3D-printed vertebral body in anterior spinal reconstruction after total spondylectomy for patients with cervical chordoma / 北京大学学报(医学版)

OBJECTIVE@#To investigate whether 3D-printed artificial vertebral body can reduce prosthesis subsidence rate for patients with cervical chordomas, through comparing the rates of prosthesis subsidence between 3D printing artificial vertebral body and titanium mesh for anterior spinal reconstruction after total spondylectomy.@*METHODS@#This was a retrospective analysis of patients who underwent surgical treatment for cervical chordoma at our hospital from March 2005 to September 2019. There were nine patients in the group of 3D artificial vertebral body (3D group), and 15 patients in the group of titanium mesh cage (Mesh group). The patients' characteristics and treatment data were extracted from the medical records, including age, gender, CT hounsfield unit of cervical vertebra and surgical information, such as the surgical segments, time and blood loss of surgery, frequency and degree of prosthesis subsidence after surgery. Radiographic observations of prosthesis subsidence during the follow-up, including X-rays, CT, and magnetic resonance imaging were also collected. SPSS 22.0 was used to analysis the data.@*RESULTS@#There was no significant difference between the two groups in gender, age, CT hounsfield unit, surgical segments, time of surgery, blood loss of posterior surgery and total blood loss. Blood loss of anterior surgery was 700 (300, 825) mL in 3D group and 1 500 (750, 2 800) mL in Mesh group (P < 0.05). The prosthesis subsidence during the follow-up, 3 months after surgery, there was significant difference between the two groups in mild prosthesis subsidence (P < 0.05). The vertebral height of the 3D group decreased less than 1 mm in eight cases (no prosthesis subsidence) and more than 1 mm in one case (mild prosthesis subsidence). The vertebral height of the Mesh group decreased less than 1 mm in five cases (no prosthesis subsidence), and more than 1 mm in eight cases (mild prosthesis subsidence). Two patients did not have X-rays in 3 months after surgery. There was a statistically significant difference between the two groups in the prosthesis subsidence rate at the end of 12 months (P < 0.01). The vertebral height of eight cases in the 3D group decreased less than 1 mm (no prosthesis subsidence) and one case more than 3 mm (severe prosthesis subsidence). Four of the 15 cases in the Mesh group decreased less than 1 mm (no prosthesis subsidence), two cases more than 1 mm (mild prosthesis subsidence), and nine cases more than 3 mm (severe prosthesis subsidence). There was a statistically significant difference between the two groups in the prosthesis subsidence rate at the end of 24 months (P < 0.01). The vertebral height of seven cases in the 3D group decreased less than 1 mm (no prosthesis subsidence), one case more than 3 mm (severe prosthesis subsidence), and one case died with tumor. One case in the Mesh group decreased less than 1 mm (no prosthesis subsidence), one case more than 1 mm (mild prosthesis subsidence), 11 case more than 3 mm (severe prosthesis subsidence), one case died with tumor and one lost the follow-up. Moreover, at the end of 12 months and 24 months, there was significant difference between the two groups in severe prosthesis subsidence rate (P < 0.01).@*CONCLUSION@#3D-printed artificial vertebral body for anterior spinal reconstruction after total spondylectomy for patients with cervical chordoma can provide reliable spinal stability, and reduce the incidence of prosthesis subsidence after 2-year follow-up.

Reto neuroquirúrgico actual del cordoma cervical / Current neurosurgical challenge of the cervical chordoma

El cordoma es un tumor osteocartilaginoso raro, de lento crecimiento, con una tasa de incidencia global de 8,4 casos por cada 10 millones de habitantes. Comúnmente aparece en la quinta y sexta década de la vida, predomina en el sexo masculino. Se presenta un paciente masculino de 47 años de edad, con cervicobraquialgia, cuadriparesia y masa tumoral palpable en región anterolateral del cuello. El estudio de resonancia magnética demostró la presencia de una lesión retrofraríngea con destrucción vertebral y compresión extradural. Se decidió resección quirúrgica de la lesión. El diagnóstico histopatológico por inmunohistoquímica arrojó como resultado, un cordoma(AU)

Chordoma is a rare, slow-growing osteocartilaginous tumor with an overall incidence rate of 8.4 cases per 10 million inhabitants. Commonly appears in the fifth and sixth decade of life, predominates in the male sex. We present a 47-year-old male patient with cervicobrachialgia, quadriparesis and palpable tumor mass in the anterolateral region of the neck. The magnetic resonance study showed the presence of a retropharyngeal lesion with vertebral destruction and extradural compression. Surgical resection of the lesion was decided. The histopathological diagnosis by immunohistochemistry resulted in a chordoma(AU)

Sacrectomía parcial por abordaje posterior único / Partial sacrectomy by single posterior approach

Introducción: Los tumores del sacro representan <7% de los tumores espinales, prevalecen los tumores secundarios por mieloma múltiple o carcinomas de próstata, mama, pulmón o colón. El cordoma es el tumor maligno primario más frecuente y el tumor de células gigantes es la lesión benigna más común. Por su evolución, compromiso de estructuras extraóseas y la escasa respuesta a los tratamientos coadyuvantes, la cirugía es el tratamiento más utilizado, la vía de abordaje y la necesidad de instrumentación dependerán del tumor por tratar. Los objetivos de este trabajo son: evaluar el uso de la resección parcial del sacro, analizar la técnica quirúrgica y reconocer las complicaciones. Materiales y Métodos: Cuatro pacientes con diagnóstico de tumor sacro ubicado por debajo de S1, que consultan por dolor y cuyas imágenes confirman la lesión. A todos se les realiza una resección en bloque por vía posterior preservando S1. Se describe la técnica. Resultados: Los estudios anatomopatológicos revelaron: un cordoma, un tumor maligno de vaina nerviosa, un condrosarcoma y una metástasis de carcinoma prostático. Se preservó la función de S1 en todos los pacientes; uno tiene disfunción vesical permanente. Se observaron una dehiscencia de la herida, una infección y una fístula de líquido cefalorraquídeo. Todos permanecen sin la enfermedad tras un seguimiento de entre 6 y 24 meses. Conclusiones: La resección parcial del sacro por abordaje posterior único se puede indicar cuando la lesión compromete desde S2 hacia distal y no hay compromiso sacroilíaco. La preservación de raíces es de vital importancia para garantizar mejores resultados posoperatorios y una menor tasa de infección. Nivel de Evidencia: IV

Introduction: Tumors of the sacrum represent less than 7% of spinal tumors. Secondary tumors due to multiple myeloma or prostate, breast, lung and colon carcinomas predominate. Chordoma is the most frequent primary malignant tumor and giant cell tumor is the most common benign lesion. For its evolution, involvement of extra bone structures and poor response to the adyuvant therapy, surgery is the most commonly used treatment, the approach and the need for instrumentation depend on tumor to treat. The objectives of this study are to evaluate the use of partial resection of sacrum, analyze the surgical technique and assess complications. Methods: Four patients with diagnosis of sacral tumor below S1, who presented with pain and images confirming the injury. All underwent an en bloc resection by posterior via preserving S1. The technique is described. Results: The pathological results were: a chordoma, a malignant nerve sheath tumor, a chondrosarcoma and a metastasis of prostatic carcinoma. S1 function was preserved in all patients; one has permanent bladder dysfunction. Wound dehiscence, infection and cerebrospinal fluid fistula were detected. Patients are free of disease after a follow-up of 6-24 months. Conclusions: Partial resection of the sacrum using a unique posterior approach may be indicated when the injury involves from S2 distally and no sacroiliac involvement is observed. Root preservation is vital to guarantee the best postoperative results and a lower rate of infection. Level of Evidence: IV

Natural history and surgical treatment of chordoma: a retrospective cohort study / História natural e tratamento cirúrgico de cordoma: um estudo retrospectivo de coorte

CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition. DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil. METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test. RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5%) were located in the sacrum, eleven (26.2%) in the skull base and six (14.3%) in the mobile spine. Surgery was performed on 28 patients (66.7%). The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS) was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins). In the inoperable group, OS was 37.7% at 24 months and 0% at five years. CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival. .

CONTEXTO E OBJETIVO: Cordoma é um tumor raro e com alto risco de recidiva locorregional. O objetivo deste estudo foi analisar os resultados a longo prazo do tratamento dessa doença. TIPO DE ESTUDO E LOCAL: Estudo de coorte realizado em um único hospital em São Paulo, Brasil. MÉTODOS: Estudo de coorte retrospectivo com 42 pacientes com cordoma tratados de 1980 e 2006 no Hospital A. C. Camargo. Os prontuários foram revistos e foi realizada a análise descritiva das variáveis clínicas e patológicas. As curvas de sobrevida foram estimadas pelo método de Kaplan-Meier e a comparação entre elas, pelo teste de log-rank. RESULTADOS: Dezenove pacientes eram homens e 23, mulheres. Vinte e cinco tumores (59,5%) estavam localizados no sacro, 11 (26,2%) na base do crânio e 6 (14,3%), na coluna móvel. A cirurgia foi realizada em 28 pacientes (66,7%). A ressecção foi considerada como tendo margens negativas em 14 casos e margens comprometidas em 14 pacientes. A sobrevida global (SG) em 5 anos foi de 45,4%. Para os pacientes cirúrgicos, a SG em 5 anos foi de 64,3% (82,2% para as margens negativas e 51,9% de margens positivas). No grupo inoperável, a SG em 24 meses foi de 37,7% e 0% em 5 anos. CONCLUSÃO: A ressecção completa está relacionada com o controle local e, definitivamente, tem impacto positivo na sobrevida a longo prazo. .

Tratamiento quirúrgico de los tumores pelvianos avanzados que implican resección sacra / Surgical treatment of the advanced pelvianos tumors that imply sacred resection

Antecedentes: La invasión ósea del sacro fue tradicionalemente considerada como criterio de irresecabilidad (pelvis congelada). Objetivo: Analizar la técnica y los resultados a mediano plazo de la Resecciones Sacras (RS) en Tumores Pelvianos Avanzados (TPA) realizadas en el Instituto Alexander Fleming. Diseño: Estudio retrospectivo. Población: 21 pacientes portadores de TPA que requirieron RS a diversos niveles. Método: Se analizaron retrospectivamente las Historias Clínicas de los 21 pacientes. El seguimiento se realizó mediante visitas periódicas en consultorio externo, o telefónicamente en los perdidos. La supervivencia se analizó con el método actuarial de Kaplan Meier. Resultados: La altura de RS fue: S1 9,5%, S2 38%, S3 28,6%, S4 14,4% y S5 9,5%. La mortalidad de la serie fue del 81%. Transtornos esfinterianos 24%; infección y dehiscencia de herida posterior 57%; infección de la herida anterior 9,5% y fístula posoperatoria 9,5%. El seguimiento promedio fue de 21 meses, la supervivencia libre de enfermedad fue de 17 meses, la supervivencia global acutuarial a 5 años fue de 37,6 y excluyendo los 2 cordomas operados del 20,2%. Conclusiones: Técnica factible realizada por grupos entrenados. Procedimiento e internación prolongados. Es necesario estricta selección de los pacientes para evitar morbimortalidad innecesaria. La invasión sacra No debe ser considerada una contraindicación para el intento de resección.

Intrasellar chordoma

Intrasellar chordomas are uncommon; there are no pathognomonic clinical or diagnostic imaging features. We report clinical, diagnostic imaging, operative findings and histopathology of a case of intrasellar chordoma. Intrasellar chordomas may mimic pituitary adenoma; our case report illustrates the difficulty inherent in the diagnosis of this neoplasm

Crohn's disease associated to chordoma: a case report

Crohn's disease is an inflammatory bowel disease characterized by remissions and exacerbations. Immunosuppressants are frequently used to induce and maintain remission in these patients. The use of the immunomodulator azathioprine has been associated to malignancies. Chordomas are rare, locally aggressive tumors arising from remnants of the notochord. A specific trigger for this tumor has not been identified and association to any medication has not been reported. The purpose of this report is to present the first case reported in the literature of Crohn's disease associated to a chordoma. The patient to be presented was on azathioprine therapy, among other medications. A review of literature revealed that Crohn's disease and chordoma have abnormalities in chromosomes 1 and 10. Inflammatory bowel disease and chordoma also have abnormalities in chromosomal regions 1p, 3p, and 7q. Despite these findings, a direct genetic relationship between these diseases is speculative.

Cordomas gigantes del clivus: ¿Se justifican las cirugías extensas? / Giant clivus chordomas: Are extensive surgeries really justified?

Debido a su localizacion y comportamiento biológico, los cordomas del clivus son tumores que aún representan un gran reto, principalmente cuando han alcanzado grandes dimensiones. En este artículo, hemos seleccionado a los pacientes con los cordomas del clivus más grandes, a fin de analizar si realmente se pueden beneficiar con procedimientos quirúrgicos extensos, con la morbilidad que ello implica. Once pacientes con cordomas gigantes del clivus fueron incluidos, todos ellos intervenidos de acuerdo a los criterios recientes de cirugía de base de cráneo. Fue analizado principalmente si el grado de resección presentaba alguna relación con el estado funcional postoperatorio y el tiempo de supervivencia libre de enfermedad; también fue evaluado si existía alguna correlación entre los hallazgos histopatológicos y el curso clínico. En este grupo fue posible lograr una resección total del tumor en 8 pacientes y parcial en 3. En todos los casos se observó una evidente mejoría en el estado funcional postoperatorio, pero particularmente en los pacientes con resecciones totales. El tiempo de supervivencia libre de enfermedad fue también mayor en los pacientes con las resecciones más amplias. No se encontró correlación alguna entre la variedad histológica de los tumores (clásico o condroide) y el pronóstico clínico.

Because of their location and biological behavior, clivus chordomas are tumors that still represent a great challenge, mainly when they have reached huge dimensions. In this paper we have selected the patients with the biggest clivus chordomas to analyze if they really can be helped with the current surgical procedures with the morbidity that it implies. Eleven patients with giant clivus chordomas were included. All of them were operated on following the recent criteria of Cranial Base Surgery. It was mainly analyzed, if the degree of the resection had any relation to the postoperative functional outcome and the disease-free survival time; it was also evaluated if there existed a correlation between the pathologic findings and the clinical course. It was possible to get a total resection in 8 patients and partial in 3. In all cases there was an evident improvement in their postoperative functional outcome, specially in those with complete resections. The disease-free survival time was also greater in the patients with more ample resections. There was not any correlation among the histologic variety of tumors (typical or chondroid) andthe clinical prognosis.

Tratamento de dor em paciente com tumor sacral inoperável: relato de caso / Management of pain in patient with inoperable sacral tumor: case report

JUSTIFICATIVA E OBJETIVOS: Diversas técnicas podem ser utilizadas no controle de dor em pacientes oncológicos. O objetivo deste relato é mostrar o uso de algumas medidas terapêuticas empregadas para tratar um paciente com quadro doloroso importante de difícil controle. RELATO DO CASO: Paciente do sexo masculino, 70 anos, com cordoma sacral e com possibilidade terapêutica muito difícil. Apresentava quadro de dor importante associado ao tumor. São relatadas diversas técnicas utilizadas no seu tratamento e os resultados obtidos. CONCLUSÕES: Os bloqueios neurolíticos, para o controle da dor em paciente com tumores cuja possibilidade terapêutica é difícil, constituem técnica eficaz quando bem indicados e realizados dentro de critérios estabelecidos.