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PubMed1999

Expression of tau proteins and tubulin in extraskeletal myxoid chondrosarcoma, chordoma, and other chondroid tumors.

Tau proteins are microtubule-associated proteins required for the polymerization of tubulin. The abnormal accumulation of tau proteins in neurofibrillary tangles is a well-known phenomenon and has been studied extensively. However, the role of tau protein in chondroid tissue and its neoplasia is unknown. In the present study, 2 extraskeletal myxoid chondrosarcomas (EMCs), 6 chordomas, 6 chondrosarcomas, 3 myxoid chondrosarcomas of bone, 2 osteochondromas, 6 chondroblastomas, and 2 nonneoplastic adult articular cartilages were immunostained with monoclonal antibodies against tau proteins and tubulin. The results showed that the coexpression of tau proteins and tubulin was present only in EMCs (2/2) and chordomas (4/6). Although tubulin was detected in chondroblastomas (5/6), osteochondromas (2/2), chondrosarcomas (5/6), and myxoid-chondrosarcomas of bone (3/3), tau expression was absent in these tumors. The perichondrial chondroblasts but not chondrocytes from nonneoplastic articular cartilage also localize tau and tubulin with a much weaker staining intensity. The results mainly demonstrate that there is frequent expression of tau proteins in some microtubule-rich neoplasms, such as EMC and chordoma. The different immunostaining pattern of tau proteins between chordoma and myxoid chondrosarcoma of bone may be useful in the differential diagnosis, especially when both neoplasms occur in the base of skull.

Adult↗

PubMed1996

Dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation.

Dedifferentiated chondrosarcomas are primary bone tumors characterized by the presence of both low-grade cartilaginous and high-grade sarcomatous components. The high-grade component usually shows histologic features of either malignant fibrous histiocytoma or fibrosarcoma. We are aware of only 10 published cases in which the high-grade component showed rhabdomyosarcomatous differentiation. To further clarify the clinical, radiographic, and pathologic features of this unusual variant, we report three additional cases of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation. The patients included two men and one woman; their mean age was 63 years. Tumors originated in the pelvis (ilium), scapula, and tibia. Two patients presented with radiographic findings typical of dedifferentiated chondrosarcoma, including a geographic, lytic lesion with areas of mineralization suggestive of cartilage in close association with a permeative component. The third patient presented with a primarily lytic, destructive lesion of the right iliac wing. Histologically, the tumors contained lobules of well-differentiated chondrosarcoma associated with a high-grade sarcoma with prominent rhabdomyoblasts. Immunohistochemical stains for actin and desmin were positive in all three tumors, and electron microscopy revealed evidence of skeletal muscle differentiation. All three patients died with metastatic disease, 1, 6, and 12 months postoperatively. This histologic variant of dedifferentiated chondrosarcoma is rare, but it shows radiographic and clinical features similar to "conventional" dedifferentiated chondrosarcoma, including a very poor prognosis.

Aged↗

PubMed1999

Parachordoma is immunohistochemically and cytogenetically distinct from axial chordoma and extraskeletal myxoid chondrosarcoma.

Parachordoma is a rare soft-tissue tumor resembling extraskeletal myxoid chondrosarcoma and chordoma. Because fewer than 30 cases have been reported and precisely characterized, we studied the clinicopathologic features of seven new cases, comparing the immunohistochemical (six cases) and cytogenetic (one case) profiles with 15 cases of chordoma and six cases of extraskeletal myxoid chondrosarcoma. Patients with these tumors ranged in age from 7 to 62 years (mean, 35 years) and included four women and three men. The tumors presented as subfascial masses of the thigh (two cases), arm (three cases), chest wall (one cases), and buttocks (one case). In six of seven cases, there was neither recurrence nor metastasis within the follow-up, which ranged from 4 months to 7 years. The tumors were composed of vague nodules of large, rounded eosinophilic cells embedded in a matrix that varied from myxoid to densely hyaline, and the latter areas occasionally resembled primitive cartilage. Transitions between the large eosinophilic cells and smaller rounded and shorter spindled ones were often noted. Multivacuolated (physaliferouslike) cells were noted in all cases but were usually few in number. The matrix stained with Alcian blue (pH 2.5), and this staining was abolished with hyaluronidase predigestion. Immunohistochemistry for a variety of cytokeratins (CKs) (8/18, 1/10, 7, and 20), epithelial membrane antigen (EMA), S-100 protein, vimentin CD34, type IV collagen, smooth muscle actin, smooth muscle myosin heavy chain, calponin, and glial fibrillary acid protein was performed. All parachordomas strongly expressed CK 8/18, but not the other cytokeratins. Additionally, they expressed EMA (five of six). S-100 protein (six of six), and vimentin (six of six) and had a linear pattern of type IV collagen immunoreactivity around nests of cells (four of five). Calponin was noted in one case, but no cases expressed smooth muscle actin, smooth muscle myosin heavy chain, or glial fibrillary acid protein. In contrast, chordoma expressed CK 8/18 (15 of 15) and CK 1/10 (14 of 15), whereas extraskeletal myxoid chondrosarcoma consistently lacked CK. Although chordoma and extraskeletal myxoid chondrosarcoma showed considerable overlap with parachordoma, with respect to EMA and S-100 protein, they infrequently displayed type IV collagen, as was seen in parachordoma. One case of parachordoma studied cytogenetically disclosed trisomy 15, and monosomies of 1, 16, and 17 in contrast to the t(9;22) reported in extraskeletal myxoid chondrosarcoma and the monosomies of 3, 4, 10, and 13 seen in chordoma. We conclude that the immunohistochemical and cytogenetic profile distinguishes parachordoma from extraskeletal myxoid chondrosarcoma and chordoma. Lack of myoepithelial markers, furthermore, suggests parachordoma is not a deeply situated adnexal tumor. Because of these differences, parachordoma is best regarded as a distinct lesion without a clear relationship to other well-characterized tumors.

Adolescent↗

PubMed2001

Platelet-derived growth factor-alpha receptor expression supports the growth of conventional chondrosarcoma and is associated with adverse outcome.

Bone cells are important targets of platelet-derived growth factors (PDGFs) because they stimulate proliferation of osteoblasts and chondrocytes. In this study we wanted to determine the expression of PDGF-AA and PDGF-alpha receptor in conventional chondrosarcomas and to compare the results with those obtained from benign enchondromas and non-neoplastic cartilage tissue. Sixty-seven chondrosarcomas, 20 enchondromas, and 5 specimens of healthy cartilage as well as 7 specimens of hypertrophic callus cartilage were immunohistochemically analyzed for the expression of PDGF-AA and PDGF-alpha receptor, respectively. Additionally, the proliferation activity was examined with the MIB-1 antibody. Clinical follow-up data were available from 53 patients. A significant overexpression of receptor and factor was found in chondrosarcomas as compared with enchondromas (PDGF-AA p = 0.013, PDGF-alpha receptor p <0.001). MIB-1 values were significantly higher in chondrosarcomas (p <0.001). In healthy joint cartilage no staining was detectable, whereas reactive cartilage of callus formation showed high expression levels. PDGF-alpha receptor expression was significantly higher in grade 3 chondrosarcomas compared with grade 2 (p = 0.022) and grade 1 tumors (p = 0.002). Survival analysis documented a significantly shorter overall survival for patients with high PDGF-alpha receptor expression (p = 0.0172, log-rank test). Because PDGF-alpha receptor expression positively correlates with the aggressiveness of chondrosarcoma, it may be considered as a possible target for novel therapeutic strategies.

Adolescent↗

PubMed2001

Primary intradural classic chondrosarcoma: case report and literature review.

OBJECTIVE AND IMPORTANCE: The exact origin of rare intradural chondrosarcomas remains obscure. We present a case report of an intradural classic chondrosarcoma (a very rare subtype of chondrosarcoma in this location), with a review of the literature, in an attempt to clarify the histogenesis of these tumors. CLINICAL PRESENTATION: A 48-year-old man presented with a 12-month history of progressive right hemiparesis. Computed tomography and magnetic resonance imaging demonstrated a left parietal space-occupying lesion. INTERVENTION: The patient underwent an image-guided, left parietal parasagittal craniotomy. An extrinsic tumor, which seemed to arise from the dura, was macroscopically removed. There was no bone involvement. The histological examination revealed a Grade II classic chondrosarcoma with tumor infiltration into the dura. Adjuvant radiotherapy was administered. CONCLUSION: Intradural chondrosarcomas are rare tumors, the majority of which are mesenchymal. Classic chondrosarcomas in this location are much rarer. Their histogenesis is uncertain. In this case, the origin seems to be from the dura. Because of the malignant potential of these tumors, radical extirpation whenever possible, followed by radiotherapy, is indicated.

Chondrosarcoma↗

PubMed1998

p53 mutations in chondrosarcoma.

Chondrosarcoma is a primary bone tumor that has several different grades and variants. We evaluated 48 chondrosarcomas for p53 overexpression and p53 mutations. p53 expression was evaluated with immunohistochemistry using monoclonal antibodies PAb421, PAb1801, and PAb240. p53 mutations were identified with single-strand conformational polymorphism (SSCP) and DNA sequencing in selected cases. Immunohistochemistry revealed nuclear staining with PAb421 and PAb1801 in the spindle cell portion of one dedifferentiated chondrosarcoma. SSCP analysis was abnormal only in the case with positive immunostaining and localized the mutation to exons 7 and 8. DNA sequence analysis identified a point mutation of G to C in codon 276, resulting in an amino acid substitution of proline for alanine. This point mutation has been reported previously in other tumors but not in chondrosarcoma. Assimilation of our results with previous studies suggests that p53 mutations are present in a minority of chondrosarcomas but when present, are in higher grade chondrosarcomas and their variants.

Bone Neoplasms↗

PubMed2002

PTEN mutation is rare in chondrosarcoma.

Chondrosarcoma is the second most common primary malignant neoplasm of bone in adults, but the major genetic events involved in the progression of this often-fatal cancer remain to be elucidated. Loss of heterozygosity of chromosome 10q has been reported in 67% of chondrosarcoma. The tumor suppressor gene PTEN is located on chromosome 10q, specifically 10q23, raising the possibility that the loss of PTEN function is responsible for some chondrosarcomas. The authors examined 40 chondrosarcoma tumors and tumor-derived cell lines for alterations in PTEN. Only one mutation resulting in a truncated PTEN protein was detected, which was in a metastasized extraskeletal myxoid chondrosarcoma. Thus, mutated PTEN is an uncommon event in the development of chondrosarcoma. The high frequency of loss of heterozygosity on 10q suggests the presence of additional tumor suppressor genes at these loci.

Bone Neoplasms↗

PubMed2004

Chondroma and chondrosarcoma of the larynx.

PURPOSE OF REVIEW: The purpose of this review is to give a detailed description of chondroma and chondrosarcoma of the larynx and to put into perspective new findings from recent literature. RECENT FINDINGS: Recent findings indicate that chondroma and chondrosarcoma of the larynx are closely related, either synchronously (areas of both tumor types within the same lesion) or metachronously (malignant transformation of chondroma over time). It is questioned whether the grading of Lichtenstein and Jaffe, and Evans et al. should be used in the larynx, because these grading systems were designed for chondrosarcoma of the long bones. Chondrosarcomas of the larynx seems to behave more like chondrosarcomas of the phalanx, because they too hardly ever metastasize or cause tumor-related deaths. Both CT and MRI play an important role in the workup of these tumors, but they cannot be used to differentiate between both tumor types. No new treatment modalities have emerged recently and surgery is still the treatment of choice. Tracheal autotransplantation may play an increasing role in the treatment of cricoid tumors because it allows for wide resection and optimal revalidation. SUMMARY: It is now firmly established that metastasis in chondrosarcoma is a poor prognostic sign, but is very rare. Without metastasis the prognosis is excellent. Cartilaginous tumors of the larynx are rare. Clinical, radiologic and pathologic characteristics of these tumors are described and a review of treatment modalities is presented. Wide excision with clear margins is usually indicated. The authors recommend that the management of cartilaginous tumors of the larynx be multidisciplinary and centralized in dedicated referral centers.

Calcinosis↗

PubMed2003

Grade 2 chondrosarcoma: stage I or stage II tumor?

We analyzed chondrosarcomas of bone to assess whether Grade 2 tumors are more appropriately grouped with Grade 1 chondrosarcomas or Grade 3 and dedifferentiated chondrosarcomas (Musculoskeletal Tumor Society Stage I or Stage II). A retrospective chart review identified 109 patients who presented with nonmetastatic chondrosarcoma. Data were gathered on each tumor's histologic grade, extent (intracompartmental versus extracompartmental), resection margin status, and site (axial versus appendicular). The cohort included 60 males and 49 females with a mean age of 50 years. Forty tumors were located in the axial skeleton and the remainder were distributed throughout the appendicular skeleton. Fifty-three tumors were Grade 1, 40 tumors were Grade 2, eight tumors were Grade 3, and eight tumors were dedifferentiated. Statistical analysis established grade as the only significant variable for this group of patients; extracompartmental tumor spread also correlated with outcome, but not independent of the grade. Margin status and site were not statistically significant with respect to outcome. Chi-square analysis also established that Grade 2 tumors are grouped more appropriately with the Grade 1 chondrosarcomas as Musculoskeletal Tumor Society Stage I neoplasms. These data should be considered when developing the surgical plan, particularly for patients with Grade 2 appendicular chondrosarcomas.

Adolescent↗

PubMed2003

Microtubule-associated protein-2 and class III beta-tubulin are expressed in extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma of uncertain histogenetic origin. Because recent reports have indicated neural-neuroendocrine differentiation in some extraskeletal myxoid chondrosarcomas, we investigated 25 tumors for expressions of microtubule-associated protein-2 and Class III beta-tubulin, which are major components of microtubules and specifically localized in neurons and their derivatives. Immunohistochemical expression of microtubule-associated protein-2 and Class III beta-tubulin was studied in extraskeletal myxoid chondrosarcomas using formalin-fixed, paraffin-embedded tissues. Cytoplasmic expressions of microtubule-associated protein-2 and Class III beta-tubulin were detected in 21 (84%) and 13 (52%) of the 25 extraskeletal myxoid chondrosarcomas, respectively, although the number of positively stained tumor cells varied. Expression of the Class III beta-tubulin gene was also assessed in two immunohistochemically positive cases by in situ hybridization using an oligonucleotide probe specific for its transcript, and both cases showed expression of Class III beta-tubulin transcript. Another case was examined with immunoelectron microscopy, and immunogold particles for Class III beta-tubulin were localized to microtubular aggregates. Our data indicate that microtubules in extraskeletal myxoid chondrosarcoma are similar to those found in neurons, further supporting the concept that neural-neuroendocrine differentiation occurs in a significant number of extraskeletal myxoid chondrosarcoma.

Chondrosarcoma↗

PubMed2005

The MAPK-AP-1/-Runx2 signalling axes are implicated in chondrosarcoma pathobiology either independently or via up-regulation of VEGF.

AIMS: To investigate whether and how the JNK/ERK-AP-1/-Runx2 signalling pathways and vascular endothelial growth factor (VEGF) are engaged in the pathogenesis of cartilaginous tumours. Chondrosarcoma is the third most common primary skeletal malignancy. Nevertheless, the molecular events underlying its pathogenesis remain elusive. JNK/ERK MAPKs and their downstream effectors, c-Jun and c-Fos (AP-1), are involved in chondroblastic differention/proliferation. These proteins interact with the Runx2 transcription factor, which is also implicated in chondroblast biology. VEGF, a key angiogenic factor, is up-regulated in human chondrosarcomas. METHODS AND RESULTS: Normal cartilage and neoplastic cells from 45 chondrosarcomas and 21 enchondromas were investigated immunohistochemically. We evaluated the cellular levels of JNK2, p-JNK2 (phosphorylated/activated JNK2), its main substrate, c-Jun, pc-Jun (phosphorylated/activated c-Jun) and c-Fos. Moreover, the levels of p-ERK (phosphorylated/activated ERK), Runx2 and VEGF were assessed. Positive immunostaining for all proteins was observed in the majority of the examined chondrosarcomas and in a small fraction of enchondromas. The expression levels of all proteins were positively and significantly correlated with each other. These levels were substantially augmented in high-grade compared with low-grade chondrosarcomas and in low-grade tumours compared with benign enchondromas, implying a potential use as molecular markers for prediction of high-grade neoplasms. CONCLUSIONS: The JNK/ERK-AP-1/-Runx2 signal transduction 'network' is associated with chondroblastic malignant transformation and chondrosarcoma development, either separately or through coordinated induction of VEGF.

Adult↗

PubMed1997

Dedifferentiated chondrosarcoma of the rib with a malignant mesenchymomatous component: an autopsy case report.

A rare variant of dedifferentiated chondrosarcoma with malignant mesenchymomatous component in a 57-year-old male is reported. The patient presented with a posterior mediastinal mass arising from the left eighth and ninth ribs showing well differentiated, low-grade chondrosarcoma. Five years later, local recurrence occurred and an excised specimen also showed the same histological features as the primary tumor. Another 6 years later, the tumor recurred and metastasized to the multiple organs, the patient dying 4 months later. Autopsy revealed that the recurrent and metastatic tumors showed malignant mesenchymomatous 'dedifferentiation' of chondrosarcoma composed of rhabdomyosarcoma, angiosarcoma, chondrosarcoma, osteosarcoma, and leiomyosarcoma, in addition to fibrosarcomatous areas. Although the less differentiated component of dedifferentiated chondrosarcoma usually shows the histological features of malignant fibrous histiocytoma and fibrosarcoma, multilineage differentiation can occur in that component. The phenomenon of 'dedifferentiation' in chondrosarcoma and the relationship to and distinction from malignant mesenchymoma of soft tissue and bone are discussed.

Autopsy↗

PubMed2005

Primary bone carcinosarcoma: chondrosarcoma and squamous cell carcinoma with keratin pearl formation.

Malignant bone tumors with epithelial differentiation are extremely rare. Only one case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma has ever been reported. Reported herein is a case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma, so-called carcinosarcoma of bone, arising in the femur of a 53-year-old man. The tumor was located within the femur and was diagnosed by curettage as a well-differentiated chondrosarcoma. No primary tumor was detected in any other organ. Within a few months the tumor had rapidly grown toward the soft tissue, and hemipelvectomy was performed. Examination of the surgical specimen revealed that the tumor was mainly composed of undifferentiated spindle sarcoma cells with scattered foci of chondrosarcoma and of squamous cell carcinoma with keratin pearl formation. The patient died approximately 6 months postoperatively. At autopsy multiple metastases were detected in the heart, both lungs, muscles, and lymph nodes. Interestingly, the chondrosarcoma and squamous cell carcinoma components were observed in several metastatic foci. The tumors in both the previously reported case and the present case contained components of chondrosarcoma and squamous cell carcinoma with keratin pearl formation, and this combination of histological features may be a unique characteristic of carcinosarcoma of bone.

Bone Neoplasms↗

PubMed2004

Absence of a correlation between the presence of a single nucleotide polymorphism in the matrix metalloproteinase 1 promoter and outcome in patients of chondrosarcoma.

PURPOSE: Increased levels of matrix metalloproteinase 1 (MMP-1) expression have been associated with poor outcome in chondrosarcoma. The existence of a single nucleotide polymorphism creating an Ets-binding site in the MMP-1 promoter may be one mechanism for elevated MMP-1 transcription. The aim of our study was to identify the prevalence of this single nucleotide polymorphism (SNP) in chondrosarcoma patients, to determine its correlation with disease outcome, and to discern whether it could serve as a prognostic marker in patients with chondrosarcoma. EXPERIMENTAL DESIGN: Sixty-seven chondrosarcoma specimens were selected sequentially from an established tumor bank with a median duration of 47 months follow-up (range, 24 to 179 months). DNA was extracted, amplified with PCR, and sequenced to determine presence (GG) or absence of the Ets-binding site created by the SNP. RESULTS: Eighteen (27%) samples were homozygous for the absence of the Ets site, 34 (51%) were heterozygous for the SNP, and 15 (22%) were homozygous for the SNP. The 5-year overall survival rate for patients was 78, 80, and 84%, respectively (P = 0.5527). The disease-free survival rate was 16, 63, and 76%, respectively (P = 0.0801). The 5-year disease-free survival rate for patients with the homozygous G/G genotype was 16%, compared with 71% for patients who were either homozygous or heterozygous for the GG allele (P = 0.0444). CONCLUSIONS: Despite a statistical correlation between MMP-1 gene expression and outcome in chondrosarcoma, this study demonstrates an absence of a correlation between the presence of the SNP and prognosis in patients with chondrosarcoma.

Adult↗

PubMed2005

Estrogen signaling is active in cartilaginous tumors: implications for antiestrogen therapy as treatment option of metastasized or irresectable chondrosarcoma.

PURPOSE: Chondrosarcoma is a malignant cartilaginous matrix-producing tumor that can be lethal in 10% to 50% of the patients. Surgery is the only effective treatment known as these tumors are notorious refractory to all types of conventional chemotherapy or radiotherapy. To identify a target for therapy, we want to determine whether estrogen signaling is active in chondrosarcoma because estrogen is important in the regulation of longitudinal growth that is initiated by chondrocyte proliferation and differentiation in the epiphyseal growth plate of long bones. EXPERIMENTAL DESIGN: We studied protein expression of the estrogen receptor in 35 cartilaginous tumors as well as mRNA levels for the estrogen receptor and for aromatase, an enzyme for estrogen synthesis and another potential therapeutic target. Furthermore, the activity of aromatase was determined in vitro by the tritiated water release assay. Dose-response experiments with chondrosarcoma cultured cells were done with estrogen, androstenedione, and exemestane. RESULTS: All chondrosarcomas tested showed mRNA and nuclear protein expression of the estrogen receptor. Also, aromatase mRNA was detected. The aromatase activity assay showed a functional aromatase enzyme in primary chondrosarcoma cultures and in a cell line. Growth of chondrosarcoma cell cultures can be stimulated by adding estrogen or androstenedione, which can be inhibited by exemestane. CONCLUSIONS: These results show, on the RNA, protein, and cell biological levels, that the ligand and the receptor are active in estrogen-mediated signal transduction. This observation implicates potential use of targeted drugs that interfere with estrogen signaling, such as those applied for treating breast cancer.

Adult↗

PubMedDate not supplied

Extraskeletal myxoid chondrosarcoma of the chest wall masquerading as a breast tumor. A case report.

BACKGROUND: Extraskeletal myxoid chondrosarcoma is a rare tumor that usually occurs in the soft tissues of extremities. Cytologic features of chondrosarcoma arising from a rib and presenting as a breast mass were reported by Molyneux et al in 1995. However, to the best of our knowledge, the cytology of extraskeletal myxoid chondrosarcoma of the chest wall presenting as a breast mass has not been documented before. CASE: A 30-year-old female presented with left-sided chest pain and a hard lump in the breast of two months' duration. Fine needle aspiration cytology was done, and a possible diagnosis of mucinous carcinoma or mixed tumor of the breast was suggested, with advice to prepare a frozen section before undertaking a radical procedure. On imprint cytology and frozen section a diagnosis of extraskeletal myxoid chondrosarcoma was made and confirmed by histopathology and immunohistochemistry. CONCLUSION: It is rare for extraskeletal myxoid chondrosarcoma to occur in the chest wall. This problem is compounded by the fact that cells of extraskeletal myxoid chondrosarcoma which resemble epithelial cells, can very closely mimic some malignant breast tumors.

Adult↗

PubMed1997

Bilateral renal metastases of nasal chondrosarcoma in a dog.

Chondrosarcoma is the most common nonepithelial sinonasal neoplasm in the dog, and metastasis is considered rare. A 7-year-old Irish Setter had bilateral renal enlargement 17 months following surgery and radiotherapy for a primary nasal chondrosarcoma. Histologic evaluation revealed chondrosarcoma metastases in both kidneys. A diagnosis of nasal chondrosarcoma with bilateral renal metastasis was made. The clinical importance of this report is that routine recommendations for the evaluation of regional and/or distant metatasis in a dog with a dignosis of nasal chondrosarcoma, namely routine whole body physical examination and thoracic radiography, failed to demonstrate the presence of abdominal metatases, which ultimately led to the demise of this dog. The biologically aggressive nature of this chondrosarcoma of the nasal cavity indicates that additional information is needed before a prognosis can be reliably established for dogs with this tumor type.

Animals↗

PubMed2004

Minichromosome maintenance protein (MCM6) in low-grade chondrosarcoma: distinction from enchondroma and identification of progressive tumors.

The distinction between chondrosarcoma and enchondroma is difficult, and no reliable immunohistochemical or molecular methods are available. Differentiation is important because the therapeutic consequences range from radiologic followup to radical operation. We studied 17 chondrosarcomas (12 grade 1) and compared them with 14 enchondromas immunohistochemically by using the monoclonal antibodies Ki-MCM6 (minichromosome maintenance protein 6), Ki-S5 (Ki-67), and Ki-S2 (repp86), in addition to the established clinical criteria. In comparison with the other markers, Ki-MCM6 proved more effective at identifying proliferative activity in grade 1 chondrosarcomas. The MCM6 labeling index correlated with tumor grade and was significantly increased in grade 1 chondrosarcomas compared with enchondromas. The 5 cases of progressive chondrosarcoma also had a significantly higher MCM6 labeling index than the nonprogressive cases. Furthermore, by means of the MCM6 labeling index, many cases of progressive disease were recognized among those of uncertain malignant potential, justifying their classification as low-grade chondrosarcomas.

Adult↗