SEARCH · PubMed Health

Results for “CHONDROSARCOMA”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 235 records · Page 13Linked to original sources

PubMed1995

Loss of heterozygosity in chondrosarcomas for markers linked to hereditary multiple exostoses loci on chromosomes 8 and 11.

Hereditary multiple exostoses (EXT; MIM 133700) is an autosomal dominant condition characterized by growth of multiple benign cartilage-capped tumors. EXT greatly increases the relative risk to develop chondrosarcoma, although most chondrosarcomas are sporadic. This observation suggests that, like the genes responsible for retinoblastoma and other dominantly inherited cancer susceptibility disorders, the genes that cause EXT may have tumor-suppressor function and may play a role in the pathogenesis of the related sporadic tumors. To investigate this hypothesis, we evaluated chondrosarcomas for loss of constitutional heterozygosity (LOH) at polymorphic loci linked to three recently identified genomic regions containing genes involved in EXT. LOH for markers linked to EXT1 on chromosome 8 was detected in a chondrosarcoma that arose in a man with EXT. Four of 17 sporadic tumors showed LOH for markers linked to EXT1, and 7 showed LOH for markers linked to EXT2 on chromosome 11. In all, LOH was observed for markers linked to EXT1 or EXT2 in 44% of the 18 tumors, whereas heterozygosity was retained for markers on 19p linked to EXT3. These findings support the hypothesis that genes on 8q and the pericentromeric region of 11 have tumor-suppressor function and play a role in the development of chondrosarcomas.

Alleles↗

PubMed1994

Chondrosarcoma of the radius with distant metastasis in a dog.

A 9-year-old castrated male Doberman Pinscher was admitted for evaluation of lameness of the left forelimb. Radiography and examination of biopsy specimens revealed a moderately differentiated chondrosarcoma of the proximal portion of the radius. The dog was treated by local excision of the neoplasm, which involved resection of the radial head and proximal portion of the radius. Despite the large size of the dog and the weight-bearing forces exerted on the cubital joint, major problems with ambulation did not develop after surgery. Functional use of the limb returned slowly; however, substantial limb use was observed despite the development of mild degenerative changes of the joint and migration of the humeroulnar articulation. Six months after surgery, metastasis of a widely disseminated, poorly differentiated chondrosarcoma to the subcutaneous tissues and thoracic and abdominal cavities was diagnosed. Local redevelopment of the chondrosarcoma in the area of the cubital joint was not detected. Resection of the radial head and proximal portion of the radius may be considered a viable, alternative, limb-sparing technique. The biologically aggressive nature of this chondrosarcoma of the appendicular skeleton indicated that additional information was needed before a reliable prognosis could be established for this dog with this tumor type. Reports of low rates of metastasis have been based on insufficient numbers of dogs to adequately or accurately determine the long-term prognosis of dogs with chondrosarcoma of the appendicular skeleton.

Adrenal Gland Neoplasms↗

PubMed1993

[Immunohistochemical localization of bone Gla protein and osteonectin in normal human bone and cartilage tissues, and in osteosarcomas and chondrosarcomas].

The immunohistochemical localization of bone Gla protein (BGP) and osteonectin (ON) was investigated in normal human bone and cartilage tissues, and in osteosarcomas and chondrosarcomas with their respective antibodies. In normal bone and heterotopic ossification tissues, BGP and ON were detected in preosteoblasts, osteoblasts and young osteocytes, the reaction of which was strongest in osteoblasts. They were also demonstrated in most osteosarcomas, and their reaction was stronger in osteosarcomas with higher differentiation. These observations suggested that BGP and ON were related to the formation of normal and tumoral osteoid. In contrast, the localization of ON in normal cartilage and chondrosarcoma tissues was markedly different from that of BGP in these tissues. In normal growth cartilage, ON reacted with chondrocytes in hypertrophic and calcifying zone and matrix in calcifying zone, whereas BGP did not react. ON was also demonstrated in the cytoplasm and calcifying portion of most chondrosarcomas. These findings indicated that ON plays an important role in calcification of normal and tumoral cartilage tissues. BGP was detected in poorly differentiated and dedifferentiated chondrosarcomas. Thus, these antibodies are expected to be useful for studying calcification of human bone and cartilage and for the diagnosis of human osteosarcomas and chondrosarcomas.

Adolescent↗

PubMedDate not supplied

Needle aspiration cytology of chondrosarcomas.

The rarity of chondrosarcomas, limited experience with the spectrum of histopathologic and cytologic features, and the drastic treatment options that ensue present a diagnostic challenge to many cytopathologists who interpret needle aspirate biopsies of these neoplasms. Twelve fine needle (FNA) aspirates were performed at University Hospitals of Cleveland on eight patients with primary (three), locally recurrent (seven) or metastatic (two) chondrosarcoma between 1980 and 1991. The cytologic features of chondrosarcoma in all three clinical settings were characterized by the presence of isolated or loosely cohesive aggregates of oval or polygonal chondrocytes with vacuolated or granular cytoplasm and a background of chondroid matrix. FNA cytology is utilized most commonly in the diagnosis of recurrent and metastatic chondrosarcoma. FNA cytology, when employed in the diagnosis of chondrosarcoma, necessitates the same considerations employed in evaluating surgical biopsies. They include (1) the cytologic features and their limitations and differential diagnoses, (2) the clinical and radiographic data, and (3) the clinical impact of the cytologic diagnosis.

Adult↗

PubMedDate not supplied

[Chondrosarcoma of the maxilla. A clinical case report].

Chondrosarcoma are malignant tumour originating in cartilaginous cells which tend to preserve their essentially cartilaginous nature throughout their evolution. They are rare at the level of the maxilla and mandibular. When localized in facial bone, chondrosarcoma follow a more aggressive pattern compared to those localized in long bones. They mainly affect individuals aged between 30 and 60 and show no sexual preference. In the majority of cases, chondrosarcoma have a relatively slow rate of growth. The only valid treatment is radical surgical removal. In overall terms, the prognosis in these patients is not good since the 5-year survival rate is less than 40%. During the period 1980-1992 only 5 cases of chondrosarcoma were diagnosed by the Division of Maxillo-Facial Surgery of the University of Turin. The authors considered it worthwhile to report the last case observed in a premaxillary localization given the advanced age of onset and the typical course of the pathology and treatment received. CASE REPORT. A male 83-year-old patient was referred to use due to a slow growing neoformation in the premaxillary region which had appeared approximately 5-6 months earlier. The patient had previously undergone the avulsion of residual upper teeth due to suspected "toothache". The neoformation filled the premaxilla, deforming the anterior middle portion of the palate and spreading towards the floor of the anterior nasal cavity. The gingiva of the upper alveolar crest presented ulceration with irregular edges and sanious bottom. On palpation the consistency was hard-ligneous and swelling, in continuity with the bone, was slightly painful. Incisional biopsy enabled the diagnosis of chondrosarcoma to be confirmed.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1995

t(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: evaluation of three cases.

Three cases of extraskeletal myxoid chondrosarcoma with typical histologic and ultrastructural features were investigated cytogenetically. All three cases showed a reciprocal chromosome translocation characterized as t(9;22)(q22-31)(q11-12), thus confirming the findings in three previously karyotyped cases of extraskeletal myxoid chondrosarcoma in the literature. These data add significantly to the evidence of t(9;22) being diagnostic for extraskeletal myxoid chondrosarcoma. Other previously published cases with a range of complex karyotypes were less well defined morphologically. In cases with limited diagnostic material this karyotype might facilitate distinction from myxoid liposarcoma, which consistently shows t(12;16). Clear cell sarcoma, Ewing's sarcoma/primitive neuroectodermal tumor, and desmoplastic round cell tumor also show involvement of chromosome 22 with formation of a hybrid gene between the Ewing's sarcoma gene on band q12 and a transcription factor gene. Whether rearrangement of the Ewing's sarcoma gene is also present in extraskeletal myxoid chondrosarcoma is not clear at present. Cloning of the (9;22) translocation might provide important clues to the pathogenesis of this type of chondrosarcoma.

Adult↗

PubMed1997

Chondrosarcoma of small bones of the hands and feet.

BACKGROUND: Cartilaginous tumors of the hands and feet are not uncommon. Most are enchondromas, but they tend to show high cellularity, enlargement of nuclei, and many double-nucleated cells. Hence, differentiation between a benign lesion and chondrosarcoma may be difficult. METHODS: The files of patients treated at the Mayo Clinic and the consultation files were reviewed for examples of chondrosarcoma of small bones of the hands and feet. Histologic features and clinical charts were reviewed in all cases, and radiographs were reviewed in 111 cases. RESULTS: Seventy-five lesions involved the feet and 88 involved the hands. Bones of the fifth finger and the calcaneus were the most common sites of involvement. Nineteen tumors were secondary. Of the 104 intramedullary lesions studied radiologically, 96 showed cortical destruction, 83 a soft tissue mass, and 52 a permeative lytic pattern. Histologically, soft tissue extension and permeation of preexisting bone indicated malignant disease. One hundred sixteen tumors were Grade 1, 44 were Grade 2, and 3 were Grade 3. Of the 12 patients with distant metastasis from chondrosarcoma, 7 died of disease. Chondrosarcomas of the calcaneus and the talus were more likely to metastasize. CONCLUSIONS: Chondrosarcoma of small bones of the hands and feet has the potential to be fatal.

Adolescent↗

PubMed1998

Primary chondrosarcoma of long bones and limb girdles.

BACKGROUND: Chondrosarcomas are common solid malignant tumors of bone, second in incidence only to osteosarcomas. The biologic evolution of chondrosarcomas is slow, requiring long follow-up intervals for meaningful survival analysis. METHODS: This study describes the clinicopathologic profiles of 344 patients, 194 male and 150 female (M:F, 1.3:1.0), with primary chondrosarcoma of long bones and limb girdles seen at 1 institution over a period of 80 years. RESULTS: The average age at presentation was 46 years (range, 5-82 years). The pelvis was the most common location (1.7% of all patients). Local pain was the most frequently reported initial symptom (81.4%). Survival analysis was limited to 233 patients whose primary treatment was given at the Mayo Clinic. All 233 patients had potential follow-up of at least 5 years. The overall 5-year survival rate was 77% (the expected rate was 96%). Local recurrence developed in 19.7% of patients and metastatic lesions in 13.7%. The recurrence rate was higher for tumors of the shoulder and pelvis than for tumors of long bones. Radiographically, chondrosarcomas had a characteristic appearance, including a combination of bone expansion and cortical thickening. Entering the tumor at surgery increased the risk of local recurrence. Histologic tumor grade was an important predictor of local recurrence and metastasis. CONCLUSIONS: With adequate initial surgical intervention, chondrosarcoma is primarily a local disease with a low metastatic rate.

Adolescent↗

PubMed2006

Dedifferentiated chondrosarcoma with leukocytosis and elevation of serum G-CSF. A case report.

BACKGROUND: G-CSF is known to function as a hematopoietic growth factor and it is known to be responsible for leukocytosis. G-CSF-producing tumors associated with leukocytosis include various types of malignancies. CASE PRESENTATION: We report the case of a 72-year-old man with dedifferentiated chondrosarcoma characterized by dedifferentiated components of malignant fibrous histiocytoma- or osteosarcoma-like features in addition to conventional chondrosarcoma, arising from his pelvic bone. After hemipelvectomy, when local recurrence and metastasis were identified, leukocytosis appeared and an elevated level of serum granulocyte-colony-stimulating factor (G-CSF) was also recognized. The patient died of multiple organ failure 2 months after surgery. Autopsy specimens showed that the histological specimens of the recurrence and metastasis were dedifferentiated components, without any conventional chondrosarcoma components. G-CSF was expressed only in the dedifferentiated components, not in the chondrosarcoma components, immunohistochemically. CONCLUSION: This is the first report of chondrosarcoma, or any other primary bone tumor, with leukocytosis, probably stimulated by tumor-produced G-CSF from the dedifferentiated components.

Journal Article↗

PubMed1996

Influence of intralesional surgery on treatment outcome of chondrosarcoma.

BACKGROUND: Intralesional (IL) surgery for chondrosarcoma is an inadequate procedure; however, sometimes it cannot be avoided. The outcome of 26 patients (PTS) who underwent IL surgery for chondrosarcoma was analyzed. METHODS: Median follow-up time was 146.5 months. Eighteen tumors were located in the axial skeleton and 8 in the extremities. The histologic grade distribution of the chondrosarcoma was: 14 Grade 1, 8 Grade 2, and 4 Grade 3. Diagnostic problems were noted in 4 of the 18 central tumors and 7 of 8 extremity tumors. RESULTS: The 20-year relapse free survival rate for the 26 PTS who underwent IL surgery was 7% and was 64% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL surgery was 68% and was 66% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL (P = 0.2266). The overall survival rates for the PTS with tumors of histologic Grade 1 and 2 or 3 tumor was 85% and 44%, respectively (P = 0.02817). The overall survival rates for PTS with central tumors and extremital tumors were 57% and 87%, respectively (P = 0.0968). CONCLUSIONS: IL surgery for chondrosarcoma often leads to local relapse. However, local relapse does not always result in metastases and death. The prognosis for PTS after IL surgery tends to differ according to tumor grade. After local relapse further limb salvage becomes difficult and many complications may occur.

Adult↗

PubMed1998

Skeletal and extraskeletal myxoid chondrosarcoma: a comparative clinicopathologic, ultrastructural, and molecular study.

BACKGROUND: Skeletal myxoid chondrosarcoma (SMC) is considered to be either a typical chondrosarcoma with prominent myxoid alterations or an altogether unique malignant cartilage tumor. Extraskeletal myxoid chondrosarcoma (EMC) is a relatively rare but well-recognized neoplasm. It was initially thought to be a low grade sarcoma of cartilage derivation and was recently found, in most cases, to contain a reciprocal t(9;22), resulting in a fusion of the EWS and CHN genes. Are SMC and EMC the same entity arising in two different locations, or are they two separate entities? To the authors' knowledge, this study represents the first systematic attempt to answer this question. METHODS: Forty consecutive cases of EMC (20 cases) and SMC (20 cases) were compared by light and electron microscopy, immunohistochemistry, and molecular analysis. The mean clinical follow-up for both groups was 55 months. Histologic criteria for SMC consisted of 95% myxoid matrix, with only minimal hyaline cartilage formation. RESULTS: The gender distribution was identical in both groups (13 males and 7 females). The mean age was 55 years for EMC patients and 45 years for SMC patients. The EMC tumors were predominantly located in the deep soft tissues of the lower extremity (60%) and buttock (20%), and the mean tumor size was 13 cm. SMC was most commonly located in the bones around the hip joint (pelvis 35%; proximal femur 20%) and shoulder (20%); the mean size was 9 cm. Histologic grade in the EMC group correlated with survival (82% of the high grade tumors metastasized). Electron microscopy performed in 8 EMC cases revealed intracisternal microtubules in 3 cases and prominent mitochondria in 5, whereas in 5 SMC cases it revealed only inconspicuous organelles. Molecular analysis for the EWS-CHN fusion RNA resulting from the t(9;22) was performed in 15 cases (9 EMC and 6 SMC) and was detected in 7 of 9 EMC cases and 0 of 6 SMC cases. In one case, the molecular structure of the EWS-CHN fusion RNA was novel. The probability of metastasis was significantly higher (P=0.004) for the EMC group than for the SMC group. CONCLUSIONS: Although similar light microscopic features are noted in EMC and SMC, fundamental differences are noted at the ultrastructural and molecular levels, suggesting that EMC and SMC represent two distinct entities in the chondrosarcoma family of tumors.

Adolescent↗

PubMed1997

Mouse CD-RAP/MIA gene: structure, chromosomal localization, and expression in cartilage and chondrosarcoma.

A cDNA encoding a novel protein has been previously isolated from two independent sources: melanoma cell cultures and chondrocytes. The protein from human melanoma cell lines and tumors is called melanoma inhibitory activity (MIA) (Blesch et al. [1994] Cancer Res. 54:5695-5701) and the protein from primary bovine chondrocytes and cartilaginous tissues is called cartilage-derived retinoic acid-sensitive protein (CD-RAP) (Dietz and Sandell [1996] J. Biol. Chem. 271:3311-3316). In order to investigate the gene regulation and function of CD-RAP/MIA, the mouse gene locus was isolated and analyzed. Developmental expression was determined by in situ hybridization to mouse embryos. Expression was limited to cartilaginous tissues and was initiated with the advent of chondrogenesis, remaining abundant throughout development. The mouse gene was isolated and sequenced from a 129Sv library and sequenced directly from an additional strain, B6C3Fe. The mouse CD-RAP/MIA gene is 1.5 kbp and consists of four exons. The promoter sequence of the gene contains many potential regulatory domains including 8 basic helix-loop-helix protein-binding domains and an AT-rich domain, both motifs shown to be present in the cartilage-specific enhancer of the type II procollagen gene. Other potential cis-acting motifs include binding sites for GATA-1, NF-IL6, PEA3, w-elements, NF kappa B, Zeste and Sp1. The gene, called cdrap, was localized to the end of an arm of chromosome 7 at the same site as the transforming growth factor beta 1 (Tgf-beta 1) and the glucose phosphate isomerase 1 (Gpi 1) genes. Potential mouse mutants that mapped to the same region of chromosome 7 were identified. Two of the potential mutants with skeletal phenotypes were sequenced, pudgy (pu) and extra toes with spotting (XsJ); however, no mutations were found in the coding sequence. To determine whether CD-RAP/MIA is associated with tumors of cartilage, mRNAs from a variety of rodent tissues and cell lines were screened. Expression was detected in a rodent tumor, the Swarm rat chondrosarcoma and a chondrosarcoma cell line derived from it, but not in other tissues or tumors of non-cartilage origin. Immunolocalization revealed CD-RAP/MIA protein localized in cartilage only. These results show that the normal expression of CD-RAP/MIA is limited to cartilage; however, pathologically, it is expressed both in melanoma and chondrosarcoma. The restricted expression of CD-RAP/MIA may provide an opportunity to monitor cartilage metabolic activity as well as the tumor activity of melanoma and chondrosarcoma.

Amino Acid Sequence↗

PubMed2000

Changes of the p16 gene but not the p53 gene in human chondrosarcoma tissues.

The role of two important tumour suppressor genes, p16 and p53, was evaluated in cartilaginous tumour tissues. Genomic DNA from 22 chondrosarcomas, 5 benign chondroid tumours, 1 sample of reactive proliferative cartilage and 2 samples of normal cartilage were analysed using polymerase chain reaction, single strand conformational polymorphism, DNA sequencing and methylation-specific polymerase chain reaction. The p16 gene was found to be partly methylated in 5 high-grade chondrosarcomas and homozygously deleted in 1 chondrosarcoma. Moreover, a polymorphism was detected in 3 malignant tumours, but not in benign tumours or normal cartilage. Analysis of the p53 gene revealed an unchanged structure in all samples. These findings show a role for p16, but not p53, in chondrosarcoma.

Adolescent↗

PubMed2000

Fine-needle aspiration of dedifferentiated chondrosarcoma of the larynx.

We present a case of dedifferentiated chondrosarcoma of the larynx in which the dedifferentiated component was initially diagnosed by fine-needle aspiration (FNA). The patient was a 74-yr-old man who presented with difficulty breathing and an anterior neck mass. A CT scan demonstrated a 4.5-cm cartilaginous lesion involving the left thyroid cartilage, with an anterior soft-tissue component. Nine years prior, the patient had an incomplete resection of a low-grade chondrosarcoma at the same site. FNA was performed on the current lesion, demonstrating a high-grade spindle-cell sarcoma with a storiform pattern. The cytomorphology together with immunocytochemistry and electron microscopy were diagnostic of malignant fibrous histiocytoma (MFH), and synthesis of the clinical, radiographic, and cytomorphologic features resulted in a diagnosis of dedifferentiated chondrosarcoma. The cytologic diagnosis was histologically confirmed by laryngectomy. Although rare, dedifferentiated chondrosarcoma should be included in the differential diagnosis of high-grade sarcomas of bone and cartilage assessed by FNA.

Aged↗

PubMed1980

The diagnosis and grading of chondrosarcoma of bone: a combined cytologic and histologic approach.

The diagnosis and grading of chondrosarcoma of bone is best established by a complementary study of its cytology, as observed in smeared preparations, and of its tissue structure and relationship to the host bone, as observed in histologic sections. Smeared preparations demonstrate the cytologic features of chondrosarcoma with greater clarity and detail than is possible with histologic sections. Histology is particularly helpful in the diagnosis of low-grade chondrosarcoma, through the demonstration of its invasive activity in relation to the host bone. The importance of grading chondrosarcoma is emphasized because of its relevance to the biological behaviour and prognosis of this tumor.

Anaplasia↗

PubMed1995

p53 expression in dedifferentiated chondrosarcoma.

BACKGROUND: p53 acts as a tumor suppressor gene because of to its negative control of the cell cycle and its central role in programmed cell death. It frequently is mutated, as observed in a variety of human neoplasms. The mutations inhibit tumor-suppressor activities of p53, which may gain a new function of tumor promotion. In this study, p53 was investigated in various components of dedifferentiated chondrosarcoma and correlated with their proliferative activities. METHODS: Immunohistochemical assays for p53, Ki-67, and proliferating cell nuclear antigen (PCNA) were used in a series of eight dedifferentiated chondrosarcomas of bone. The cartilaginous component was low grade (Grade I-II) in five cases. It was predominantly low grade with foci of a high grade (Grade III) chondrosarcoma in the remaining three cases. The noncartilaginous (dedifferentiated) high grade component consisted of malignant fibrous histiocytoma in five cases and osteosarcoma in three. RESULTS: Regardless of the histological type, diffuse strong nuclear staining for p53 occurred in the high grade noncartilaginous component of all eight of the tumors. The low grade cartilaginous component of six cases was negative for p53, with focal weak staining in the two remaining cases. The high grade cartilaginous component showed strong positive staining for this protein in all three cases. Ki-67 and PCNA expression were similar to that of p53. CONCLUSIONS: The percentage of p53 positive staining roughly was parallel to the proliferating fraction of cells in various components of dedifferentiated chondrosarcoma. Moreover, p53 overexpression was consistently present in the high grade noncartilaginous (dedifferentiated) component of the tumor and was accompanied by increased proliferative activity.

Cell Differentiation↗

PubMed1995

Chondrosarcoma of the jaw and facial bones.

BACKGROUND: Osteosarcomas of the jaw frequently have chondroblastic differentiation, causing confusion with chondrosarcomas. METHOD: Clinicopathologic features and results of treatment were analyzed for a series of 56 patients (27 males and 29 females from 1.5 to 88 years of age) with chondrosarcoma of jaw and facial bones. Twelve patients (21.4%) were younger than 20 years. RESULTS: The major symptom was nasal obstruction or a painless mass; the median interval from the first symptom until initial treatment was 1 year. Of the 56 chondrosarcomas, 25(44.6%) involved the alveolar portion of the maxilla and maxillary sinus; 23 (41.1%) involved the nasal septum, ethmoid, and sphenoid; 6 (10.7%) involved the mandible; and 2 (3.6%) involved the nasal tip. Of the 19 patients with radiographic studies, 15 (78.9%) had an expanding soft tissue mass with varied matrix calcification and destruction of bone and 2 had a purely lytic lesion. The lesion was difficult to assess in the two others. Most tumors had a lobulated growth pattern of hyaline cartilage. Hypercellularity, nuclear pleomorphism, and binucleation were common features. Forty-three tumors were grade 1, 13 were grade 2, and none were grade 3. Modalities of treatment were known for 51 of the 56 patients. Forty-six patients (90.2%) had surgical treatment, 2 (3.9%) had combination radiation therapy and chemotherapy, 1 (2%) had radiation therapy alone, and 2 (3.9%) had biopsy only. Follow-up adequate for analysis was obtained for 42 patients. Of these, 14 (33.3%) had local recurrence; uncontrolled recurrence developed in 9 (21.4%) patients. No distant metastases were documented. Overall actuarial survival at 5, 10, and 15 years was 80.7%, 65.3%, and 56%, respectively. Survival was analyzed for location, size, and histologic grade of tumor. No statistically significant differences were found. CONCLUSIONS: Chondrosarcomas of the jaw and facial bones are extremely rare, locally aggressive tumors.

Adolescent↗

PubMed2002

Prognostic relevance of cell biologic and biochemical features in conventional chondrosarcomas.

BACKGROUND: Conventional chondrosarcoma is the second most common malignant solid tumor of bone, and its management still poses a challenge for the orthopedic surgeon. Currently, tumor grade is the only parameter of prognostic significance besides stage and, possibly, resection margins. Additional independent prognostic markers therefore would be highly valuable for patient management. METHODS: In the current study, the authors evaluated biologic markers for various chondrocytic phenotypes by histochemical and immunohistochemical technology in a large series of clinically well defined cases of enchondromas and conventional chondrosarcomas, each with at least 5 years of clinical follow-up. RESULTS: The authors' results confirm the strong correlation between clinical behavior and cell differentiation as expressed by marker genes. The phenotypes of the tumor cells are the biologic substrate of the histopathologic appearance of the neoplasms and, thus, the biologic basis for classic tumor grading. Collagen Types II and X, as well as the proteoglycan aggrecan, suggest a mature neoplastic phenotype and good prognosis, i.e., low recurrence rate, rare metastasis, and long survival. Conversely, collagen Type I, together with cell spindling, indicates a transition to a more proliferative, so-called "dedifferentiated" phenotype, which clearly is associated with a poorer prognosis. The changes in cellular phenotypes are accompanied by changes in proliferative activity. Thus, low-grade neoplasms showing mainly mature and terminally differentiated (hypertrophic) chondrocytes display only scant proliferation whereas less differentiated chondrosarcomas with biologically dedifferentiated chondrocytes show significantly higher proliferative activity, a feature that is also highly correlated with prognosis. CONCLUSIONS: These data indicate that molecular markers are to a large extent the biologic basis of the conventional grading, rather than representing independent prognostic markers. The authors' results further indicate that COL1 has significant value in the distinction between enchondromas and low-grade chondrosarcomas including these that are histologically similar. Further understanding of chondrocytic phenotypes will be a promising way to provide new tumor markers for better understanding, diagnosis, and treatment of chondroid neoplasms.

Biomarkers, Tumor↗