor which was previously irradiated. However, there were new intradural lesions at the level of C3, T11/T12 and L4/L5/S1 vertebrae. DISCUSSION: With the advent of contemponaeous surgery, radiotherapy options and even available chemotherapy to treat Chordomas (Imatinib), patients may experience enlarged survival and thus face complications such as drop metastases along neuroaxis. Our case illustrates a late (6 years) follow-up presentation of an initial posterior fossa intradural chordoma. It suggests that whole neuraxis involvement may be the final presentation of all patients harbouring chordomas and surviving after adequate initial treatment.


Assuntos
Cordoma/patologia , Neoplasias Infratentoriais/secundário , Neoplasias da Medula Espinal/secundário , Adulto , Cordoma/cirurgia , Feminino , Humanos , Neoplasias Infratentoriais/cirurgia , Imagem por Ressonância Magnética
15.
World Neurosurg ; 125: e1125-e1131, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30790740

RESUMO

INTRODUCTION: The primary treatment for patients with sacral chordoma is en bloc surgical resection with negative margins, which has been shown to reduce local recurrence and tumor-related morbidity. Here we describe the use of intraoperative neuronavigation using preoperative spine magnetic resonance imaging fused to intraoperative computed tomography (CT) to create 3-dimensional tumor reconstructions in the operating room for intraoperative identification of bone and soft-tissue margins for maximal safe tumor resection. METHODS: A single-institution retrospective chart review was completed to encompass our experience of 6 consecutive patients who had sacral chordoma resections using our described navigation protocol. We collected data on patient demographics, previous surgeries, radiation therapy, preoperative examination, spinal levels involved, dural involvement, estimated blood loss, surgery time, tissue diagnosis, follow-up, postoperative examination, complications, and recurrence. Primary outcome was en bloc resection with negative margins as planned preoperatively. RESULTS: Negative surgical margins were achieved in 5 of 5 patients, who were preoperatively planned for en bloc resection with negative margins. The most common levels involved were S4-S5. All patients had a stable or improved neurologic examination after en bloc surgical resection. The average follow-up was 5.4 months ± 84.6 days. No patient had residual or recurrent tumor at last follow-up. CONCLUSIONS: Magnetic resonance imaging-CT fusion and 3-dimensional reconstruction techniques using an intraoperative CT scanner with image-guided navigation to aid preoperative planning and surgical resection of sacral chordomas are not well represented in the literature. This technique can be used for planning en bloc surgical resections and for more precisely identifying tumor margins intraoperatively.


Assuntos
Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Imagem por Ressonância Magnética , Neuronavegação , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Cirurgia Assistida por Computador/métodos , Tomografia Computadorizada por Raios X , Idoso , Feminino , Humanos , Imagem Tridimensional/métodos , Período Intraoperatório , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Cuidados Pré-Operatórios , Estudos Retrospectivos , Sacro/patologia , Sacro/cirurgia , Resultado do Tratamento
16.
Br J Neurosurg ; 33(4): 388-393, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30741028

RESUMO

Purpose: Clival Chordomas are locally aggressive tumours which pose a significant treatment challenge. Endoscopic endonasal approach for clival chordomas is correlated with higher resection rates and lower morbidity rates in comparison to open approaches. We present our initial single institution experience and short-term patient outcomes following endoscopic endonasal approach for resection of clival chordomas. Materials and methods: This is a retrospective analysis of ten patients undergoing endoscopic endonasal approach for clival chordomas in our neurosurgical unit over a 6 year period between August 2010 and September 2016. The procedures were performed using two surgeons, four hands, binostril endoscopic endonasal approach with a Karl Storz® endoscope and intraoperative BrainLab® image guidance. Results: Overall 15 endoscopic endonasal approach resections of clival chordoma were performed in 10 patients with median follow up period of 39.5 months (range 9-76). Gross total resection was achieved in 4 cases (40%), near total resection in 4 cases (40%) and subtotal resection in 2 cases (20%). 5 cases (50%) required revision resections. Cerebrospinal fluid leak occurred in 2 patients. 1 case of meningitis occurred in a patient with revision surgery. There were no new neurological deficits post operatively with 3 patients demonstrating resolution of diplopia post operatively. No recurrence occurred following gross total resection. 1 out of 4 cases of near total resection showed evidence of progression during the follow up period. Both cases of subtotal resection demonstrated evidence of progression with one dying of unrelated cause during the follow up period. Conclusion: Endoscopic endonasal approach represents a safe technique for debulking and resection of clival chordomas. Due to the rarity of clival chordomas, it is important that patients with this pathology are managed in high volume skull base centres where a multi-disciplinary team approach is available.


Assuntos
Cordoma/cirurgia , Cirurgia Endoscópica por Orifício Natural/métodos , Neuroendoscopia/métodos , Neoplasias da Base do Crânio/cirurgia , Adolescente , Adulto , Idoso , Vazamento de Líquido Cefalorraquidiano/etiologia , Vazamento de Líquido Cefalorraquidiano/cirurgia , Fossa Craniana Posterior/cirurgia , Diplopia/etiologia , Diplopia/cirurgia , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/cirurgia , Nariz/cirurgia , Cuidados Pós-Operatórios/métodos , Reoperação , Estudos Retrospectivos , Cirurgiões , Resultado do Tratamento , Adulto Jovem
17.
J Surg Oncol ; 119(7): 856-863, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30734292

RESUMO

BACKGROUND: We reviewed the disease control and complications of the treatment of sacrococcygeal chordoma from four tertiary cancer centers with emphasis on the effects of radiotherapy in surgically treated patients. METHODS: A total of 193 patients with primary sacrococcygeal chordoma from 1990 to 2015 were reviewed. There were 124 males, with a mean age of 59 ± 15 years and a mean follow-up of 7 ± 4 years. Eighty-nine patients received radiotherapy with a mean total dose of 61.8 ± 10.9 Gy. RESULTS: The 10-year disease-free and disease-specific survival was 58% and 72%, respectively. Radiation was not associated with local recurrence (hazard ratio [HR], 1.13; 95% confidence interval [CI], 0.59-2.17; P = 0.71), metastases (HR, 0.93; 95% CI, 0.45-1.91; P = 0.85) or disease-specific survival (HR, 0.96; 95% CI, 0.46-2.00; P = 0.91). Higher doses (≥70 Gy; HR, 0.52; 95% CI, 0.20-1.32; P = 0.17) may be associated with reduced local recurrence. Radiotherapy was associated with wound complications (HR, 2.76; 95% CI, 1.64-4.82;, P < 0.001) and sacral stress fractures (HR, 4.73; 95% CI, 1.88-14.38; P < 0.001). CONCLUSIONS: In this multicenter review, radiotherapy was not associated with tumor outcome but associated with complications. The routine use of radiotherapy with en-bloc resection of sacrococcygeal chordomas should be reconsidered in favor of a selective, individualized approach with a radiation dose of ≥70 Gy.


Assuntos
Cordoma/radioterapia , Sacro/efeitos da radiação , Neoplasias da Coluna Vertebral/radioterapia , Cordoma/cirurgia , Relação Dose-Resposta à Radiação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sacro/patologia , Sacro/cirurgia , Neoplasias da Coluna Vertebral/cirurgia , Resultado do Tratamento
18.
Eur J Phys Rehabil Med ; 55(4): 526-529, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29898586

RESUMO

BACKGROUND: Sacral neoplasms often present as large masses that often require sacrectomy. Multiple sacral nerve roots may be compromised post-sacrectomy and postoperative complications may result in impaired mobility, pain, orthostasis, and neurogenic bowel and bladder. CASE SERIES: Case 1, 58 year-old female with a sacral solitary fibrous tumor underwent a high-level sacrectomy and bilateral gluteal muscle flaps. Her rehabilitation course included management of pain, orthostasis, and neurogenic bowel and bladder. Case 2, 67 year-old male with sacral chordoma underwent high-level sacrectomy and bilateral gluteal muscle flaps. His rehabilitation course was complicated by refractory orthostatic hypotension, pain, and wound impairment, which resulted in slow rehabilitation progression and bowel and bladder training. Progression of activity in both cases was limited by surgical restrictions to support wound healing. CLINICAL REHABILITATION IMPACT: Multidisciplinary efforts after a sacrectomy are vital to successful rehabilitation. Highly functional outcomes are seen, including independent bowel and bladder management and return to preoperative ambulatory status.


Assuntos
Cordoma/cirurgia , Fibroma/cirurgia , Complicações Pós-Operatórias/reabilitação , Sacro , Neoplasias da Coluna Vertebral/cirurgia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
19.
Eur J Orthop Surg Traumatol ; 29(1): 9-15, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30066091

RESUMO

Sacral chordoma are rare low-to-intermediate grade malignant tumours that occur most commonly within the sacrum. Sacrectomy with wide resection margins seems to offer the best long-term prognosis.  This study aims to review the management of sacral chordomas including the duration of symptoms, features, treatment, complications and local recurrence rate following surgery at a tertiary centre. We retrospectively reviewed 11 patients treated at our institution between years 1999 and 2015. Patient data included age, sex, history, radiographs, surgical details, onset of recurrence, subsequent treatment, disease-free survival and overall survival were analyzed. Nine patients underwent surgical management with 1 through a sacral approach and eight patients through a combined abdominosacral approach. Despite wide resection in our series, sacral chordoma poses a major problem with approximately 60% of patients having local recurrence in their follow-up.


Assuntos
Neoplasias Ósseas/cirurgia , Cordoma/cirurgia , Recidiva Local de Neoplasia/cirurgia , Adulto , Idoso , Neoplasias Ósseas/patologia , Cordoma/patologia , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sacro , Retalhos Cirúrgicos , Deiscência da Ferida Operatória/etiologia
20.
World Neurosurg ; 121: e246-e261, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30261397

RESUMO

INTRODUCTION: With the introduction into the neurosurgical practice of minimally invasive methods using endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. OBJECTIVE: To analyze the results of surgical treatment of patients with various centrally located tumors of the base of the skull that extend into the anterior region of the posterior cranial fossa using the endoscopic endonasal transclival approach. METHODS: The personal surgical experience of the first author is 136 patients with various tumors (e.g., chordomas, meningiomas, pituitary adenomas, and fibrous dysplasia). RESULTS: Radicality of tumor removal was total 61.03%; subtotal 25%; partial 13.23%; and insufficient removal 0.74%. Postoperative cerebrospinal fluid leaks occurred in 9 cases (6.62%) and meningitis in 13 cases (9.56%). In 2 cases, surgical treatment had a lethal outcome (1.47%). CONCLUSIONS: The endoscopic endonasal transclival approach can be used to obtain access to centrally located skull base tumors. This approach allows for a radical and low-risk removal of various skull base tumors of central localization that, until recently, were considered to be almost inoperable.


Assuntos
Fossa Craniana Posterior/cirurgia , Neoplasias da Base do Crânio/cirurgia , Adulto , Cordoma/cirurgia , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Meningioma/cirurgia , Pessoa de Meia-Idade , Cirurgia Endoscópica por Orifício Natural/métodos , Neuroendoscopia/métodos , Procedimentos Neurocirúrgicos/métodos , Neoplasias Hipofisárias/cirurgia , Complicações Pós-Operatórias/etiologia , Tomografia Computadorizada por Raios X
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