A Rare Case of Clival Hemangioma Simulating Chordoma / Um caso raro de hemangioma clival simulando cordoma

Primary intraosseous hemangiomas are rare, benign, vascular malformations that account for 1% of all primary bone neoplasias. A 59-year-old female patient with unknown comorbidities had a history of headache, visual impairment and dizziness that led to the diagnosis of a clivus tumor. Two resections were attempted through transcranial and transnasal transsphenoidal approach in the last two years in another hospital. The initial MRI scan showed an expansive lesion with T2 hyperintense signal and diffuse, heterogenic contrast enhancement. Clival chordoma was the main diagnostic hypothesis done. A CT scan was performed to evaluate the extent of clival invasion, the sinus anatomy, and the clival destruction - all simulating clival chordoma. The interdisciplinary tumor board decided to proceed with endoscopic endonasal tumor resection. There were no postoperative complications and the histopathological analysis revealed a primary intraosseous haemangioma. Skull base intraosseous hemangiomas are rare entities, with a limited number of case reports found after literature reviews, especially in the clival region. The clinical pattern and imaging characteristics can vary widely according to the tumor extension and development, simulating some other common tumors found at this topography. We present a case report of a clival intraosseous hemangioma presenting as an isolated abducens paresis with a positive outcome after intranasal endoscopic resection after two years of follow-up.

Os hemangiomas intraósseos primários são malformações vasculares benignas raras que representam cerca de 1% de todas as neoplasias ósseas primárias. Uma paciente de 59 anos do sexo feminino com comorbidades desconhecidas história de cefaleia deficiência visual e tontura que levou ao diagnóstico de tumor de clivus. Duas ressecções foram tentadas por via transcraniana e transesfenoidal transnasal nos dois anos anteriores ao presente estudo em outro hospital. A ressonância magnética inicial mostrou lesão expansiva com sinal hiperintenso em T2 e realce difuso e heterogêneo pelo contraste. Cordoma clival foi a principal hipótese diagnóstica. Uma tomografia computadorizada foi realizada para avaliar a extensão da invasão clival a anatomia do seio e a destruição clival - todas simulando cordoma clival. A comissão interdisciplinar do tumor decidiu prosseguir coma ressecção endoscópica do tumor endonasal. Não houve complicações pós-operatórias e a análise histopatológica revelou hemangioma intraósseo primário. Os hemangiomas intraósseos da base do crânio são entidades raras com número limitado de relatos de casos encontrados após revisões da literatura principalmente na região clival. O padrão clínico e as características de imagem podem variar amplamente de acordo com a extensão e desenvolvimento do tumor simulando alguns outros tumores comuns encontrados nesta topografia. Apresentamos um relato de caso de hemangioma clival intraósseo apresentando-se como uma paresia isolada do abducente com evolução positive após ressecção endoscópica intranasal e dois anos de acompanhamento.

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.

A case-control study of endoscopic endonasal approach and transcranial approach in the treatment of skull base chordomas / 中国神经精神疾病杂志

Objective To investigate the surgical outcomes and strategies selection of endoscopic endonasal approach and craniotomy in the treatment of cranial base chordomas.Methods Thirty-one patients diagnosed pathologically with cranial base chordoma in Tianjin huanhu hospital from Jan.2010 to Sep.2020 were analyzed retrospectively.The patients were divided into the endoscopic endonasal group and the craniotomy microscope group according to the different surgical approaches.The surgical results and follow-up between the two groups were compared.Results In the endoscopic endonasal group,there were 7 cases of gross total resection,9 cases of subtotal resection and 2 cases of partial resection.The main complications included death in 2 cases,cerebrospinal fluid leakage in 8 cases,cranial nerve injury in 2 cases and hypopituitarism in 1 case.In the craniotomy microscope group,there were 2 cases of gross total resection,10 cases of subtotal resection,and 1 case of partial resection.The main complications included cerebrospinal fluid leakage in 1 case,cranial nerve injury in 3 cases,epilepsy in 1 case and epidural hematoma in 1 case.There was no statistical significance in the resection rate between the two groups(P＞0.05).The rate of cerebrospinal fluid leakage in the endoscopic group was significantly higher than that in the craniotomy microscope group,and the comparison was statistically significant(P＜0.05).There was no statistically significant between the two groups for tumor recurrence or progression.Conclusions The endoscopic endonasal approaches for resection of cranial base chordomas have improved the gross total resection rate,but craniotomy is still an important surgical method for tumor resection.It is necessary to select an appropriate surgical approach according to the lesion location and pattern of tumor growth.

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)

Dedifferentiated chordoma: A rare tumor with diagnostic challenge

Chordomas are rare locally invasive malignant bone tumors arising from remnants of embryonic notochord. Dedifferentiated chordoma (DC), a rare subtype, is characterized by the presence of a sarcomatous component in conventional chordoma (CC) which may arise de novo or as a malignant transformation of previously treated chordoma. The presence of dedifferentiation warrants a poor prognosis due to distant metastasis and recurrences. De novo DCs pose a diagnostic challenge especially in small biopsies and at metastatic sites. Here, we report the case of a 45-year-old female presenting with a long history of backache and constipation, finally diagnosed as DC. Radiological as well as histomorphological pictures of the tumor posed diagnostic challenges because they can mimic other tumors occurring in a similar location. We found this case worth reporting as de novo DC is rarely reported in the literature and it has the potential to pose diagnostic as well as therapeutic challenges.

Isolated Intradural Prepontine Chordoma Presenting with Imaging Features of Epidermoid Cyst / Cordoma pré-pontino intradural isolado apresentando características de imagem de cisto epidermóide

Chordoma is an erratic aggressive tumor of the brain that typically involves the clivus. The majority of the clivus chordomas reside in the extradural space. Here, we report a unique case of chordoma arising from the prepontine intradural space without bony involvement and presenting with radiological features typical of an epidermoid cyst on magnetic resonance imaging.

Prognostic factors and nomogram to predict the relapse-free survival of cervical chordoma / 中华骨科杂志

Objective:To explore the relevant prognostic factors and construct a nomogram to predict the relapse-free survival of cervical chordoma.Methods:Data of 48 patients with cervical chordoma treated by operation from November 1994 to June 2018 were retrospectively analyzed, including 28 males and 20 females, aged 48.5±15.4 years (range 5-70 years). Data extraction comprised patients age, gender, Karnofsky performance status scale (KPS), duration of preoperative symptoms, location, involved segments, preoperative Frankel score, diagnostic biopsy method, adjuvant radiotherapy, surgical option and complications. Follow-up was conducted at 3, 6, 12 months after surgery and annually, X-Ray/CT/MRI were used to evaluate the progress of disease. Univariate analysis was performed using Kaplan-Meier survival analysis and Log Rank test to identify prognostic factors relevant to relapse-free survival, and multivariate Cox regression analysis was used in multivariate analysis, then R 3.6.2 was used to construct a nomogram.Results:Mean follow-up time was 66.6±51.1 months (range 14-228 months), 35 cases relapsed up to follow-up time, the cumulative 1-year, 3-year and 5-year relapse-free survival were 70.8%, 42.1% and 30.9%. Univariate analysis showed that diagnostic biopsy method ( P=0.016), adjuvant radiation therapy ( P=0.027), surgical option ( P<0.001) were relevant to relapse-free survival of cervical chordoma. Multivariate Cox regression analysis showed that surgical option (intralesional resection after extracapsular separation vs. directly intralesional resection), HR=0.209, 95% CI (0.076, 0.575) had significant impacts on relapse-free survival of cervical chordoma. A nomogram with c-index of 0.760 to predict 1-year, 3-year and 5-year relapse-free survival was conducted basing on age, gender, location, involved segments, diagnostic biopsy method, adjuvant radiation therapy, surgical option. Conclusion:Aspiration biopsy, intralesional resection after extracapsular separation and adjuvant radiation therapy could prolong the relapse-free survival of cervical chordoma. The nomogram in this study could predict 1-year, 3-year and 5-year relapse-free survival of cervical chordoma with relatively good accuracy.

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.

Mandibular metastasis as a presenting feature of a clival chordoma

Chordomas are rare tumors which arise from the embryological remnants of the notochord. These tumors can potentially arise from any region within the craniospinal axis and often clinically present as a diagnostic challenge. Chordomas are rare in patients younger than 40 years of age. The most common primary cancers that metastasize to the jaw bones are the ones originating from the breast, lung, kidney adrenal, colo-rectum, or prostate. Mandibular metastasis from a primary chordoma is an extremely rare occurrence with only five prior reports, three originating from primaries in the sacrococcygeal region, one from a lumbar spine primary and the other from a primary arising from the spheno-occipital region. A literature review did not reveal any prior reports of mandibular metastasis at presentation from a clival chordoma. We possibly report the first case of such an unusual clinical scenario in a 7-year-old male child and further discuss the evaluation and management of these rare tumors

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 unique case of eyelid metastasis from chondroid chordoma

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.

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.

A Case of Sacrococcygeal Chordoma / 대한피부과학회지

No abstract available.

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.

Dedifferentiated chordoma of sacrococcygeal region: a clinicopathologic analysis and review of literature / 中华病理学杂志

Objective@#To study the clinicopathologic features of dedifferentiated chordoma.@*Methods@#Four cases of dedifferentiated chordoma of sacrococcygeal region were collected at Beijing Jishuitan Hospital, from 2009 to 2014. HE and immunohistochemistry (EnVision method) were used to observe the clinical, radiological and histological features of dedifferentiated chordoma and to make the diagnosis and differential diagnosis. The literature was reviewed.@*Results@#Four cases of dedifferentiated chordoma were all located in the sacrococcygeal region. The mean age at diagnosis was 57 years (range 49-64 years). There were 1 female and 3 males. Histologically, there were two components of conventional chordoma and dedifferentiated sarcoma with or without transitional area between them. The histology of dedifferentiated components includes undifferentiated sarcoma and fibrosarcoma. Immunohistochemical study showed that cytokeratin, epithelial membrane antigen, S-100, Brachyury were positive in the portion of chordoma while p53 were positive in the portion of undifferentiated sarcoma. INI1 and vimentin were both positive.@*Conclusions@#The dedifferentiated chordoma is very rare. The diagnosis should combine the histological characters and immunohistochemical results and should be differentiated from the sarcomatoid chordoma, poorly differentiated chordoma and other high grade sarcoma. p53 may play a role in the malignant transformation mechanism of chordoma.

Preliminary study of pencil beam scanning proton and carbon ion therapy for chordoma and chondrosarcoma of head and neck / 中华放射肿瘤学杂志

Objective To evaluate the short-term efficacy and adverse events of pencil beam scanning proton and carbon ion therapy in the treatment of chordoma and chondrosarcoma of the head and neck.Methods Between July 2014 and July 31,2017,61 patients with chordoma and chondrosarcoma of the head and neck receiving proton and heavy ion therapy as the first course of radiotherapy were enrolled.Among them,45 patients were diagnosed with chordoma and 16 cases of chondrosarcoma,39 male and 22 female.The median age was 38 years old (range:14-70 years).The median maximum tumor diameter was 4.1 cm (range:0-8.6 cm).The clivus and the cervical spine were the primary tumor sites.Results Eight patients received proton therapy,21 patients were treated with proton combined with carbon ion therapy and 32 patients received carbon ion therapy.All patients successfully completed the planned radiotherapy.The medial follow-up time was 21 months (range:7-47 months).No grade 3-4 acute toxicity was observed.Only one patient suffered from radiation-induced temporal lobe injury.The 2-year progression-free survival (PFS)and overall survival (OS) were 91％ and 100％.Conclusions Pencil beam scanning proton and heavy ion therapy yields relatively favorable short-term outcomes in the treatment of chordoma and chondrosarcoma of the head and neck.Nevertheless,the long-term clinical efficacy and safety remain to be investigated during follow-up.

Consideraciones histopatológicas del tumor óseo cordoma

El Cordoma es una neoplasia maligna del tejido óseo, cuya localización es en la columna vertebral o en la base del cráneo de los seres humanos. Sus células recuerdan a las de la notocorda. Se postula que tiene su origen en restos notocordales, sin embargo la edad más frecuente de aparición es después de los 40 años en el sexo masculino. Los sitios de predilección son el sacro y la base del cráneo. En los periodos finales de su aparición existen trastornos de la micción, defecación y movilidad de los miembros inferiores, y trastornos visuales cuando su localización es en la base del cráneo. El tratamiento quirúrgico radical no es muy alentador por su localización anatómica, de ahí lo usual que resultan las recidivas. Las metástasis son poco frecuentes y casi siempre los órganos diana son los pulmones, menos frecuente en piel y ovario

The Chordoma is a malignant neoplasm of bone tissue, whose location is in the spine or at the base of the skull of human beings. His cells remind of the notochord. It is postulated that it has its origin in Notocordales remains; however the most frequent age of appearance is after 40 years in the male sex. The sites of predilection are the sacrum and the base of the skull. In the final periods of their appearance there are disorders of urination, defecation and mobility of the lower limbs, and visual disorders when their location is at the base of the skull. Radical surgical treatment is not very encouraging because of its anatomical location, hence the usual recurrences. Metastases are rare and almost always the target organs are the lungs, less common in skin and ovary

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.

Analysis CT and MRI features of chordoma and misdiagnosis / 实用放射学杂志

Objective To explore the CT and MRI features of chordoma and analysis misdiagnosis.Methods The CT and MRI imaging data were analyzed retrospectively in 1 8 patients with chordoma.The imaging findings of the preoperative misdiagnosed lesions were analyzed and summarized.Results 1 2 cases were diagnosed correctly preoperatively,6 cases were misdiagnosed.Chordoma was located in the skull base in 9 cases,in the sacrum in 8 cases,and in cervical vertebrae in 1 case.CT and MRI showed oval or round shape lesions in 12,and irregular shape in 6.14 cases of them displayed clear boundary,4 cases displayed unclear boundary.CT showed soluble osseous bone destruction and uneven isodensity or slightly low density mass.Spot or lamellar shaped calcifications or residual substances of bone were found around or inside of the lesions in 9 cases.MRI showed equal or low signal intensity on T1 WI and high signal intensely on T2 WI.After the CT and MRI contrast enhancement,mass showed mild-to-moderate heterogeneity enhancement. Conclusion Chordoma has certain characteristics on CT and MRI imaging.The combination use of CT and MRI scans has an important value in localization quantitative and qualitative diagnosis of chordoma.