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PubMed2006

Array-comparative genomic hybridization of central chondrosarcoma: identification of ribosomal protein S6 and cyclin-dependent kinase 4 as candidate target genes for genomic aberrations.

BACKGROUND: Enchondromas are benign lesions that can occur as solitary tumors or multiple tumors (Ollier disease) and may be precursors of central chondrosarcomas. Recurrent chondrosarcomas can be of a higher grade compared with primary tumors, suggesting possible progression. METHODS: Genome-wide array-comparative genomic hybridization (CGH) was used to investigate copy number changes in enchondromas and central chondrosarcomas to elucidate both primary genetic events and the events related to tumor progression. Analyses of variance, Student t tests, and hierarchical clustering were used for the current analyses. Array-CGH data were compared with complementary DNA (cDNA) and quantitative reverse-transcriptase polymerase chain reaction expression array data. RESULTS: Genomic imbalances were rare in enchondromas and in grade I chondrosarcomas, whereas they were frequent in high-grade tumors. No genomic imbalances that were specific for Ollier disease were found. The authors identified 22 chromosome regions that were imbalanced in > or =25% of tumors, and 3 of those regions were located on chromosome 12 (12p13, 12p11.21-p11.23, and 12q13, containing among others the PTPRF-interacting protein-binding protein 1 (PPFIBP1) gene. Loss of chromosome 6 and gain of 12q12 were associated with higher grade. Comparison of array-CGH with cDNA expression showed correlations for the ribosomal protein S6 (RPS6) and cyclin-dependent kinase 4 (CDK4) genes. CONCLUSIONS: In the current study the authors identified genomic regions and new candidate genes (RPS6, CDK4, and PPFIBP1) that were associated with tumor progression and prognosis in patients with high-grade chondrosarcomas.

Adaptor Proteins, Signal Transducing↗

PubMed1991

Experimental transplantation of the Swarm rat chondrosarcoma into bone: radiological and pathological studies.

The Swarm rat chondrosarcoma has been the subject of extensive biochemical studies. However, to our knowledge, there are no previous reports in the literature on transplantation of this tumor into bone. This article describes the natural history of the tumor when implanted into the bone of the rat, and correlates its histological growth pattern with its radiological appearance. Our results showed that the tumor grows slowly in the bone. The rate of intramedullary growth, however, was variable and was not the same in all the animals. Its growth pattern resembles human chondrosarcoma, with extensive invasion of the marrow and cortex. In the first few weeks after implantation, the only radiological changes noted were mild medullary radiopacities. At a later stage, 12-14 weeks postimplantation, as the tumor infiltrated the bone, significant radiological abnormalities were observed in the medullary cavity and cortex. Periosteal reaction was seen after the tumor invaded the cortex with the production of a soft-tissue mass. Distant dissemination was rare; only 1 of 24 rats developed pulmonary metastases. The Swarm rat chondrosarcoma is a well-differentiated malignant tumor that histologically resembles well-differentiated human chondrosarcoma. Transplanted into bone, it may be useful as an experimental model for comparative studies with human chondrosarcoma.

Animals↗

PubMed1998

Chemotherapy and P-glycoprotein expression in chondrosarcoma.

Chondrosarcomas are alleged to be resistant to chemotherapy. A retrospective review of our experience primarily with dedifferentiated chondrosarcomas treated with chemotherapy was performed to reevaluate the efficacy of chemotherapy for this tumor. There were 18 patients: 14 stage IIB and four stage III. Seventeen patients had dedifferentiated chondrosarcoma. The median age at diagnosis was 57 years. Fourteen of the patients underwent wide excision of the tumor, two underwent amputation, and two had no surgery. The femur and the pelvis were the most common locations of the primary tumor. Chemotherapy for 11 of the patients consisted of cisplatin and doxorubicin. Survival was analyzed with the Kaplan-Meier method; the median survival was 12 months. The hypothesis that chondrosarcomas express P-glycoprotein was tested. Expression of P-glycoprotein was evaluated by immunostaining with use of the C494 and C219 antibodies on 41 benign and malignant cartilage tumors, six of which were from the patients in the chemotherapy group. Immunostaining revealed that 37 of 41 cartilage tumors expressed P-glycoprotein. The rate of survival of patients with high-grade chondrosarcoma treated with chemotherapy is poor. P-glycoprotein expression is common in benign and malignant cartilage lesions. The lack of response to chemotherapy may be related to the expression of P-glycoprotein.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

PubMed2005

cDNA expression profiling of chondrosarcomas: Ollier disease resembles solitary tumours and alteration in genes coding for components of energy metabolism occurs with increasing grade.

Conventional central chondrosarcomas are malignant cartilaginous tumours, occasionally arising secondary to either solitary or multiple (Ollier disease) enchondromas. Recurrences may have progressed in grade. The aims of the present study were to identify putative differences in gene expression between solitary and Ollier disease-related tumours, and to elucidate signalling pathways involved in tumour progression by genome-wide cDNA expression analysis. Arrays enriched for cartilage-specific cDNAs and genes involved in general tumourigenesis were used to analyse enchondromas (n = 3, two with Ollier disease), chondrosarcomas of different grades (n = 19, three with Ollier disease), normal resting-zone cartilage (n = 2), and chondrosarcoma cells in culture (n = 7). The arrays were analysed by unsupervised hierarchical clustering, significant analysis of microarray, and T-tests. Confirmation of data was performed by immunohistochemistry and quantitative reverse transcriptase polymerase chain reaction (RT-PCR). Ollier disease cases and solitary tumours revealed similar expression profiles, suggesting that the same signalling pathways are involved in tumourigenesis. Interestingly, JunB protein expression was significantly higher in grade I chondrosarcomas than in enchondromas (p = 0.009), which could be of diagnostic relevance. Upon chondrosarcoma progression, matrix-associated genes are down-regulated, reflecting the histology of high-grade tumours. An increase in glycolysis-associated, and a decrease in oxidative phosphorylation-related, genes was found in high-grade tumours. These findings suggest an adaptation in energy supply upon progression towards higher grade.

Adolescent↗

PubMed2006

Peripheral chondrosarcoma progression is accompanied by decreased Indian Hedgehog signalling.

Hedgehog (HH) signalling is important for specific developmental processes, and aberrant, increased activity has been described in various tumours. Disturbed HH signalling has also been implicated in the hereditary syndrome, Multiple Osteochondromas. Indian Hedgehog (IHH), together with parathyroid hormone-like hormone (PTHLH), participates in the organization of growth plates in long bones. PTHLH signalling is absent in osteochondromas, benign tumours arising adjacent to the growth plate, but is reactivated when these tumours undergo malignant transformation towards secondary peripheral chondrosarcoma. We describe a gradual decrease in the expression of Patched (PTCH) and glioma-associated oncogene homologue 1 (GLI1) (both transcribed upon IHH activity), and GLI2 with increasing malignancy, suggesting that IHH signalling is inactive and PTHLH signalling is IHH independent in secondary peripheral chondrosarcomas. cDNA expression profiling and immunohistochemical studies suggest that transforming growth factor-beta (TGF-beta)-mediated proliferative signalling is active in high-grade chondrosarcomas since TGF-beta downstream targets were upregulated in these tumours. This is accompanied by downregulation of energy metabolism-related genes and upregulation of the proto-oncogene jun B. Thus, the tight regulation of growth plate organization by IHH signalling is still seen in osteochondroma, but gradually lost during malignant transformation to secondary peripheral chondrosarcoma and subsequent progression. TGF-beta signalling is stimulated during secondary peripheral chondrosarcoma progression and could potentially regulate the retained activity of PTHLH.

Adolescent↗

PubMed1997

MRI of mesenchymal chondrosarcoma of the orbit: case report and review of the literature.

Extraskeletal mesenchymal chondrosarcoma is a relatively uncommon entity, an orbital location being extremely rare. A review of the literature revealed 16 reported cases of primary orbital mesenchymal chondrosarcoma demonstrated by plain film and CT. To the best of our knowledge, the MRI features of orbital extraskeletal mesenchymal chondrosarcoma have not been previously reported. We present the case of an 18-year-old man with a 2-year history of progressive proptosis of the right eye who underwent CT, dynamic CT, MRI without and with gadolinium enhancement, and magnetic resonance angiography of the orbits. CT of orbital mesenchymal chondrosarcoma demonstrates a well-defined mass with multiple areas of fine and coarse calcification and shows moderate contrast enhancement. The noncalcified portions of the mass demonstrate signal intensity lower than or equal to gray matter on T1-weighted images and are isointense to the gray matter on T2-weighted images. Dynamic CT reveals delayed contrast enhancement. MRI has proven to be a valuable diagnostic tool in the diagnosis and differentiation of well-defined intraorbital masses. By a combination of CT and MRI, it appears mesenchymal chondrosarcoma can be differentiated from other intraorbital lesions, such as cavernous hemangioma, hemangiopericytoma, orbital amyloidosis and fibrous histiocytoma.

Adolescent↗

PubMed2002

Acral synovial chondrosarcoma.

Acral chondrosarcoma is rare. Synovial chondrosarcoma is even rarer. Synovial chondrosarcoma arising without evidence of pre-existing or concurrent synovial chondromatosis is exceedingly rare. We present a case of acral synovial chondrosarcoma involving both sides of the metacarpophalangeal joint of the thumb in a 69-year-old man. Radiographically, the lesion mimicked gout. On MR imaging, the lobulated contours of the soft tissue mass suggested synovial chondromatosis. Histological examination revealed a chondrosarcoma, which on the basis of imaging findings we present as having arisen from the synovium. The tumor invaded a portion of the cartilage of the metacarpophalangeal joint and equally destroyed the bones of the distal metacarpal and base of the proximal phalanx of the thumb, while sparing the bony joint surfaces.

Aged↗

PubMed2006

A case of metacarpal chondrosarcoma of the thumb.

Enchondroma is the most common primary benign bone tumor of the hand. Chondrosarcomas in this location, however, are extremely rare. It often is difficult to make a histological distinction between benign cartilaginous tumors and low-grade chondrosarcomas, because enchondromas at this site often show histological features suggestive of malignancy. However, distinguishing the two conditions is clinically relevant because chondrosarcomas of the hand require prompt and more radical treatments such as ray amputations. On the other hand, Mankin has recently given attention to a less aggressive behavior of chondrosarcomas of the phalanges compared with those of other locations. And also, ray amputation does not cause much functional deficit in the finger but does for the thumb. This is a case report of chondrosarcoma affecting the metacarcal bone of the thumb, treated by en block resection and preserving the function of the thumb by bone graft reconstruction, with reference to the literature.

Bone Neoplasms↗

PubMed2005

The use of Bcl-2 and PTHLH immunohistochemistry in the diagnosis of peripheral chondrosarcoma in a clinicopathological setting.

Distinguishing osteochondroma from low-grade secondary peripheral chondrosarcoma can be difficult. In osteochondroma, growth-signalling pathways are thought to be downregulated through exostosin (EXT) inactivation. A previous pilot study focusing on expression of putative EXT downstream effectors indicated that progression of osteochondroma towards grade I chondrosarcoma was characterised by upregulation of Bcl-2 and parathyroid hormone-like hormone (PTHLH). We investigated their use as diagnostic markers in a large nationwide series of 71 osteochondromas and 34 chondrosarcomas. Bcl-2 immunohistochemistry proved to be a valuable diagnostic tool: scoring negative in 95% (specificity) of the osteochondromas and positive in 57% (sensitivity) of the chondrosarcomas, reaching a positive predictive value of 84% and negative predictive value of 82%. Positivity was not related to age, hereditary status, gender or thickness of the cartilage cap. Presence of internal controls and verification using mRNA in situ hybridisation strengthened the reliability of the immunohistochemical staining. PTHLH showed more variable staining, being positive in osteochondromas from females or adolescent males, suggesting age- and gender-dependent expression. Thus, in cases where the distinction between osteochondroma and chondrosarcoma is difficult, Bcl-2 is a valuable diagnostic marker for malignancy, regardless of tumour size, patient gender or age, and this can be extended with PTHLH for non-adolescent male patients.

Adolescent↗

PubMed1999

Extraskeletal myxoid chondrosarcoma: multimodal diagnosis and identification of a new cytogenetic subgroup characterized by t(9;17)(q22;q11).

Extraskeletal myxoid chondrosarcoma is a rare malignant soft tissue tumour that can be difficult to diagnose correctly, especially preoperatively. We describe four cases of extraskeletal myxoid chondrosarcoma of the extremities diagnosed by a multimodal approach. The cytological examination of fine-needle aspirates showed small and round, mildly pleomorphic cells lying in sheets and cords, but also dispersed within a myxoid and metachromatic intercellular substance. Histological, electron microscopic and immunocytochemical examination also yielded findings compatible with the diagnosis of extraskeletal myxoid chondrosarcoma. Cytogenetic analysis demonstrated a t(9;22)(q22;q12) in two tumours and a t(9;17)(q22;q11) in the third and fourth. The translocation t(9;22)(q22;q12) has been described repeatedly in extraskeletal myxoid chondrosarcoma but never in other tumours; hence, the detection of this pathognomonic chromosome abnormality in short-term cultured cells from fine-needle aspirates verified the diagnosis in two of the cases. The t(9;17)(q22;q11) found in the last two cases probably represents a new cytogenetic subgroup of extraskeletal myxoid chondrosarcoma as it, too, is unknown in other contexts. The multimodal approach taken in these four cases enabled a definite diagnosis of a rare malignant tumour whose cytological and histological features alone are usually not sufficiently distinct to rule out other differential diagnostic possibilities.

Aged↗

PubMed2000

Heat shock protein 72 expression in chondrosarcoma correlates with differentiation.

PURPOSE: Heat shock proteins (hsp) are involved in tumor immunity, and a correlation with survival, occurrence of metastases, and drug resistance has been reported. It was the aim of this study to investigate the expression of heat shock proteins in chondrosarcomas and chondromas. METHODS: Hsp expression was investigated immunohistochemically on paraffin-embedded sections of 37 consecutive patients (24 male and 13 female, mean age 48 years) with chondrosarcoma and of ten patients (six male, four female, mean age 36 years) with chondroma. RESULTS: Chondromas showed a positive staining for hsp27 in 100%, for hsp60 in 30%, for hsp72 in 80%, for hsp73 in 80%, and for hsp90 in 90%. In chondrosarcoma a decreased expression was found for hsp27 (62% positive, P<0.05) and hsp72 (43% positive, P<0.05), whereas no significant difference to chondromas was detected in the expression of hsp60 (49% positive), hsp73 and hsp90 (73% and 81% positive, respectively). In addition, hsp72 expression showed a correlation with differentiation of the tumors (P<0.05); the lowest hsp72 expression was found in G3 chondrosarcomas (only 13% positive). No correlation with respect to differentiation was found for the expression of the other hsps. CONCLUSIONS: This study shows a different expression of hsps in chondrosarcomas and chondromas. Together with the correlation of hsp72 expression with low differentiation, this finding could lead to new experimental and diagnostic strategies.

Adolescent↗

PubMed2005

Incidentally detected parafalcine chondrosarcoma.

Parafalcine chondrosarcoma is extremely rare, and may be difficult to differentiate preoperatively from falx meningioma. An 18-year-old woman presented with a parafalcine chondrosarcoma incidentally detected as a small lesion 2 years before admission, suggesting falx meningioma. Brain computed tomography and magnetic resonance imaging just before admission revealed the parafalcine lesion had increased by about nine times in volume during the last 2 years. Single-photon emission computed tomography (SPECT) after intravenous administration of both thallium-201 chloride ((201)TlCl) and N-isopropyl-p-[(123)I]iodoamphetamine ((123)I-IMP) demonstrated no abnormal uptake of either tracer. Histological examination revealed classic low-grade chondrosarcoma. Parafalcine chondrosarcoma should be considered at this site if relatively rapid growth is observed. SPECT using (201)TlCl and (123)I-IMP may be useful to discriminate parafalcine low-grade chondrosarcoma from meningioma or other tumours originating in this region.

Adolescent↗

PubMed1989

Complex cytogenetic aberrations in a well-differentiated chondrosarcoma.

The cytogenetic analysis of a well-differentiated (grade I) chondrosarcoma is presented. Despite a low-grade histopathology, metaphase preparations of tumor cells were characterized by numerous structural chromosome aberrations and a striking degree of genetic instability; the t(9;22)(q31;q12) that has been described recently in extraskeletal myxoid chondrosarcoma was not observed. This report suggests that chondrosarcomas may be heterogeneous cytogenetically. In chondrosarcoma, it appears that "high-grade" cytogenetic abnormalities do not necessarily engender a high-grade histology. These findings offer a possible explanation for the clinical aggressiveness of certain low-grade chondrosarcomas.

Aged↗

PubMedDate not supplied

A rare cause of laryngeal distress: chondrosarcoma of the larynx.

Laryngeal chondrosarcomas are much less aggressive lesions than chondrosarcomas that arise elsewhere in the body. Two cases of chondrosarcoma of the larynx encountered during a seven year period are presented, with a review of the literature. Computed tomographic (CT) and magnetic resonance imaging (MRI) findings are described. The MRI findings of laryngeal chondrosarcoma are compared to those of cartilaginous tumors elsewhere in the body. The differential diagnosis, prognosis, and treatment of laryngeal chondrosarcomas are discussed.

Aged↗

PubMed2006

Cyclooxygenase-2 overexpression associated with a poor prognosis in chondrosarcomas.

Recent studies have shown increased levels of cyclooxygenase (COX)-2 in various human malignancies, including some bone and soft tissue tumors, but little is known about the presence of COX-2 in chondrosarcomas. We performed immunohistochemical staining for COX-2 in 74 chondrosarcomas and compared the staining results with the characteristics and outcome of the patients. Thirty-seven men and 37 women between the ages of 14 and 81 years (median, 50 years) participated in the study. The tumors were located in the axial skeleton in 47 cases and in the long bones in 27 cases. The largest diameters of the tumors ranged from 2.7 to 32 cm (median, 8.0 cm). The immunohistochemistry findings revealed the overexpression of COX-2 in 16 (22%) of the 74 patients. Thirteen (41%) of 32 grade 2 and 2 (67%) of 3 grade 3 chondrosarcomas showed overexpression for COX-2, whereas only 1 (3%) of 39 grade 1 chondrosarcomas showed overexpression. Overexpression of COX-2 was significantly associated with histologic grade (P < .001) and a decreased disease-specific survival (P < .001). These findings suggest that COX-2 overexpression in chondrosarcoma is a negative prognostic factor that may be strongly associated with histologic grade.

Adolescent↗

PubMed2002

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↗

PubMed1997

Loss of heterozygosity in the retinoblastoma tumor suppressor gene in skull base chordomas and chondrosarcomas.

BACKGROUND: The retinoblastoma (Rb) gene is a well characterized tumor suppressor gene in which loss of heterozygosity has been implicated in a number of malignancies including osteosarcoma and breast carcinoma. Chordomas and chondrosarcomas are rare skull base neoplasms with a propensity for local recurrences, resistance to conventional radiotherapy, and a 5%-30% incidence of metastases. Except for the so called "chondroid chordoma," histologic features do not correlate with the clinical behavior or growth patterns of these tumors. No study to date has investigated what role tumor suppressor genes or oncogenes play in the development and continued growth of these rare neoplasms. METHODS: In order to evaluate the role of the retinoblastoma tumor suppressor gene in chordomas and chondrosarcomas we screened seven chordomas and two chondrosarcomas located at the skull base for loss of heterozygosity (LOH) of the Rb gene. Genomic DNA was extracted from tumor specimens as well as matched control tissue and utilizing a polymerase chain reaction technique, intron 17 and 20 were amplified from each specimen. The intron 17 product was then digested with the restriction endonuclease X ba1 followed by electrophoresis on a 1% agrose gel. The intron 20 amplified products were electrophoresed on a nondenaturing 6% polyacrylamide gel. RESULTS: We demonstrated LOH at intron 17 of the retinoblastoma gene in 2/7 chordomas and in 0/2 chondrosarcomas. The two chordomas possessing LOH were particularly aggressive tumors demonstrating extensive involvement of the skull base and rapid recurrences following radical resections. CONCLUSIONS: Alterations of the Rb gene may play a role in the growth of skull base chordomas with LOH of the Rb gene serving as a marker for more aggressive tumors. This report represents the first study evaluating the Rb gene in chordomas or chondrosarcomas and is the first report of allelic loss of the Rb gene in skull base chordomas.

Adolescent↗

PubMed2000

Clonal chromosome abnormalities in enchondromas and chondrosarcomas.

We report cytogenetic findings in short-term cell cultures from five enchondromas and four chondrosarcomas. Clonal chromosome aberrations were found in one case of enchondroma, and in all cases of chondrosarcoma. The only enchondroma with nonrandom abnormalities had a reciprocal t(8;17)(q23;p13), and monosomies 9, 19, and/or 22. In contrast to the few karyotypic findings in one of five enchondromas, the four chondrosarcomas were commonly characterized by cytogenetic heterogeneity, with a tendency for increasing karyotypic complexity in higher grade tumors. Two cases, one grade III and one metastasizing grade II chondrosarcoma, revealed hypodiploid stem- and sidelines with loss of chromosomes 6, 10, 13, 14, and 22, as common chromosomal abnormalities, suggesting a distinct karyotypic pattern in a subset of biologically aggressive chondrosarcomas.

Adolescent↗