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[A rare variant of chondrosarcoma--mesenchymal chondrosarcoma of the scapula].

A rarely occurring tumor, mesenchymal chondrosarcoma of the shoulder blade, is described. The tumor, found in a 19-year-old male, consisted of two components; a typical chondrosarcoma showing a moderate degree of anaplasia, and a tumor tissue composed of poorly differentiated cells with large numbers of thin-walled vessels. The two tissues were separated from each other by connective-tissue elements, though cells of the second component were seen in places to pass into the tissue of hyalin cartilage. This variant of chondrosarcoma proved to be highly sensitive to chemotherapy, allowing considerable prolongation of the patient's survival.

Adult↗

Cell differentiation and matrix gene expression in mesenchymal chondrosarcomas.

Mesenchymal chondrosarcomas are small-cell malignancies named as chondrosarcomas due to the focal appearance of cartilage islands. In this study, the use of in situ detection techniques on a large series of mesenchymal chondrosarcoma specimens allowed the identification of tumor-cell differentiation pathways in these neoplasms. We were able to trace all steps of chondrogenesis within mesenchymal chondrosarcoma by using characteristic marker genes of chondrocytic development. Starting from undifferentiated cells, which were negative for vimentin and any other mesenchymal marker, a substantial portion of the cellular (undifferentiated) tumor areas showed a chondroprogenitor phenotype with an onset of expression of vimentin and collagen type IIA. Cells in the chondroid areas showed the full expression panel of mature chondrocytes including type X collagen indicating focal hypertrophic differentiation of the neoplastic chondrocytes. Finally, evidence was found for transdifferentiation of the neoplastic chondrocytes to osteoblast-like cells in areas of neoplastic bone formation. These results establish mesenchymal chondrosarcoma as the very neoplasm of differentiating premesenchymal chondroprogenitor cells. The potential of neoplastic bone formation in mesenchymal chondrosarcoma introduces a new concept of neoplastic (chondrocytic) osteogenesis in musculoskeletal malignant neoplasms, which qualifies the old dogma that neoplastic bone/osteoid formation automatically implies the diagnosis of osteosarcoma.

Apoptosis↗

Trisomy 8 as the sole cytogenetic abnormality in a case of extraskeletal mesenchymal chondrosarcoma.

Mesenchymal chondrosarcoma is a rare malignant tumor that comprises about 3-10% of all sarcomas. Reports of cytogenetic studies of mesenchymal chondrosarcoma are limited and no consistent cytogenetic abnormality has surfaced. Some mesenchymal chondrosarcomas have a t(11;22) translocation suggesting a relationship with the PNET/Ewing tumor family. We report what to our knowledge is the first case of trisomy 8 as the sole cytogenetic abnormality in a mesenchymal chondrosarcoma.

Child↗

Overexpression of p53 and rare genetic mutation in mesenchymal chondrosarcoma.

Mesenchymal chondrosarcoma is extremely rare and accounts for less than 2% of all chondrosarcomas. The pathogenesis and the molecular genetic events which contribute to the development of mesenchymal chondrosarcoma are not well elucidated, due in part to the lack of sufficient tumor tissue available. To characterize the involvement of the p53 gene abnormality in this disease, we analyzed expression and sequence alteration of p53 by immunohistochemical analysis of the protein expression and quantitative DNA/PCR and PCR-SSCP assays of the gene in 33 paraffin-embedded tissue specimens. Immunohistochemical analysis demonstrated that 19 (61.3%) of 31 had nuclear overexpression of p53 while 7 (22.6%) showed cytoplasmic expression. The remaining 5 (16.1%) were negative for p53 staining. The nuclear positivity of p53 was observed within a range of 22-64% (mean 37.3%) of tumor cells and showed a positive staining in mesenchymal components as well as chondroid components. Quantitative DNA/PCR analysis revealed that 6 (18.2%) of the 33 specimens carried significantly reduced or undetectably low levels of p53 indicating the genomic deletion of the gene in these tumors. In contrast, however, DNA/PCR-SSCP analysis failed to detect any types of mutations resulting in amino acid substitution within exons 5-9 regions of the gene. Taken together, our data suggests that genetic alteration of p53 is a relatively rare event in mesenchymal chondrosarcomas but substantial fraction of this type of tumors carries abnormal overexpression of p53, which might result from as yet unidentified epigenetic mechanism(s).

Chondrosarcoma, Mesenchymal↗

[Neoplastic chondroneogenesis as a characteristic of mesenchymal chondrosarcomas].

Mesenchymal chondrosarcomas are rare skeletal malignancies, which are typically characterized by tumor compartmentation. One tumor area is formed by small, undifferentiated neoplastic cells, whereas the second compartment is composed of cartilaginous areas. In this study, the application of in situ detection techniques enabled us to characterize the different tumor compartments according to their cellular differentiation patterns. The use of characteristic marker genes identified all steps of chondrogenesis within the different tumor compartments. Undifferentiated tumor cells in the small-cell areas were negative for vimentin and the cytoprotein S-100, whereas other tumor cells expressed collagen type IIA and vimentin indicating a chondroprogenitor cellular phenotype in these small-cell areas. Fully differentiated chondrocytic cells expressing collagen type II were found in the chondroid areas. The focal expression of type X collagen indicated hypertrophic differentiation of the neoplastic chondrocytes. The results characterize mesenchymal chondrosarcomas as skeletal malignancies that arise from undifferentiated chondroprogenitor cells and have the potential to undergo all steps of chondrocytic differentiation.

Biomarkers, Tumor↗

Extraskeletal mesenchymal chondrosarcoma.

Extraskeletal mesenchymal chondrosarcoma is a rare malignancy. It is characteriged by a bimorphic histologic pattern, with a mesenchymal tissue mixed with malignant hyaline cartilage. It also has high incidence of local recurrence and distant metastasis. All cases have been reported fatal associated with this tumor in spite of complete surgical excision. A 35-year-old female presented with a palpable mass about 5 x 3 x 2.5 cm3 in size over her left forearm and was reported to be an extraskeletal mesenchymal chondrosarcoma. Marginal resection followed by regional radiotherapy was done. No local recurrence or distant metastasis was found two years after surgery. Thorough physical examination, series radiographic studies, and multiple sites of tumor biopsies before radical resection of the tumor may decrease the misdiagnostic rate for extraskeletal mesenchymal chondrosarcoma. Appropriate tumor treatment, close patient follow-up and timely treatment for local recurrence or distant metastasis may increase the survival rate.

Adult↗

Intracranial extra-skeletal mesenchymal chondrosarcoma.

Intracranial Mesenchymal Chondrosarcoma is a very rare and uncommon entity that affects young adults. We came across one such patient who presented with severe headache and intermittent nausea and vomiting. The clinical, radiological preoperative diagnosis was a meningioma, on histological examination it turned out to be mesenchymal chondrosarcoma of tentorial region in posterior fossa, uncommon site for this entity.

Adult↗

[Mesenchymal chondrosarcoma].

Five mesenchymal chondrosarcomas of the bones, soft tissues and the orbit were subjected to histological and electronmicroscopic examination. In all instances the authors found in addition to the differentiated cartilaginous component extensive areas formed by round or spindle-shaped non-differentiated mesenchymal elements which on electron microscopic examination had a striking resemblance with Ewing's Sarcoma cells or resembled fibroblasts. The vascular portions present in all examined tumours reminded of the structure of a haemangiopericytoma. Osteoplasia found in soft tissue tumours had a non-tumourous character.

Adult↗

Primary mesenchymal chondrosarcoma of the lung.

Mesenchymal chondrosarcoma has been well documented in the somatic soft tissue and bone. It is a rare subtype of chondrosarcoma characterized by the presence of islands of chondroid or by less osteoid tissue enmeshed within dense sheets of primitive small blue mesenchymal cells with hemangiopericytoma-like vessels, or by both. The vast majority of previously published pulmonary mesenchymal chondrosarcoma was metastatic. To the best of our knowledge, only one case of primary pulmonary mesenchymal chondrosarcoma has been described in the literature. Herein, we report the second case of primary mesenchymal chondrosarcoma of the lung and emphasize that biopsy may yield only nonspecific small blue cells, whereas a detailed evaluation of the resected specimen allows definite diagnosis of this rare lung tumor.

Adult↗

Mesenchymal chondrosarcoma. An immunohistochemical study.

Mesenchymal chondrosarcoma is an uncommon small-cell neoplasm of bone and soft tissue, the chondrogenic nature of which has been generally accepted. However, the phenotypic attributes of the small-cell population in this neoplasm have not been well characterized, and its relationship to "precartilage mesenchyme" remains unclear. In an attempt to address this issue, we performed an immunohistochemical analysis of nine cases, using antibodies to vimentin, S100 protein, Leu-7 antigen, neuron-specific enolase, synaptophysin, desmin, muscle-specific actin, cytokeratin, and epithelial membrane antigen, and the avidin-biotin-peroxidase complex (ABC) method. The small cells of mesenchymal chondrosarcoma failed to express S100 protein, whereas all components of the tumors (small cells, lacunar chondroblasts, and chondroid matrix) stained for Leu-7 antigen in six cases. Neuron-specific enolase was identified in the small cells of four cases and in the lacunar cells of seven. None contained desmin, actin, cytokeratin, epithelial membrane antigen, or synaptophysin. The immunophenotype of mesenchymal chondrosarcoma resembled that of embryonic cartilage and thus did not contradict the premise that this tumor was the neoplastic counterpart of fetal chondroid tissues. However, immunohistologic studies are not overly helpful in the differential diagnosis between mesenchymal chondrosarcoma and other small round cell lesions.

Adolescent↗

Translocation der(13;21)(q10;q10) in skeletal and extraskeletal mesenchymal chondrosarcoma.

Cytogenetic studies of mesenchymal chondrosarcoma are few and to date, no specific or recurrent aberrations have been found. In this investigation, the cytogenetic and molecular cytogenetic (spectral karyotypic and fluorescence in situ hybridization) findings for two mesenchymal chondrosarcomas, one arising skeletally and the other extraskeletally, are reported. An identical Robertsonian translocation involving chromosomes 13 and 21 [der(13;21)(q10;q10)] was detected in both cases, possibly representing a characteristic rearrangement for this histopathologic entity. Both cases also exhibited loss of all or a portion of chromosomes 8 and 20 and gain of all or a portion of chromosome 12. The observation of similar chromosomal abnormalities in both skeletal and extraskeletal mesenchymal chondrosarcoma supports a genetic as well as histopathologic relationship between these anatomically distinct neoplasms.

Adult↗

Central nervous system mesenchymal chondrosarcoma.

Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival.

Adolescent↗

[Mesenchymal chondrosarcoma of the maxilla].

Mesenchymal chondrosarcoma is a variant that shows some peculiarities in comparison with classical chondrosarcomas. On the basis of a case report on a young man with a mesenchymal chondrosarcoma of the maxilla, the clinical symptomatology and diagnostics, morphological findings and actual therapy are discussed. In addition, problems of differential diagnosis are also discussed.

Adult↗

Mesenchymal chondrosarcoma of the maxilla.

Mesenchymal chondrosarcoma (MC) is a rare tumour, with a predilection for the head and neck region. We describe a case of mesenchymal chondrosarcoma arising in the right maxilla extending to the basi-sphenoid. Its computed tomography (CT) and magnetic resonance imaging (MRI) and histopathological features and the management are presented. We also reviewed the literature of reported cases involving the maxilla.

Adolescent↗

Mesenchymal chondrosarcoma of the cerebellum.

Mesenchymal chondrosarcoma is a rare malignant neoplasm of bone and soft tissues. An unique case is described of an 8-year-old child with a midline cerebellar lesion. Pertinent clinical and radiologic findings along with histopathologic features are described. To our knowledge, this is the first case of mesenchymal chondrosarcoma arising in the cerebellar parenchyma of a child.

Cerebellar Neoplasms↗

Ultrastructural study of conventional chondrosarcomas and myxoid- and mesenchymal-chondrosarcomas.

Five cases of conventional chondrosarcomas (CS.) of graded malignancy, 3 cases of myxoid CS. and 2 cases of mesenchymal CS. were studied by electron-microscopy. The chondrocyte like tumor cells of conventional CS. were characterized by: an ovoid shape, eccentric nucleus, abundant endoplasmic reticulum with dilated cisternae of RER; cytoplasmic glycogen, lipid droplets, and filaments plus numerous thin cytoplasmic projections. The histologically high grade tumors showed fewer cytoplasmic organelles, bizarre nuclei and more prominent nucleoli than the better differentiated ones. The tumor cells of myxoid CS. were chiefly fusiform. The cells frequently presented a pattern of rows with good cellular cohesion, and scanty cytoplasmic projections. The most prominent cytoplasmic feature was a conspicuous RER. Abundant cytoplasmic filaments and cytoplasmic glycogen were also observed. The undifferentiated areas of the mesenchymal CS. showed primitive mesenchymal cells with rounded nuclei, and scanty cytoplasm which was poor in organelles and glycogen. The cytoplasmic membranes were very cohesive and cytoplasmic projections were not present. Scanty cytoplasmic filaments and conspicuous desmosome like junctions were observed. The intercellular matrix of conventional and myxoid CS. consisted of fibrils, glycosaminoglycan granules and collagen fibers. In the undifferentiated zones of the mesenchymal CS. the intercellular matrix was very scanty and did not contain collagen fibrils. The more immature cells correspond to the small undifferentiated cells of mesenchymal chondrosarcoma.

Adult↗

[Mesenchymal chondrosarcoma of the radius with metastases in the internal organs].

A rare variant of chondrosarcoma--a mesenchymal chondrosarcoma of the right radiocarpal articulation--is described. The tumour was very small, painless, and did not disturb the joint function but possessed a high mitotic activity and gave lymphogenic metastases to the viscera of the right half of the body. It was not diagnosed clinically.

Adult↗

Establishment of clonal cell lines, with or without cartilage phenotypes, from a hamster mesenchymal chondrosarcoma.

A hamster mesenchymal chondrosarcoma was found in the soft tissues of the cheek pouch and has been successfully transplanted. The tumor was composed principally of two cell types: poorly differentiated mesenchymal cells and focal areas of cells in islands showing cartilaginous differentiation. The clonal cell lines, MCS-1 and MCS-8 which closely correspond to the respective cell types in the original tumor were also established. MCS-1 cells formed an undifferentiated sarcoma in the subcutaneous layer of nude mice and MCS-8 cells formed a chondrosarcoma of a common type.

Animals↗