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.

Cordoma Sacral: Relato de uma Rara Neoplasia Maligna / Sacral Chordoma: Report of a Rare Malignant Neoplasm / Cordoma Sacral: Relato de uma Rara Neoplasia Maligna

Introdução: O cordoma é um tipo de sarcoma cuja malignidade óssea primária se origina da notocorda e se localiza no eixo espinhal entre o clivus e o sacro. A primeira descrição dessa patologia foi em 1857. Na epidemiologia da doença, são mais afetados pacientes entre 40 e 60 anos, sendo o principal sítio de acometimento a região sacrococcígea. O quadro clínico é variável conforme o local acometido com sintomas geralmente inespecíficos, gerando atrasos no diagnóstico feito por biópsia. Entre as opções de tratamento, o principal método é a ressecção cirúrgica com margens, que pode ser associada à radioterapia ou à radiocirurgia quando necessário; os sítios de metástases mais comuns são pulmões, ossos, fígado e linfonodos locais. Relato do caso: Paciente, sexo feminino, 62 anos, iniciou com quadro de dor em região coccígea com piora ao sentar-se e surgimento de lesão nodular com crescimento progressivo recebendo diagnóstico de cordoma, após biópsia da lesão, depois de três anos. Em razão da lesão extensa, optou-se inicialmente por tratamento com quimio e radioterapia para citorredução. Pela pouca responsividade, foi submetida ao tratamento de sacralectomia com sucesso, porém apresentou como complicação deiscência de ferida operatória e necessidade de reabordagem, desde então sem recorrência no seguimento clínico. Conclusão: Assim, evidencia-se a necessidade de novas pesquisas sobre o cordoma, um tumor raro e de baixa responsividade aos tratamentos não cirúrgicos, visando a melhorar a terapêutica quimioterápica dessa neoplasia potencialmente deformante.

Introduction: Chordoma is a type of sarcoma, a primary bone malignancy that originates from the notochord and is located on the spinal axis between the clivus and the sacrum. The first description of this pathology occurred in 1857. Patients between 40 and 60 years old are the most affected according to the disease's epidemiology, the main site involved is the sacral/coccygeal region. The clinical condition is variable depending on the site affected, with generally nonspecific symptoms, delaying the diagnosis made by biopsy. Among the treatment options, surgical resection with margins is currently the main method, and may be associated with radiotherapy or radiosurgery when necessary; the most common metastatic sites are lungs, bones, liver and local lymph nodes. Case report: A 62-year-old female patient had pain in the coccygeal region, worsening while sitting and the appearance of a nodular lesion with progressive growth, diagnosed as a chordoma three years later, after biopsy of the lesion. Due to the extensive lesion, initially she was submitted to chemotherapy and radiotherapy for cytoreduction, but because of the poor response, she was successfully submitted to sacralectomy, however, dehiscence of the surgical wound was detected, and the patient underwent a new approach; since then, no recurrence in the clinical follow-up. Conclusion: Apparently, it is clear the necessity for further investigations on chordoma, a rare tumor with poor response to non-surgical treatments, in order to improve the chemotherapy for this potentially deforming neoplasm.

Introducción: El cordoma es un tipo de sarcoma, una malignidad ósea primaria que se origina en la notocorda y se localiza en el eje espinal entre el clivus y el sacro. La primera descripción de esta patología fue en 1857. En la epidemiología de la enfermedad, los pacientes entre 40 y 60 años son los más afectados, siendo el principal sitio de afectación la región sacrocoxígea. El cuadro clínico es variable según el sitio afectado, con síntomas generalmente inespecíficos, lo que provoca retrasos en el diagnóstico realizado mediante biopsia. Entre las opciones de tratamiento, la resección quirúrgica con márgenes es actualmente el principal método, pudiendo asociarse a radioterapia o radiocirugía cuando sea necesario; los sitios más comunes de metástasis son los pulmones, los huesos, el hígado y los ganglios linfáticos locales. Informe del caso: Paciente, sexo femenino, de 62 años inició con dolor en la región coccígea, empeorando al sentarse y aparición de una lesión nodular con crecimiento progresivo, recibiendo diagnóstico de cordoma, luego de biopsia de la lesión, después de tres años. Debido a la extensión de la lesión optó inicialmente por tratamiento con quimio y radioterapia para citorreducción, por la poca reactividad fue sometida con éxito al tratamiento de sacralectomía, pero presentó como complicación dehiscencia de la herida quirúrgica y necesidad de reabordaje. Desde entonces sin recurrencia en el seguimiento clínico. Conclusión: Por lo tanto, es evidente la necesidad de seguir investigando sobre el cordoma, un tumor poco frecuente con escasa respuesta a los tratamientos no quirúrgicos, con el fin de mejorar la terapia de quimioterapia para esa neoplasia potencialmente deformante.

Cordoma sacral metastático / Metastatic sacral chordoma

Os cordomas sacrais (CS) são tumores ósseos malignos primários da coluna vertebral de ocorrência rara, com incidência entre 0,000005-0,000027%. O objetivo deste estudo é relatar um caso de CS metastático. Homem de 41 anos, sem comorbidades, chega ao serviço de referência apresentando lesão sacral. Ressonância magnética mostrou tratar-se de tumor com 9,3 cm sugestivo de mieloma ou cordoma. Realizou-se biópsia e histopatológico, confirmando o diagnóstico de CS. O paciente submeteu-se à excisão cirúrgica do tumor. Seis meses após a cirurgia, evoluiu com recidiva e implantes metastáticos em coluna vertebral, partes moles da parede torácica, fígado e espa-ço pleural, evoluindo com paraplegia. Não havia indicação de radioterapia e/ou quimioterapia adjuvante. Não havia também possibilidade de liberação de imatinibe pelo Sistema Único de Saúde. Em cerca de 28 meses de seguimento clínico mensal, o paciente foi a óbito. O caso apresentado mostrou um CS sem sucesso cirúrgico, o que é associa-do a pior prognóstico. O paciente apresentou disseminação sistêmica do tumor e paraplegia poucos meses após a cirurgia, indo a óbito em 28 meses de seguimento. (AU)

Sacral chordomas (SC) are rare primary malignant bone tumors of the vertebral column, with an incidence between 0.000005-0.000027%. This study aims to describe a case of metastatic SC. A 42-year-old man without comorbid conditions, arrived at the referral center, presenting with a sacral lesion. MRI showed a tumor measuring 9.3 cm that was suggestive of myeloma or chordoma. A biopsy with histopathology study was performed, confirming the diagnosis of SC. The patient underwent surgical tumor excision. Six months after surgery, the tumor recurred with metastatic vertebral column implants, soft tissues of the chest wall, liver, and pleural space, and the patient developed paraplegia. There was no indication of adjuvant radiotherapy and/or chemotherapy. There was also no possibility that the Unified Health System would approve imatinib. At about 28 months of monthly clinical follow-up, the patient died. The case presented showed unsuccessful SC surgery, which is associated with a worse prognosis. The patient had systemic tumor dissemination and paraplegia a few months after surgery, dying at 28 months of follow-up. (AU)

Pediatric SMARCB1/INI1-deficient poorly differentiated chordoma of the skull base: report of five cases and review of literature / 中华病理学杂志

Objective: To investigate the clinicopathological characteristics and differential diagnosis of pediatric SMARCB1/INI1-deficient poorly differentiated chordoma (PDC) of the skull base. Methods: Five cases of SMARCB1/INI1-deficient PDC were identified in 139 cases of chordoma diagnosed in Sanbo Brain Institute, Capital Medical University, Beijing, China from March 2017 to March 2021. The clinical and imaging data of the 5 PDCs were collected. H&E and immunohistochemical staining, and DNA methylation array were used, and the relevant literatures were reviewed. Results: All 5 PDCs were located at the clivus. The average age of the patients was 6.4 years, ranging from 3 to 16 years. Three patients were female and two were male. Morphologically, in contrast with classical chordomas, they presented as epithelioid or spindle tumor cells organized in sheets or nests, with necrosis, active mitoses, and infiltration into surrounding tissue. All cases showed positivity of CKpan, EMA, vimentin and brachyury (nuclear stain), and loss of nuclear SMARCB1/INI1 expression. S-100 protein expression was not frequent (2/5). Ki-67 proliferative index was high (20%-50%). All cases had over-expressed p53. It was necessary to differentiate SMARCB1/INI1-dificient PDC from SMARCB1/INI1-dificient tumors occurring at skull base of children or the tumors with epithelial and spindle cell morphological features. The 3 PDCs with DNA methylation testing showed the methylation profiles different from the pediatric atypical teratoid/rhabdoid tumors. They formed an independent methylation profile cluster. The clinical prognosis of the 5 patients was poor, and the overall survival time was 2-17 months. Conclusions: PDC is a special subtype of chordoma, which often affects children and occurs in the clivus. The PDC shares epithelioid or spindle cell morphologic features which are different from the classic chordoma. Besides the typical immunohistochemical profile of chordoma, PDC also has loss of nuclear SMARCB1/INI1 expression and distinct epigenetic characteristics.

Cordomas cervicales. Reporte de casos / Cervical chordomas. Case report

Introducción La detección temprana del cordoma constituye un desafío médico dada su baja frecuencia, calculada entre 2-4% de todos los tumores primarios del hueso, requiriendo un alto índice de sospecha clínica. El diagnóstico tardío incrementa la morbimortalidad y compromete la sobrevida del paciente, por lo que el conocimiento actualizado sobre su etiopatogenia, clínica y tratamiento es de suma importancia para el cirujano espinal, quien forma parte esencial del grupo interdisciplinario terapéutico. Reporte de casos Reportamos dos casos de cordomas cervicales, localmente agresivos sin extensión sistémica, el primero de ellos ubicado en la columna cervical y el segundo en la unión craneocervical. Ambos con diferentes formas de presentación, síntomas, tiempo de evolución (5 años y 7 meses respectivamente), infiltración tumoral y abordaje quirúrgico, pero similar tratamiento oncológico. El objetivo del manuscrito es demostrar la variedad de presentación cervical de la entidad y las diferentes formas de abordarla. Se incluye una revisión actualizada de la literatura donde se evidencia la importancia de la inmunoterapia como nuevo horizonte terapéutico, y se reafirma la resección quirúrgica como base del mismo. Discusión Se presentan los resultados imagenológicos de descompresión medular, resección ósea y de partes blandas, los tipos de fijación espinal y la evolución clínica, sin recidiva local a los 2 años y a los 12 meses respectivamente en cada caso, luego del manejo quirúrgico y la radioterapia adyuvante.

Background Early detection of chordoma is a medical challenge given its low frequency, a high index of clinical suspicion is required for the diagnosis. The late diagnosis increases morbidity and mortality and compromises the survival of the patient. Knowledge about its etiology, clinical manifestations and treatment is of utmost importance for the spinal surgeon as well as for the therapeutic group. The aim of the report is to demonstrate the variety of cervical presentation of the entity and the different ways of approaching it. Case Report We report two cases of locally aggressive cervical chordomas without systemic extension, the first one located in the cervical spine and the second in the craniocervical junction. Both with different forms of presentation, symptoms, evolution time (5 years and 7 months respectively), tumor infiltration and surgical approach, but similar oncological treatment. Discussion The imaging results of medullar decompression, bone and soft tissue resection, types of spinal fixation and clinical evolution were presented. No local recurrence at 2 years and 12 months respectively after surgical management and adjuvant radiation therapy were found.

Metastatic poorly differentiated chordoma: the eyes do not see what the mind does not know

Chordoma is a rare tumor. It has unique clinical, pathological and immunohistochemical characteristics. Accurate diagnosis is essential as the tumor shows an aggressive clinical course and requires a multimodal therapeutic approach. A case with wide spread distant metastatic disease that was initially thought to represent metastatic thyroid carcinoma is presented. Appropriate clincopathologic correlation and the histologic findings raised the possibility of poorly differentiated chordoma. The diagnosis was confirmed by immunohistochemistry for INI-1 and Brachyury. The approach to the diagnosis emphasizing the clinical and pathologic findings of this case is discussed and reviewed in the context of the published literature.

A Case of Sacrococcygeal Chordoma / 대한피부과학회지

No abstract available.

Incidentally-Discovered Extraosseous Cystic Nasopharyngeal Chordoma in a Papillary Thyroid Cancer Patient

Skull base chordomas are rare, malignant tumors arising from primitive notochord remnants of the axial skeleton and comprise approximately 25–35% of all chordoma cases. Nasal endoscopy in previous case reports has characterized nasopharyngeal chordomas as firm, semi-translucent masses protruding from the posterior nasopharyngeal wall with a pink, “meaty” appearance. However, the nasopharyngeal chordoma in the present case had a soft, cystic appearance, unlike the tumors previously described. Herein, an unusual case of an incidentally discovered nasopharyngeal chordoma is reported in a patient with papillary thyroid cancer; the discovered chordoma had a benign cystic appearance with no abnormal positron emission tomography-computed tomography (PET-CT) uptake.

Chordomas: Histopathological Study in View of Anatomical Location

BACKGROUND: Chordomas are aggressive bone tumors that have a predilection for the axial skeleton including the skull base and spinal/sacral bones. However, the histopathological and clinical differences between skull base chordoma (SBC) and sacral/spinal chordoma (SC) are unclear as previous studies have been focused on patient prognosis and treatment outcome. This study aimed to evaluate the clinicopathologic features and prognosis of chordoma according to its location. METHODS: Patients with chordomas were enrolled, and the histopathologic features were compared according to the tumor location. RESULTS: A total of 52 patients were enrolled. SBCs had more abundant chondroid matrix and diffuse growth pattern, while SCs had non-chondroid, myxoid matrix and a lobulating pattern, typical of chordoma. Old age and residual tumors were risk factors for shorter overall survival in SBCs. The chondroid matrix was an independent risk factor for shorter disease-free survival in the overall population. CONCLUSION: Chordomas have different histopathologic features depending on the anatomical location.

Anesthesia management of cervical chordoma resection: A case report / 北京大学学报(医学版)

Chordoma is a slow-growing, locally invasive, lowgrade malignant tumor with a prevalence of one in 100 000, accounting for 1%-4% of all malignant bone tumors. At present, it is considered that chordoma originates from ectopic embryonic chordal tissue and can occur in any part of the spine from the skull base to the sacrum. About 50% of chordoma occurs in the sacrococcygeal region, about 30% in the skull base, and the rest occurs in the active spinal region. Cervical chordoma is rare, but it may be accompanied by difficult airways. The tumors compress the pharynx and throat forward, which can cause upper airway obstruction. If the anesthesia is not properly handled, the patient may die of asphyxia. The core issues of airway management during the perioperative period of cervical chordoma surgery involve three main parts: preoperative airway evaluation, airway management and extubation management. Difficult airway assessment often relies on physical examination indicators, such as inter-incisor gap, thyromental distance, neck circumference, Mallampati test, etc. But the accuracy is insufficient. The application of imaging examination in the observation of different tissues can make up for the inaccurate evaluation of the internal structure of the airway. Because chordoma destroys cervical vertebral body and accessories, cervical stability is impaired. Excessive cervical vertebral extention should be avoided during tracheal intubation to prevent severe compression of the spinal cord. It is better to fix the head by an assistant and perform neutral tracheal intubation. Considering that the patient with a difficult airway that could be predicted before operation, the strategy of tracheal intubation under conscious sedation with topical anesthesia was selected. After sedation and topical anesthesia, the patient was successfully intubated with optical stylet. After operation, the patient returned to ICU with tracheal catheter. On the 4th day after operation, the tracheal tube was pulled out. On the 5th day after operation, the patient was transferred to the orthopaedic ward and discharged on the 7th day after operation. It is of great significance to establish specific strategies for such operations to reduce related complications, speed up post-operative rehabilitation and save medical resources. We reported the anesthetic management of cervical chordoma cured in Peking University Third Hospital.

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)

Clinical outcome of proton therapy for patients with chordomas

PURPOSE: To investigate the clinical outcome of proton therapy (PT) in patients with chordoma. MATERIALS AND METHODS: Fifty-eight patients with chordoma treated with PT between June 2007 and December 2015 at the National Cancer Center, Korea, were retrospectively analyzed. The median total dose was 69.6 cobalt gray equivalent (CGE; range, 64.8 to 79.2 CGE). Local progression-free survival (LPFS), distant metastasis-free survival (DMFS), overall survival (OS), and diseasespecific survival (DSS) rates were calculated by the Kaplan–Meier method. RESULTS: With the median follow-up of 42.8 months (range, 4 to 174 months), the 5-year LPFS, DMFS, OS, and DSS rates were 87.9%, 86.7%, 88.3%, and 92.9%, respectively. The tumor location was associated with the patterns of failure: the LPFS rates were lower for cervical tumors (57.1%) than for non-cervical tumors (93.1%) (p = 0.02), and the DMFS rates were lower for sacral tumors (53.5%) than for non-sacral tumors (100%) (p = 0.001). The total dose was associated with both the LPFS rate and DMFS rate. The initial tumor size was associated with the DMFS rate, but was not associated with the LPFS rate. Three patients had grade 3 late toxicity with none ≥grade 4. CONCLUSION: PT is an effective and safe treatment in patients with chordomas. The tumor location was associated with the patterns of failure: local failure was common in cervical tumors, and distant failure was common in sacral tumors. Further refinement of PT, such as the utilization of intensity modulated PT for cervical tumors, is warranted to improve the outcome.

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

Cordoma en la articulación facetaria de C4: reporte de caso / Chordoma in C4 Facet Joint: A Case Report

Los cordomas son tumores óseos primarios, poco frecuentes, derivados de remanentes no diferenciados de la notocorda. Por su origen histológico, suelen ubicarse en la línea media del esqueleto axial, y los lugares de presentación más frecuentes son la base del cráneo y la columna. Se caracterizan por presentar un crecimiento lento, por lo que tienden a ser clínicamente silenciosos hasta alcanzar tamaños que causan manifestaciones que varían según el sitio de presentación; sin embargo, tienen alta agresividad y recurrencia local. El tratamiento es quirúrgico e, idealmente, se busca una resección completa de la lesión. El artículo presenta el caso de un hombre de 20 años de edad, quien desarrolló un cordoma en la articulación facetaría superior izquierda de C4, que es una localización rara.

Chordoma are rare primar? bone tumours derived from non'difieren tiated remains of the notochord. Due to their histological origina, the most common site of presentation is on the mid'line of the axial skeleton, with a distribution that is most frequent on the sacral bone, skull base and mobile spine. These tumours have a slow growth rate, which means that symptoms occur when the size of the mass causes different manifestations according to its site of presentation. Howeveti they have aggressive behaviour with high rates of local recurrence. Ideal treatment is based on surgical block removal if possible. This article presents the case of a 20' year'old male patient with diagnosis of a chordoma on the leít superior facetar? articulation of C4.

A Case of Coincidental Intrasellar Chordoma and Pituitary Adenoma

Although chordomas are midline tumors, primarily intrasellar chordomas are extremely rare. In this report, the authors describe the case of a 68-year-old female with partial abducens nerve palsy in the right eye due to the intrasellar cystic tumor. After endonasal trans-sphenoidal surgery, intraoperative and histopathological findings confirmed the co-occurrence of an entirely intrasellar chordoma and pituitary adenoma. To our knowledge, the present case is the third reported case of an intrasellar chordoma with a pituitary adenoma.

Diagnosis of occipital neuralgia due to upper cervical chordoma

No abstract available.