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Establishment and characterization of the permanent human cell line C3842 derived from a secondary chondrosarcoma in Ollier's disease.

The permanent human cell line C3842 was established from a secondary chondrosarcoma in a typical case of Ollier's disease. In the present study, we analyzed the morphological, cytogenetic and molecular biological characteristics of the cultured cells in comparison with the original tumor and investigated the invasion properties of the tumor model using functional imaging of proteolysis, matrigel assay and chick chorioallantoic membrane assay. C3842 cells exhibit the typical features of malignant cartilage tumor cells in vitro, including the expression of collagen types II, IX, XI and aggrecan. The proteolytic ability of C3842 cells is attributed to the expression of several proteases, such as cathepsin B, urokinase plasminogen activator and matrix-metalloproteinase-2, which enable the cells to degrade collagen type I and to permeate matrigel matrix. In accordance with the biological features in vivo, C3842 cells are not able to invade through the epithelium of the chick chorioallantoic membrane. In conclusion, the cell line C3842 provides the first model of a secondary chondrosarcoma in Ollier's disease in vitro, which is characterized by distinct features of such malignant cartilage tumors.

Adult↗

Mutation of p53 with loss of heterozygosity in the osteosarcomatous component of a dedifferentiated chondrosarcoma.

We investigated a dedifferentiated chondrosarcoma of a 61-year-old woman with an osteosarcomatous high-grade component for p53 alteration. The low-grade cartilaginous and the high-grade osteosarcomatous components of the tumor were macrodissected and evaluated separately by immunohistochemistry and molecular biology. We used PCR-SSCP analysis and direct sequencing to screen exons 4-8 for p53 mutations. The p53 intron 1-polymorphism was investigated for loss of heterozygosity. A functionally relevant p53 missense mutation in codon 193 of exon 6 (A-to-T transversion) with loss of wild-type allele was detected only in the dedifferentiated component. Using the monoclonal antibody DO-1, immunohistochemistry failed to show p53 overexpression. This evidence of p53 mutation may be regarded as at least a co-factor that "switched" the preexisting low-grade conventional chondrosarcoma to a highly malignant dedifferentiated tumor.

Chemotherapy, Adjuvant↗

Extraskeletal myxoid chondrosarcoma with multiple skeletal metastases.

Pulmonary metastases are not unusual in extraskeletal myxoid chondrosarcoma; however, only two patients have been reported with multiple bony metastases. We report here one patient with extraskeletal myxoid chondrosarcoma associated with lung and multiple bony metastases. After chemotherapy, the primary lesion was resected, but lung and multiple bony metastases were found 20 months later. The bony metastases were in the right femur, right humerus, and at multiple vertebral levels. Because of a pathologic fracture of the right femur, the metastases in the right femur and right humerus were surgically stabilized. After chemotherapy, the lung metastases were resected, and those in the vertebral bodies were treated with radiotherapy.

Bone Neoplasms↗

Electrochemotherapy for digital chondrosarcoma.

Electrochemotherapy (ECT) delivers nonpermeable anticancer drugs to cell interiors by temporally increasing the permeability of the cytoplasmic membrane under locally applied pulsating electrical stimuli. This treatment results in consistent and enhanced pharmacological effects of drugs on the targeted tissue. ECT has been used for surface skin cancer but never for musculoskeletal tumors. This report describes a clinical trial of ECT for digital chondrosarcoma. A 74-year-old woman with a digital chondrosarcoma was administered electric stimulation with two surface electrodes 10 min after intratumoral multiple injection of bleomycin sulfate and 15 s after intraarterial perfusion of bleomycin sulfate. Biopsy performed after ECT showed 90% tumor necrosis. Marginal resection of the tumor was followed by autologous bone grafting to fill the bone defect. Although the follow-up period was short (3 years), the patient remained disease-free after ECT and was satisfied that amputation of the affected finger could be avoided. This preliminary result suggests that ECT is a viable modality for limb-preserving treatment of patients with sarcoma of the extremities.

Aged↗

Effect of dexamethasone on growth inhibition and chondrogenic maturation of human chondrosarcoma.

The effect of dexamethasone, a synthetic glucocorticoid, on in vitro and in vivo growth and differentiation of the human chondrosarcoma cell line (OUMS-27) was studied. Cells were treated with various doses of dexamethasone, and increasing doses produced an inhibitory effect on OUMS-27 tumor cell proliferation and induced maturation. Cell counts for OUMS-27 on day 9 ranged from 59% of the control at 10(-8) M to 45% of the control at 10(-5) M dexamethasone. Northern blot analysis revealed that the type II collagen mRNA level in cells given dexamethasone was lower than that in the controls, and the type X collagen mRNA level was higher than that in the controls. Phase-contrast microscopy revealed that cells grown in control medium formed monolayers consisting of small, polygonal cells, whereas dexamethasone-treated cells became larger and more irregular in shape. In the in vivo study the growth rate of masses in nude mice induced by inoculating OUMS-27 cells was also reduced in a dose-dependent manner with dexamethasone administration. These results suggest that dexamethasone caused growth inhibition and induced chondrogenic maturation of human chondrosarcoma cells.

Animals↗

Intra-parenchymal mesenchymal chondrosarcoma of the cerebellum: case report and review of the literature.

A 44-year-old male presented with a 3-week history of clumsiness and numbness of the left hemibody. CT scan and MRI revealed a 2 cm mass in the right hemisphere of the cerebellum. The patient underwent a sub-occipital craniotomy with gross total resection of the intra-parenchymal lesion. On pathology, the lesion was found to be compatible with a mesenchymal chondrosarcoma. The patient received adjuvant radiation treatment and remains free of disease 60 months after completion of treatment. Mesenchymal chondrosarcomas are neoplasms that rarely arise intra-cranially. Thirty cases have been found in the literature. Our case resembles more closely six of these cases because the tumour had no dural attachment. We describe our case in more detail and review similar cases found in the English literature.

Adult↗

Chondrosarcoma of the rib.

A 73-year-old woman evidencing an abnormal shadow on chest X-ray film since 1993 was admitted after a tumor of the left 8th rib was diagnosed in 2001. Computed tomography showed a low-density mass with coarse calcification arising from the left 8th rib and protruding into the abdominal cavity. The tumor was diagnosed as low-grade chondrosarcoma by incision biopsy and was resected together with the left 7th, 8th, and 9th ribs. The chest wall was reconstructed using Marlex mesh. Histological findings were compatible with Grade I chondrosarcoma. The patient had a long clinical course without distant metastasis thanks to the tumor's low malignancy.

Aged↗

Pleural chondrosarcoma.

Thoracic extraskeletal chondrosarcomas are uncommon tumors. A case of primary chondrosarcoma of the pleura is presented. Complete surgical resection without adjuvant therapy, even in the presence of pleural seeding, appears to offer a favorable long-term prognosis.

Aged↗

Insulin stimulates secretion of a collagenase inhibitor by Swarm rat chondrosarcoma chondrocytes.

Swarm rat chondrosarcoma chondrocytes produce an inhibitor of collagenase similar to that found in bovine articular chondrocytes and extracts of bovine scapular cartilage. These cells synthesize normal levels of cartilage type proteoglycans when cultured in serum free medium with insulin. Collagen synthesis is also increased when insulin is added to chondrosarcoma chondrocytes. We have demonstrated that insulin stimulates collagenase inhibitor production by these chondrocytes. Enhancement of inhibitory activity occurs over the range of 10 to 1000 ng/ml. A 3.2 fold stimulation was observed at a concentration of 1 microgram/ml. There was a lag period of 24 to 48 hours before the insulin effect became evident. Latent or active collagenase was not detectable under these conditions. These results suggest that the hormone insulin controls the levels of collagen in this tumor by stimulating synthesis of collagen and inhibitors of collagenase.

Animals↗

Isolation and characterization of proteoglycans and glycosaminoglycans from human chondrosarcoma.

The proteoglycans and glycosaminoglycans of four human chondrosarcomas with different degrees of malignancy (I-III) have been studied. The hydrodynamic size of proteoglycan subunits and the tissue concentration of total glycosaminoglycans decreased with increasing grade of malignancy. The glycosaminoglycan distribution pattern of all chondrosarcomas showed a similar ratio of chondroitin-4-sulfate:chondroitin-6-sulfate but an increasing portion of keratan sulfate from grade I (6.5%) to grade III (19.2%). Determinations of the molecular weight (Mr values) of glycosaminoglycans were made after 3H labeling by alkaline reduction of proteoglycans in the presence of NaB3H4. The Mr of [3H]chondroitin sulfate isomers decreased markedly from grade I (35,500) to grade III (15,100) while the chain length of [3H]keratan sulfate showed minor variations (Mr 5600-6200). The previously reported decrease in the molecular weight of keratan sulfate with increasing degree of malignancy (S. Pal, W. Strider, R. Margolis, G. Gallo, S. Lee-Huang, and L. Rosenberg, 1978, J. Biol. Chem. 253, 1279-1289) was not observed.

Chondrosarcoma↗

Chondrosarcoma in a cow.

Chondrosarcomas are rare in cattle and none has been described in detail. A 30 cm diameter chondrosarcoma centred upon the costochondral junctions of the left 9th to 12th ribs of a 5-year-old Aberdeen Angus cow is described. In areas with a cartilaginous matrix the chondrocytes were plump, sometimes binucleate, sometimes bizarre, with occasionally 2 or more cells per lacuna. The other major matrix was loose and fibrillar, and the cells were spindle-shaped or stellate, with a moderate mitotic rate. Small areas appeared fibrosarcomatous. Both major types of matrix were present in the metastases, which extensively involved the pleura, intrathoracic lymph nodes and lungs. Less numerous and smaller metastases were present on the peritoneal surfaces and within several abdominal and pelvic organs. It is postulated that local extension of the tumour from the primary mass was followed by lymphatic spread to the lungs.

Animals↗

Cytotoxic activity of various snake venoms on melanoma, B16F10 and chondrosarcoma.

Elapid, crotalid and viperid venoms were screened in vitro and in vivo for cytotoxicity towards B16F10 melanoma and chondrosarcoma cell lines. The cytotoxic activity of elapid venoms was considerably higher than that of viperid or crotalid venoms. Elapid venoms disrupted the cell membrane within the first hour, leading to cell death. The strongest activity was found in the venom of Naja nigricollis. The venoms of some Viperidae and of all Crotalidae examined caused the cells to become rounded, without loss in their original volume, and to form aggregates. These changes were reversible when cells were changed to fresh medium. In vivo experiments with the venom of Naja nigricollis were in total agreement with the results achieved in vitro with melanoma cells and the venom exhibited similar cytotoxic activity on chondrosarcoma, inhibiting its development in vivo.

Animals↗

Chondrosarcoma metastatic to the oral cavity.

Metastases to the oral cavity from nonoral malignant lesions occur rarely. We report the case of a woman with chondrosarcoma of the pelvis in whom gingival metastases, in addition to pulmonary, skin, and brain metastases, developed. Clinical course, x-ray correlation, histopathologic review, and treatment modalities are described. The six previously reported cases of chondrosarcoma metastatic to the oral cavity are tabulated and analyzed. Metastases should be included in the differential diagnosis of gingival lesions in patients with prior or current nonoral malignant tumors.

Bone Neoplasms↗

Chondrosarcoma of the jaws: clinical findings, histopathology, and treatment.

Three cases of chondrosarcoma involving the jaws are presented, one in the maxilla and two in the mandible. The salient points of clinical presentation elucidated by this series of cases are that a widened periodontal ligament space is present in chondrosarcomas as well as in osteosarcomas, and that a slowly increasing diastema may be the earliest clinical sign. The most important lesson to be learned from the histopathology is that one should not accept a diagnosis of a benign cartilaginous tumor of the jaws. Treatment of these lesions should consist of wide surgical excision and consideration of adjunctive or palliative radiotherapy, especially in the maxilla. It should also be noted that recurrences may develop 10 to 20 years later, and follow-up should be lifelong.

Adult↗

Myxoid chondrosarcoma of soft tissue and bone: a retrospective study of 11 cases.

This article describes 11 cases of myxoid chondrosarcoma (MCS), with 10 arising in soft tissues and one developing in bone. Most of the tumors (six) were located in the lower extremities. Two lesions developed in the fingers, a previously unreported location for MCS. Four cases showed secondary bone destruction, which is a rare feature of this tumor. S100 protein was expressed by tumor cells in all the specimens. Four out of eight tumors studied by electron microscopy contained intracisternal microtubular structures. Two tumors showed areas of spindle cell proliferation that merged with the areas of typical myxoid pattern. The cells in these areas had fibroblastic/myofibroblastic features by electron microscopy and were found to express cytokeratin by immunohistochemistry. The concomitant expression of cytokeratin and S100 protein in the spindle cells suggests that they represent a less differentiated cartilaginous component with unusual features. The clinical significance of the presence of such spindle cell areas presently remains unknown. Although myxoid chondrosarcoma is a slow-growing tumor, it has a high potential for metastases. Four of 11 patients in this series developed metastases.

Adolescent↗

A combined infratemporal and posterior fossa approach for the removal of giant glomus tumors and chondrosarcomas.

Giant glomus jugulare tumors with a large posterior fossa extension are considered either inoperable or at least requiring of a two-stage operation. Likewise, the surgical approach and treatment for chondrosarcomas of the temporal bone are controversial. We describe a combined approach in which, with the aid of microsurgical and laser techniques, such tumors can be removed in one stage. The surgical approach involves a lateral infratemporal approach combined with a posterior fossa craniectomy. This technique was used in seven cases: five glomus jugulare tumors and two chondrosarcomas. There were no deaths, and surgical morbidity consisted of weakness in the facial nerve in four of these patients and gastrointestinal hemorrhage and respiratory distress syndrome in one patient.

Brain Neoplasms↗

Primary intraspinal dura mesenchymal chondrosarcoma.

An 18-year-old woman presented with 6 months of local paraspinal pain and 2 months midthoracic myelopathy. She was proved to have an intraspinal tumor. After successful surgical removal, it was found histologically to be a mesenchymal chondrosarcoma. Local irradiation was administered for the prevention of local recurrence. This case is reported with a review of the literature of primary mesenchymal chondrosarcoma of the spinal dura.

Adolescent↗