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PubMed1983

Chondrosarcoma of the paranasal sinuses and nasal septum.

A case of chondrosarcoma of the left antro-ethmoid region and nasal septum is presented as a second primary malignant tumour in a patient who had undergone a previous excision of a rodent ulcer in the cheek. X-ray studies and multiple biopsies of the nasal cavity were undertaken to determine as accurately as possible the extent of the tumour. The tumour was excised via a lateral rhinotomy. Chondrosarcoma of the maxillofacial region is a rare tumour. The treatment of choice is by surgical excision. Radiotherapy may be considered in non-resectable or recurrent tumours.

Chondrosarcoma↗
PubMed1988

Chondrosarcoma of the sphenoid--a case report and review.

The case of 57-year-old woman is presented with a chondrosarcoma of the sphenoid. The main symptom was rapid loss of vision and in order to halt this the tumour was debulked. The literature relating to chondrosarcomas of the head and neck is reviewed. These rare tumours are not radio-sensitive and surgery represents the best form of treatment at the present time. The prognosis is poor.

Chondrosarcoma↗
PubMed1989

Laryngeal chondrosarcoma: is conservative surgery adequate treatment?

A case of laryngeal chondrosarcoma in a 63-year-old woman is presented. The report illustrates most of the features of this rare neoplasm, the difficulties in the histopathological evaluation and supports the previous ones that recommend an initial conservative attitude towards low grade laryngeal chondrosarcomas.

Chondrosarcoma↗
PubMed1993

Chondrosarcoma of the hyoid bone.

Chondrosarcoma of the head and neck is relatively rare. We report the sixth case known to us in which the chondrosarcoma had originated in the hyoid bone. The patient, a 66-year-old man, underwent surgery. The resected tumour was a 5 cm encapsulated lesion composed of lobulated, firm, gray-white tissue. Histologically it showed mature chondrocytes without capsular invasion. There was high cellularity and a few binucleated chondrocytes. Although the tumour grade was prognostic significance, our literature review of the five other cases suggests that the location of the primary lesion and the adequacy of its resection to be more important. Our patient continues to do well 15 months post-operatively. However, long-term follow-up is essential as recurrences have been reported even after eight or 10 years.

Aged↗
PubMed1997

Chondrosarcoma of the petrous apex. Dilemmas in diagnosis and treatment.

A case of chrondrosarcoma of the petrous temporal bone is presented. Chondrosarcomas rarely occur intracranially and typically present apex mass. The dilemmas faced in the diagnosis and treatment of petrous apex chondrosarcomas are discussed. This case also gives interesting insight into the natural history of this tumour.

Chondrosarcoma↗
PubMed1994

Rat chondrosarcoma ATP sulfurylase and adenosine 5'-phosphosulfate kinase reside on a single bifunctional protein.

The sulfate-activation pathway consists of the sequential action of ATP sulfurylase (ATP: sulfate adenylyltransferase, EC 2.7.7.4) and adenosine 5'-phosphosulfate kinase ATP:adenylylsulfate 3'-phosphotransferase, EC 2.7.1.25). Both sulfurylase and kinase from rat chondrosarcoma were copurified through substrate affinity chromatography using stable analogs of APS (adenosine 5'-phosphosulfate) and PAPS (3'-phosphoadenosine 5'-phosphosulfate). A 56-kDa protein, containing both activities, was then purified to apparent homogeneity through reversed-phase chromatography and observed on denaturing polyacrylamide gels. The molecular mass of the native active unit containing both activities in the purified preparation corresponded to approximately 60 kDa by analytical gel filtration. Coincident binding and elution of ATP sulfurylase and APS kinase by immunoaffinity chromatography, using polyclonal serum generated against the 56-kDa protein, also demonstrated that the enzyme contains both activities. Lastly, a single N-terminal amino acid sequence was obtained from the 56-kDa band isolated by gel electrophoresis. These results all suggest that ATP sulfurylase and APS kinase from rat chondrosarcoma residue on a single bifunctional protein.

Amino Acid Sequence↗
PubMed1994

Intermediate channeling between ATP sulfurylase and adenosine 5'-phosphosulfate kinase from rat chondrosarcoma.

Biosynthesis of the activated sulfate donor PAPS (3'-phosphoadenosine 5'-phosphosulfate) involves the sequential action of two enzyme activities. ATP sulfurylase catalyzes the formation of APS (adenosine 5'-phosphosulfate) from ATP and free sulfate, and APS is then phosphorylated by APS kinase to produce PAPS. Using rat chondrosarcoma ATP sulfurylase and APS kinase, a newly developed assay system, which permits measuring the accumulation of both APS and PAPS in the presence of both enzyme activities, produces a PAPS/APS ratio corresponding to a "channeling efficiency" of 96%. The velocity of the APS kinase reaction measured in the overall system with endogenously synthesized APS is 8-fold greater than that of the isolated kinase reaction using exogenous APS. Most conclusively, isotope dilution and enrichment experiments show that the APS intermediate does not equilibrate with APS in the bulk medium but remains largely bound in the rat enzyme system. In contrast, control experiments with a nonchanneled system containing a mixture of the sulfurylase and kinase isolated from Penicillium chrysogenum give the results expected for a nonchanneled pathway. These data indicate that APS is channeled between the active sites of ATP sulfurylase and APS kinase during the production of PAPS in rat chondrosarcoma.

Adenosine Phosphosulfate↗
PubMed2002

Synthesis and in vitro evaluation of quaternary ammonium derivatives of chlorambucil and melphalan, anticancer drugs designed for the chemotherapy of chondrosarcoma.

To enhance affinity for malignant cartilaginous tumors (chondrosarcomas), quaternary ammonium (QA) conjugates of chlorambucil and melphalan were prepared by linking the QA moiety to nitrogen mustards via an amide bond. They exhibited closely similar and sometimes more favorable values than their parent compounds. In the cell lines tested, the two QA conjugates displayed appreciable cytotoxicity, the QA conjugate of chlorambucil even showing an enhanced efficiency against chondrosarcoma compared with chlorambucil.

Animals↗
PubMed1999

Recurrent chondrosarcoma of the cranial base: a durable response to ifosfamide-doxorubicin chemotherapy.

The case of a 46-year old woman with recurrent chondrosarcoma of the cranial base, refractory to neurosurgical intervention and external radiotherapy is reported. She received five cycles of systemic chemotherapy utilizing ifosfamide and doxorubicin which resulted in a durable clinical and radiographic response lasting 52+ months. A review of the management options for recurrent chondrosarcoma of the cranial base is also presented.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Chondrosarcoma of the pubis imitating bladder tumor radiologically: a case report.

Chondrosarcoma is a rare tumor that more frequently involves the pelvic bones, the femur, and the humerus. The rareness of the tumor makes the diagnosis difficult. Malignant cartilage tumors typically have an unstable radiographic appearance. In this report we aimed to point out the difficulties of the radiological differentiation of the pelvic chondrosarcoma and bladder tumor.

Adult↗
PubMed2004

A distinct phenotype characterizes tumors from a putative genetic trait involving chondrosarcoma and breast cancer occurring in the same patient.

Recently, we documented an increased risk for the occurrence of breast- and cartilaginous tumors in the same patient, statistically pointing towards a potential genetic trait. This trait is most probably not associated with mutations in the two major hereditary breast cancer genes since no cases of enchondroma or chondrosarcoma were found in Dutch BRCA1 and BRCA2 families. We were able to collect and review the tumor tissue samples from 34 patients with both breast- and cartilaginous tumors and compared histopathological and immunohistochemical features of these tumors with controls. Breast cancer controls were available from literature data generated to compare familial breast cancers with nonselected cases. Clinical markers for chondrosarcoma controls were collected from the Netherlands Committee of Bone Tumors. Immunohistochemical data on chondro-tumor controls were available from our own files. Breast tumors of patients with cartilaginous sarcomas showed a significantly higher mitotic count (P=0.001), contained less lymphocyte infiltrate (P=0.025) and less nuclear pleomorphism. Remarkably, all cartilaginous tumors are of one common histological category originating centrally (P=0.014). Estrogen receptor and p53 expression were significantly higher (P<0.001) in breast cancer associated with chondro-tumors. p21 staining was more often negative in chondro-tumors associated with breast cancer. In seven cases of breast cancer, we found a slight decrease in CHEK2 expression. However, we could not identify the CHEK2 1100delC mutation in these cases nor in cases with normal CHEK2 expression. Hierarchical cluster analysis of all parameters within chondro-tumor-associated breast cancer specimens revealed two different subgroups, the largest one associated with estrogen receptor-positive breast cancer, which may distinguish sporadic cases from those belonging to the potential genetic trait. These distinct phenotypic findings support the existence of a new hitherto unrecognized syndrome, characterized by an increased risk to develop both breast cancer and centrally originating cartilaginous tumors.

Adenocarcinoma↗
PubMed2000

Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and ploidy analysis of 23 cases.

Twenty-three cases of extraskeletal myxoid chondrosarcoma, evaluated at the Mayo Clinic between 1968 and 1996, were studied for clinicopathologic features, immunohistochemical profile, Ki-67 activity, and ploidy status to identify adverse prognostic factors. Females and males were equally affected, and the median age at diagnosis was 50 years. The tumors were located mainly in the lower extremities (83%), and the median tumor size was 9.5 cm. Sixteen tumors showed low cellularity (70%), and eight tumors had high mitotic activity (more than two per 10 high-power fields). The tumors were immunoreactive for vimentin (89%), synaptophysin (72%), epithelial membrane antigen (28%), and S-100 protein (17%). Nine tumors were diploid, three aneuploid, and one tetraploid. Mean Ki-67 activity was 11% (range, 1 to 45%). The 10-year overall survival rate was 78%. On univariate analysis, tumor size > or = 10 cm, high cellularity, presence of anaplasia or rhabdoid features, mitotic activity more than two per 10 high-power fields, Ki-67 > or = 10%, and Ki-67 "hot spot" > or = 25% were associated with decreased metastasis-free or overall survival. Ploidy status was not associated with any adverse outcome. The presence of any of these adverse prognostic factors can indicate the possibility of a more aggressive behavior in extraskeletal myxoid chondrosarcoma, and a closer follow-up is suggested.

Adult↗
PubMed1998

Chondrosarcoma of the maxilla: panoramic radiographic and computed tomographic with multiplanar reconstruction findings.

Chondrosarcomas are extremely rare tumors of which approximately 10% are found in the maxillofacial region. In this report, we present the imaging findings of a maxillary chondrosarcoma on a panoramic radiograph of the jaws and computed tomography with multiplanar reconstructions. We recommend the latter as an excellent way to image evolving or suspected lesions of the maxilla, particularly for surgical treatment planning.

Adult↗
PubMed2000

The intermediates of aggrecanase-dependent cleavage of aggrecan in rat chondrosarcoma cells treated with interleukin-1.

We have examined the abundance and structure of intermediates in the chondrocyte-mediated degradation of aggrecan by aggrecanase(s). Degradation products were identified by Western-blot analysis with antibodies to cleavage-site neoepitopes and to peptides within the globular domains. Rat chondrosarcoma tumour contained full-length aggrecan and all of the individual peptides expected from single independent cleavages at each of the four aggrecanase sites in the chondroitin sulphate (CS) domain. Kinetic analysis of the products present in rat chondrosarcoma cell cultures treated with interleukin-1b showed that the first aggrecanase-mediated cleavages occurred at the four sites within the CS attachment region to generate two stable intermediates, Val(1)-Glu(1459) and Val(1)-Glu(1274). These species were subsequently cleaved at the Glu(373) site in the interglobular domain to form the terminal products, Val(1)-Glu(373), Ala(374)-Glu(1274) and Ala(374)-Glu(1459). It therefore appears that the aggrecanase-mediated processing of native aggrecan by chondrocytes in situ is initiated within the CS-attachment region and completed by cleavage within the interglobular domain. Since it has been shown that digestion of aggrecan monomer in solution with recombinant ADAMTS-4 [Tortorella, Pratta, Liu, Austin, Ross, Abbaszade, Burn and Arner (2000) Sites of aggrecan cleavage by recombinant human aggrecanase-1 (ADAMTS-4). J. Biol. Chem. 275, 18566-18573] exhibits similar kinetics, it appears that preferential proteinase cleavage in the CS-rich region is determined by properties inherent in the aggrecan monomer itself, such as preferred peptide sequences for enzyme binding or enhanced accessibility to the core protein at these sites.

Aggrecans↗
PubMed1983

Differences in the rates of aggregation of proteoglycans from human articular cartilage and chondrosarcoma.

Pieces of adult human articular cartilage and chondrosarcoma were incubated in the presence of [35S]sulphate. After continuous or pulse-change incorporation of radioactivity, proteoglycans were extracted with 4.0 M-guanidinium chloride, purified by equilibrium density-gradient centrifugation and fractionated by gel chromatography. A comparison of the results suggests that the formation of stable aggregates occurs at a lower rate in articular cartilage than in chondrosarcoma.

Adult↗
PubMed1988

Aberrant regulation of the metabolism of the insulin receptor in Swarm rat chondrosarcoma chondrocytes.

Treatment of Swarm rat chondrosarcoma chondrocytes for 3 days in media containing either non-recombinant pig or recombinant human insulin (1 micrograms/ml) increased the rate of proteoglycan synthesis approximately 6-fold compared with cells cultured in the absence of insulin. The concentrations of human and pig insulin that stimulated the cells to double their rate of proteoglycan synthesis were approximately 1 ng/ml and approximately 2 ng/ml respectively. Because physiological concentrations of insulin do not influence proteoglycan synthesis in non-transformed chondrocytes, the findings indicated a possible abnormality in the insulin-dependent regulation of the insulin receptor in these tumour cells. Like most cells, chondrosarcoma chondrocytes down-regulated their insulin receptors when incubated with insulin for 30 min. However, the number of plasma-membrane and intracellular insulin receptors did not decrease when the chondrocytes were exposed to insulin chronically for 4 days. Chondrocytes were cultured in media containing 2H-, 13C- and 15N-labelled amino acids, and the heavy-isotope density-shift method was used to investigate both the rate of degradation and the rate of synthesis of the insulin receptor. Although the rate of synthesis of the receptor was slightly faster in the insulin-treated cultures, as assessed by a slightly faster rate of appearance of the 'heavy' receptor, the rate of degradation of the receptor was slower in the insulin-treated cultures. The half-lives for the 'light' receptors were approx. 18 h and 10 h for chondrocytes cultured in insulin-containing and insulin-free media respectively. These studies in vitro indicate that the apparent up-regulation of insulin receptors that occurs in this transformed cell upon long-term exposure to insulin is primarily the result of a decreased rate of receptor degradation.

Animals↗
PubMed1990

Intermediary metabolism in the Swarm rat chondrosarcoma chondrocyte.

The rat chondrosarcoma chondrocyte has the dual capacity to metabolize glucose (mainly via glycolysis) and glutamine (via an oxidative pathway). Glutamine metabolism, unlike that of glucose, is unable to sustain intracellular ATP concentrations. Glutamine consumption by the chondrosarcoma chondrocyte, however, is significantly in excess of its utilization as an amide-group donor in hexosamine synthesis, implying a novel and major role in cell metabolism.

Adenine Nucleotides↗
PubMed1991

An unsulphated region of the rat chondrosarcoma chondroitin sulphate chain and its binding to monoclonal antibody 3B3.

The chondroitin sulphate chains of proteoglycans are not uniformly sulphated. Commonly, regions of under- and over-sulphation are found. It is probable that variability in chondroitin sulphation has physiological significance, although such structure-function relationships largely remain unexplored. Chondroitin sulphate from rat chondrosarcoma proteoglycan has been found to possess no oversulphated residues. It is primarily chondroitin 4-sulphate, although a significant proportion of unsulphated disaccharides (14%) are also present. It appears that some unsulphated disaccharides are concentrated only at the point of attachment to the linkage region (i.e. it is the major unsaturated disaccharide remaining attached to chondrosarcoma proteoglycan core produced by chondroitinase ABC digestion). This proteoglycan core binds monoclonal antibody (MAb) 3B3. Although 3B3 principally binds to 6-sulphated 'stubs' of proteoglycan cores [Couchman, Caterson, Christner & Baker (1984) Nature (London) 307, 650-652], given a high concentration of unsulphated 'stubs', it can alternatively bind to these residues. It is also evident that caution must be exercised in using MAb 3B3 to identify chondroitin 6-sulphated proteoglycans.

Animals↗