Recurrence of clival chordoma along the surgical pathway.
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Chordomas are locally aggressive malignant tumors of notochordal origin whose metastatic potential is increasingly recognized. Surgical pathway recurrence has been noted only rarely in the literature. We present three patients with clival chordomas whose sole or initial recurrence was along the pathway of prior surgical access. A characteristic mass found along the pathway of prior surgical access for resection of a chordoma should suggest recurrent chordoma. (+info)
Sacral chordoma--a case report.
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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. (+info)
Isolated cardiac metastasis from sacral chordoma.
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A 64-year-old woman presented with right heart failure caused by a cardiac tumor centered in the free wall of the right ventricle, accompanied by pericardial effusion. A match between the biopsy specimen and tissue removed 4 years earlier resulted in the diagnosis of a cardiac metastasis from a chordoma. Immunohistochemical staining was also useful in establishing the diagnosis. To alleviate the right ventricular outflow obstruction, a palliative operation was planned, resecting the tumor and performing a right ventriculoplasty, which was cancelled due to the extent of infiltration of the tumor, and instead a right atrium to pulmonary artery shunt was attempted using a vascular prosthesis, only to fail due to an inability to maintain blood flow through the prosthesis. Presently there are no definitive treatment options available, and some palliative chemotherapy is being performed. Single cardiac metastases from a chordoma are extremely rare. (+info)
Chordoid glioma: a neoplasm unique to the hypothalamus and anterior third ventricle.
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BACKGROUND AND PURPOSE: Chordoid glioma is a new clinicopathologic entity that occurs in the region of the hypothalamus/anterior third ventricle. The aims of this study were to describe the characteristic radiographic features of chordoid glioma, identify specific imaging features that may enable differentiation of chordoid glioma from other suprasellar tumors, and increase neuroradiologists' awareness of this newly described tumor, facilitating prospective diagnosis. METHODS: CT scans and/or MR images of six patients with chordoid glioma were reviewed retrospectively to determine whether any characteristic radiographic features would emerge. Reports of the clinical presentation, pathologic findings, and radiographic findings of another six patients were reviewed and included, for a total patient population of 12 (mean age +/- SD, 46 +/- 13 years). RESULTS: Imaging features were strikingly similar for all tumors. In each case, the mass was ovoid, was well circumscribed, was located in the region of the hypothalamus/anterior third ventricle, and enhanced uniformly and intensely. Tumors were hyperdense to gray matter on CT scans and were isointense on T1-weighted MR images and slightly hyperintense on long-TR MR images. In two patients, vasogenic edema extended into the optic tracts, and in three, there was hydrocephalus. CONCLUSION: Chordoid glioma is a recently described unique histopathologic entity that has been added to the World Health Organization glioma classification scheme and must be included in the differential diagnosis of a suprasellar mass. Distinctive imaging features are its location, ovoid shape, hyperdensity on CT scans, and uniform intense contrast enhancement. (+info)
Matrix gene expression analysis and cellular phenotyping in chordoma reveals focal differentiation pattern of neoplastic cells mimicking nucleus pulposus development.
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Chordoma is the fourth most common malignant primary neoplasm of the skeleton and almost the only one showing a real epithelial phenotype. Besides classic chordoma, so-called chondroid chordoma was described as a specific entity showing cartilage-like tissue within chordomatoid structures. However, since its first description, strongly conflicting results have been reported about the existence of chondroid chordoma and several studies suggested chondroid chordomas being in fact low-grade conventional chondrosarcomas. In the present study, we used cytoprotein expression profiling and molecular in situ localization techniques of marker gene products indicative of developmental phenotypes of chondrocytes to elucidate origin and biology of chondroid chordoma. We were able to demonstrate the chondrogenic potential of chordomas irrespectively of the appearance of overt cartilage formation by identifying the multifocal expression of type II collagen, the main marker of chondrocytic differentiation. Additionally, the cartilage-typical large aggregating proteoglycan aggrecan was present throughout all chordomas and, thus, a very characteristic gene product and marker of these neoplasms. Biochemical matrix composition and cell differentiation pattern analysis showed a high resemblance of classic chordomas and in chordoid areas of chondroid chordomas to the fetal chorda dorsalis, whereas chondroid areas of chondroid chordomas showed features similar to adult nucleus pulposus. This demonstrates on the cell function level the chondrocytic differentiation potential of neoplastic chordoid cells as a characteristic facet of chordomas, mimicking fetal vertebral development, ie, the transition of the chorda dorsalis to the nucleus pulposus. Our study firmly establishes a focal real chondrocytic phenotype of neoplastic cells in chordomas. Chondroid chordoma is neither a low-grade chondrosarcoma nor a misnomer as discussed previously. (+info)
Familial chordoma, a tumor of notochordal remnants, is linked to chromosome 7q33.
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Chordoma is a rare tumor originating from notochordal remnants that is usually diagnosed during midlife. We performed a genomewide analysis for linkage in a family with 10 individuals affected by chordoma. The maximum two-point LOD score based on only the affected individuals was 2.21, at recombination fraction 0, at marker D7S2195 on chromosome 7q. Combined analysis of additional members of this family (11 affected individuals) and of two unrelated families (one with 2 affected individuals and the other with 3 affected individuals), with 20 markers on 7q, showed a maximum two-point LOD score of 4.05 at marker D7S500. Multipoint analysis based on only the affected individuals gave a maximum LOD score of 4.78, with an approximate 2-LOD support interval from marker D7S512 to marker D7S684. Haplotype analysis of the three families showed a minimal disease-gene region from D7S512 to D7S684, a distance of 11.1 cM and approximately 7.1 Mb. No loss of heterozygosity was found at markers D7S1804, D7S1824, and D7S2195 in four tumor samples from affected family members. These results map a locus for familial chordoma to 7q33. Further analysis of this region, to identify this gene, is ongoing. (+info)
Tentorial enhancement on MR images is a sign of cavernous sinus involvement in patients with sellar tumors.
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BACKGROUND AND PURPOSE: This study was undertaken to analyze enhancement patterns of the dura around sellar tumors and to compare the results with tumor invasion or compression of the cavernous sinuses. Postoperative enhancement patterns on MR images were compared with preoperative findings. METHODS: Contrast-enhanced coronal and sagittal MR images were examined prospectively in 96 patients with sellar tumors (65 macroadenomas, 15 microadenomas, 14 Rathke cleft cysts, and two chordomas at the sella). All patients underwent surgical treatment, and pre- and postsurgical features on MR images were compared. RESULTS: Presurgical MR images showed dural enhancement in 36.5% of the patients: asymmetric tentorial enhancement in 24 patients, symmetric tentorial enhancement in seven, and sphenoidal ridge or clivus enhancement in four. Asymmetric tentorial enhancement disappeared after surgical decompression in seven patients. For evaluation of cavernous sinus invasion ipsilateral to the enhancement, sensitivity and specificity of the asymmetric tentorial enhancement sign were 81.3% and 86.3%, respectively. Sensitivity and specificity of the sign were 42.9% and 93.6% for cavernous sinus involvement, including compression and invasion. CONCLUSION: Asymmetric tentorial enhancement is a useful sign in the diagnosis of invasion or severe compression of the cavernous sinus by sellar tumor. The sign may represent venous congestion or collateral flow in the tentorium due to obstructed flow in the medial portion of the cavernous sinus. (+info)
Vertebral reconstruction with cortical allograft: long-term evaluation.
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Reconstruction of large anterior vertebral column defects is indicated in a number of pathological conditions including tumor, infection, trauma and post-traumatic deformity. Several substitutes and techniques are available for the functional restoration of the vertebral column. Vascularized bone transfers, autografts, allografts or xenografts have been used, as well as metal or ceramic implants. All of these bear potential advantages and drawbacks in terms of associated morbidity of graft harvesting, disease transmission, mechanical failure, implant incorporation and overall long-term clinical outcome. In the present paper we report our experience with the use of freeze-dried, gamma-irradiated, cortical allograft for the reconstruction of large, anterior segmental defects of the spine, involving at least one vertebral body with its two adjacent discs. Cortical allografts were used in 67 cases operated for a variety of conditions. No case of disease transmission, infection or long-term mechanical graft failure occurred in our entire series, with a mean follow-up of 31 months. Fusion and mechanical stability was reliably obtained. Specific advantages include the absence of donor site morbidity, the possibilities for exact trimming to the size of the defect, superior mechanical strength as compared to available autograft, and reliable fusion with the host bone with partial bone remodeling, preventing fatigue failure. We conclude that freeze-dried, irradiated cortical allografts are safe and effective for anterior reconstruction of the spine. (+info)