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.

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.

Dedifferentiated chordoma: a case report

Sarcomatous transformation [dedifferentiated chordoma] in chordoma is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma arising from the sacrococcygeal region of a 60-year-old man, in third tumor recurrence. This tumor showed features of sarcoma with areas more typical of chordoma The chordoma-like areas expressed cytokeratin, epithelial membrane antigen and S-100 protein in all tumor cells and the spindle-cell component exhibited vimentin positivity in all of them but negative for other markers. The results showed that the sarcomatous areas as seen in the recurrent chordoma lack epithelial cell features of chordoma and suggest the possibility of altered differentiation pathway of the tumor stem cell or emergence of a new malignant cell population within the recurrent tumor

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.

Chondroid chordoma of petrous temporal bone with extensive recurrence and pulmonary metastases.

Chondroid chordoma is a variant of chordoma; it may rarely involve the petrous temporal bone and has a high propensity for recurrence. Chordoma rarely metastasizes but it is, nevertheless, associated with a poor outcome. We report a rare case of chondroid chordoma with extensive recurrence and pulmonary metastases.

The outcome of surgical treatment for tumors of the craniocervical junction.

OBJECTIVE: The authors report the clinical, radiological, and surgicalfindings ofpatients with craniocervical junction tumors surgically treated in the institution over the last 8 years. MATERIAL AND METHOD: A retrospective study was performed. Clinical, radiological, and operative data were evaluated, and follow-up information was obtained from outpatient examinations, and telephone interviews. RESULTS: There were 25 patients consisting of nine chordomas, eight meningiomas, three cysts, two schwannomas, one each of aneurysmal bone cyst, plasmacytoma, and metastasis. Twenty-nine operative procedures were performed, classified as 12 anterior nine posterior-lateral, and eight posterior approaches. Gross total removal was achieved in 17 cases, subtotal removal in six cases, and partial removal in two cases. Re-operation was performed in six cases. Median follow-up time was 31 months. The authors found significant improvement in Karnofsky Performance Scale scores. CONCLUSION: Appropriate surgical approaches provide successful tumor removal with less surgical morbidities, nevertheless recurrent tumors occasionally occur and so, long-term follow-up is mandatory.

Parachordoma of tibia--a case report.

A rare case of a 24-year old male patient with 'parachordoma' of left tibia having the characteristic histopathological and histochemical features of chordoma occurring in an unconventional site, which was clinically and radiologically diagnosed as osteoclastoma is presented here. The case is presented considering the extreme rarity of the lesion in literature in long bones.

Dedifferentiated chordoma--a case report.

A case of a recurrent sacro-coccygeal chordoma is reported which histologically showed an admixture of a conventional chordoma and a pleomorphic sarcoma. These tumours are called dedifferentiated chordomas and carry an ominous prognosis.

Sacral chordoma--a case report.

Chordoma, a rare malignant tumour of early adulthood, rarely presents in children. We report such a case of rare malignant tumour which was diagnosed in the first decade of life.

Sacrococcygeal chordoma in a 9-year-old boy

Os autores apresentam um caso de cordoma sacroccígeo em um menino de 9 anos de idade. O paciente foi admitido no hospital com história de dor na regiäo sacral e nos membros inferiores com dois anos de evoluçäo, piorando nas últimas quatro semanas. O exame físico revelou atrofia muscular moderada em ambos os membros inferiores, diminuiçäo do reflexo patelar e presença do sinal de Lasègue à esquerda. Os exames de imagem da regiäo lombar mostraram um processo expansivo na regiäo sacrococcígea com erosäo parcial do coccix e focos de calcificaçäo, sem evidência de metástases para linfonodos regionais. Foi realizada biópsia diagnóstica que mostrou neoplasia formada por cordöes e ninhos de células de citoplasma amplo, por vezes vacuolado, com núcleos moderadamente pleomórficos com cromatina grumosa. O estudo imunohistoquímico revelou positividade para CK, proteína S-100, CEA, vimetina e EMA. Cordomas säo tumores raros que representam em torno de 2 por cento de todas as neoplasias malignas do osso. Os locais de maior acometimento säo as extremidades da coluna espinhal e säo incomuns nas primeiras duas décadas de vida, especialmente na regiäo sacrococcígea

Cordomas: estudo anátomo-clínico de 8 casos / Chordomas: anatomo-clinical study of 8 cases

Foram estudados e revistos 8 casos de pacientes com cordomas, diagnosticados no período compreendido entre janeiro de 1953 e dezembro de 1985, sendo 6 de localizaçäo intracraniana, 1 de origem sacrococcígea provável e 1 cervical. Analisaram-se dados clínicos de exame físico e neurológico, bem como métodos diagnósticos. Ressaltam-se a raridade do tumor, o predomínio deste em pacientes do sexo feminino e em território intracraniano (estes dois últimos dados conflitantes com os da literatura) e seu acometimento na faixa etária de 30 anos (idade média de 31 anos). Os cordomas apresentam sintomatologia diversa, dependente de sua localizaçäo; apesar de possuirem caracteres histológicos benignos, a sobrevida em 5 anos é baixa, por causa do seu comportamento infiltrativo, da ocorrência de recidivas após tratamento radioterápico e/ou cirúrgico e de eventuais metástases

Cordoma: a propósito de dos casos / Chordoma: apropos of 2 cases

Se describen dos observaciones de cordoma de la región sacrocoxígea (un varón y una mujer). Los estudios realizados, incluidos T.C., fueron completos; en un caso se efectuó la necropsia. Se hace referencia a la historia del tumor, su clínica más común y su tratamiento paliativo