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)

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.

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.

Image-Guided Percutaneous Lipiodol-Pingyangmycin Suspension Injection Therapy for Sacral Chordoma

A 74-year-old man presented with a progressively worsening pain in sacrum and was diagnosed to have a sacral chordoma by biopsy in May, 2004. Percutaneous intratumoral injection with lipiodol-pingyangmycin suspension (LPS) was carried out under image guidance and repeated when the pain in sacrum recurred and the tumor increased. During a 6-year follow-up period, three sessions of this treatment were executed. CT imaging and Karnofsky Performance Score were used to evaluate the size of tumor and quality of life, respectively. The patient was free of pain after each procedure and had a high quality of life with a Karnofsky Performance Score above 80 points. The tumor lesion in sacral area was effectively controlled. No complications were observed. Percutaneous intratumoral injection with LPS under image guidance may be an effective and safe alternative for the patients with sacral chordoma.

Extraosseous Chordoma of Retropharyngeal Space.

Chordomas are malignant bone neoplasms that originate from embryonic notochordal remnants, and they affect the skull base and the spine1. The cervical spine chordoma extends rarely into retropharyngeal space. However, primary chordoma of retropharyngeal space with no involvement of axial skeleton is very rare. These tumours are called as extraosseous chordoma2. We present a rare case of a 36-year old male patient presenting with extraosseous retropharyngeal space chordoma with no bone involvement anywhere in the entire skeleton.

Tumores intracranianos em pacientes encaminhados para estudos por tomografia de coerência óptica como portadores de glaucoma sem hipertensão ocular: relato de dois casos / Intracranial tumors in patients referred for optical coherence tomography examination as glaucoma suspects: case report

A tomografia de coerência óptica (OCT) tem se mostrado muito útil na avaliação de pacientes com glaucoma. São relatadas duas pacientes referidas com a suspeita de glaucoma sem hipertensão para avaliação por tomografia de coerência óptica que, na verdade, eram portadoras de tumores intracranianos - um cordoma de clivo no primeiro caso e um craniofaringeoma no segundo. Os achados à tomografia de coerência óptica - diminuição difusa da espessura da camada de fibras nervosas circumdiscais desproporcionalmente acentuada nos setores nasal e temporal - levantaram a suspeita de acometimento na região do quiasma e permitiram o diagnóstico destes importantes tumores intracranianos.

Optical coherence tomography (OCT) has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.

Cordoma sacral / Sacral chordomas

Cordomas são neoplasias raras que se originam dos remanescentes da notocorda primitiva 50% dos casos têm localização sacral, sendo mais frequente ao nível de S4/S5. Nós descrevemos um caso de cordoma sacral ao nível de S1 e discutimos a apresentação clínica, achados de imagem, tratamento cirúrgico e evolução.

Chordomas are rare neoplasias originating from the remaining primitive notochord. 50% of the cases have sacral localization, being more frequent at levels S4/S5. Here we report a case of sacral chordoma at level S1 and discuss its clinical presentation, imaging findings, surgical treatment, and progress.

Giant Vertebral Notochordal Rest: Magnetic Resonance and Diffusion Weighted Imaging Findings

A giant vertebral notochordal rest is a newly described, benign entity that is easily confused with a vertebral chordoma. As microscopic notochordal rests are rarely found in adult autopsies, the finding of a macroscopic vertebral lesion is a new entity with only seven previously presented cases. We report here radiological findings, including diffusion weighted images, of a patient with a giant notochordal remnant confined to the L5 vertebra, with an emphasis on its distinction from a chordoma.

FNAC diagnosis of a pre-sacral chordoma: a case report.

Chordoma is a malignant tumour that arises from the remnants of the fetal notochord. A case of a 46 year old man presenting with a pre-sacral mass diagnosed as a chordoma on FNAC is described.

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.

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.

Chordoid Meningioma: A Case Report

The term "chordoid meningioma" means meningioma, which is pathologically similar to chordoma, and previously reported that rarely associated with microcytic anemia and/or dysgammaglobulinemia especially in pediatric population. We present a case of this rare variant, which comprises less than 0.5% of all meningiomas. A 33-yr-old man visited our hospital, complaining visual field defect worsening over 7 yr. Neurological examination showed left homonymous hemianopsia. The brain magnetic resonance imaging revealed well enhancing right temporo-occipital mass with cystic portion. Histopathologic findings of resected tumor were compatible with chordoid meningioma which included trabeculae of eosinophilic, vacuolated cells in a myxoid matrix with prominent lymphoplasmacellular infiltration. The neoplastic cells were positive for vimentin and epithelial membrane antigen and negative for glial fibrillary acidic protein and cytokeratin. This is an adult case of chordoid meningioma without anemia or dysgammaglobulinemia.

A unique presentation of retroclival chordoma.

Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.

Infrasellar craniopharyngioma mimicking a clival chordoma: a case report.

An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed.

Cordoma de ápice petroso: relato de um caso / Petrous apex chordoma: a case report

Cordomas são neoplasias raras que se originam dos remanescentes da notocorda primitiva. Estes remanescentes persistem ao longo de todo o esqueleto axial. Os cordomas intracranianos, mais freqüentemente, se localizam no clivus, próximo à sincondrose esfenooccipital, tipicamente na linha média. Nós descrevemos um caso atípico de cordoma fora da linha média, mais especificamente no ápice petrose, e discutimos as causas embriológicas que determinam esta localização, bem como sintomas, achados de imagem, tratamento cirúrgico e evolução.

Cordomas de cabeza y cuello: presentación de 10 casos / Head and neck chordomas: presentation of 10 cases

Se describen 10 casos de cordomas localizados en la región de cabeza y cuello, recopilados en el período de 1987 a 1997, cuyo tiempo de evolución fue de 41 meses (9-96 meses) y tamaño que fue valorable en cinco pacientes, variando entre 1.5 y 10cm. (6.3 cm. Promedio). La localización más frecuente fue la región del clivus (30 por ciento), seguida de la región cervical y la nasofaringe. Nueve de los casos fueron cordomas convencionales y uno cordoma condroide. Hubo estudios de inmunohistoquímica en cinco casos, que presentaron reactividad a queratina, proteína S-100 y EMA. El tratamiento fue quirúrgico con radioterapia postoperatoria. El 60 por ciento presentaron recurrencias o persistencia tumoral. El seguimiento promedio fue de 15.6 meses