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PubMed2006

Chondrosarcoma of the ring finger: a case report and review of the literature.

Enchondromas are the most common benign cartilaginous bone tumors arising in the medullary cavity of the small bones of the hand. In contrast, chondrosarcomas, commonly occurring in the pelvis and proximal femur and humerus, are very uncommon at this site. We report an unusual case of chondrosarcoma arising in the ring finger proximal phalanx with its radiological and histological features and reviewed the literature. The patient was an 80-year-old man whose chief complaint was swelling and pain for seven years. The findings of cortical irregular thickening by plain radiography and computed tomography and soft tissue extension by magnetic resonance imaging suggested the tumor was chondrosarcoma rather than a common enchondroma. Thorough curettage and artificial bone grafting was performed because of the age of the patient, his senile dementia and the strong desire of the patient and his family. Histological examination revealed that the tumor was composed of polygonal cells with eosinophilic cytoplasm proliferating in the chondromatous matrix with partially myxoid changes. Nuclear irregularity, binucleated cells, bone permeation and encasement were observed and the tumor was diagnosed as grade 2 chondrosarcoma. The tumor recurred five months after surgery. Amputation of the ring finger including the distal part of the 4th metacarpal was performed. At two years after surgery, the patient was free from recurrence or lung metastasis. In conclusion, details of radiological as well as pathological findings are essential for differential diagnosis between benign enchondroma and chondrosarcoma in the hand. Chondrosarcoma of the hand requires a prompt and more radical treatment than enchondroma. Wide excision is recommended to avoid local recurrence or metastasis.

Aged, 80 and over↗

PubMed1997

Usefulness of radiography in differentiating enchondroma from central grade 1 chondrosarcoma.

OBJECTIVE: The purpose of this study was to evaluate clinical symptoms and radiographic features that allow radiologists to differentiate between enchondroma and central grade 1 chondrosarcoma. Such differentiation is important because of differences in management. MATERIALS AND METHODS: Clinical symptoms and location and size of 35 enchondromas and 43 central grade 1 chondrosarcomas were analyzed. Radiographic features were assessed independently by three observers. The chi-square test and linear discriminant analysis were used to identify features with discriminating strength. Kappa values were calculated to validate the consistency of observations among observers. A consensus diagnosis made by histology and long-term follow-up was used as the standard. RESULTS: No statistically significant correlation was found between clinical symptoms and the benign or malignant nature of the neoplasms. Grade 1 chondrosarcomas were more likely to be found in the axial skeleton and in flat bones. Also, chondrosarcomas were significantly larger than enchondromas (p < .001). Ill-defined margins and lobulated contours were the only morphologic features seen on radiographs that allowed significant discrimination (p = .004 and .009, respectively). An optimal combination of four radiographic features still left 72 of the 78 lesions with a 10-90% probability of malignancy, indicative of poor discriminating power. Kappa values generally showed poor to fair agreement. CONCLUSION: Location in the axial skeleton and size greater than 5 cm are the most reliable predictors of central grade 1 chondrosarcoma. Morphologic features seen on radiographs and clinical symptoms do not improve the ability to differentiate between enchondromas and central grade 1 chondrosarcomas.

Adolescent↗

PubMed2005

Chondrosarcoma of the head and neck.

Chondrosarcoma of the head and neck region is a rare disease, representing approximately 0.1% of all head and neck neoplasms. The 5-year survival rate of chondrosarcoma is 70-80%, showing relatively good prognosis; however, it is known to progress slowly and eventually cause multiple metastases. In this study, we reviewed chondrosarcoma cases experienced at Yonsei University Medical College during the last 15 years to investigate its clinical characteristics and treatment outcome. The medical records of 8 patients who were diagnosed with chondrosarcoma of the head and neck region and underwent surgical treatment between December 1990 and December 2002 were retrospectively reviewed. The primary sites were sinus, mastoid, jugular foramen and thyroid cartilage. In all patients, the initial treatment modality was surgery, and postoperative radiation therapy was performed in 4 cases. The pathological findings showed chondrosarcoma in 7 cases and mesenchymal chondrosarcoma in 1 case. The treatment outcome resulted in 3 cases of recurrence, of which 2 cases were treated successfully and the other case expired of disease, indicating a survival rate of 87.5%. In the case that resulted in death, complete excision could not be achieved. Therefore, we concluded that wide excision is a beneficial initial treatment of this rare disease.

Adult↗

PubMed2000

Laryngeal chondrosarcoma with regard to the ultrastructure.

Most common among the laryngeal sarcomas is the chondrosarcoma. The difficulties in differentiation between benign and malignant behavior of cartilaginous tumors are responsible for the danger of misinterpretation of chondrosarcoma as chondroma. A case of a laryngeal chondrosarcoma is presented examined by light and electron microscopy to determine if the ultrastructure of chondrosarcoma could be helpful in the correct diagnosis. The cells and their formation play a more important role than the extracellular matrix in the differentiation of tumor's behavior. As special criterias of malignancy by electron microscopy are the presence of dominant mitochondria, fat vacuoles, dilated rough endoplasmatic reticulum and the irregular shape of chondrocytes. The knowledge of the ultrastructure of chondrosarcomas may be helpful to distinguish between a benign chondroma and low-grade chondrosarcoma, especially when only small tumor biopsies are valuable.

Aged↗

PubMedDate not supplied

Correlation between the histological grade of chondrosarcoma and the expression of MMPs, ADAMTSs and TIMPs.

BACKGROUND: The malignant degree of human chondrosarcoma can be difficult to determine using only histological findings. We, therefore, assessed the expression of matrix metalloproteinases (MMPs), a disintegrin and metalloproteinase (ADAM) with thrombospondin motifs (ADAMTSs) and tissue inhibitor of metalloproteinases (TIMPs) in chondrosarcoma and ascertained the relationships to histological degree of malignancy and prognosis. MATERIALS AND METHODS: In 28 chondrosarcoma cases, immunostaining was performed using antibodies against MMP 2, 3, 7, 9, 13, ADAMTS 4, 5 and TIMP 1, 2, 3. RESULTS: The chondrosarcoma were classified into groups of 7, 15 and 6 cases based on histologically malignant grade I, II and III, respectively. All target proteins were expressed in chondrosarcoma. Positive correlations (p < 0.05) existed between immunostaining scores and histological grades for all proteins except MMP 9, with strong correlations (p < 0.01) for MMPs 2, 3 and 13, both ADAMTSs and all 3 TIMPs. No correlation existed between prognosis and immunostaining scores. CONCLUSION: These target proteins could, thus, indicate the degree of malignancy in human chondrosarcoma.

ADAM Proteins↗

PubMed1991

[Immunohistochemical study on clear cell chondrosarcoma].

Clear cell chondrosarcoma, a subtype and separate entity from the conventional chondrosarcoma, is characterized by its special histologic features, site of predilection, slow growth and better prognosis. Three cases are presented with elucidation of clinicopathologic correlation and detection by ABC immunohistochemical method using several antibodies. The observation of positive reaction to S-100 protein, vimentin, anti-alpha-chymotrypsin and Lysozyme by the tumor cells of clear cell chondrosarcoma, similar to chondrosarcoma and chondroblastoma, proves that this tumor has its origin from the cartilaginous tissue. It was found for the first time that the clear cell chondrosarcoma was positive for wheat germ agglutinin and concanavalin A. The authors believe that clear cell chondrosarcoma may result from the anaplastic change of chondroblastoma cells into another subtype of that tumor. The osteoblastlike multinucleated giant cells, retaining the antigens of phagocytes, are not considered to be neoplastic.

Adult↗

PubMed1980

The prognostic significance of nuclear DNA content in chondrosarcoma.

The nuclear DNA content in 48 chondrosarcomas and 4 chondromas was determined. Grading according to O'Neal-Ackerman was also performed. All chondromas were found to be diploid (normal nuclear DNA content). Twenty-two of the chondrosarcomas showed the same DNA distribution curve as the chondromas and were characterized as diploid, while 26 were found to be hyperploid (increased nuclear DNA content). A comparative study of grade and ploidy in relation to ten-year survival and metastasis rates showed that ploidy determinations in chondrosarcomas give significant prognostic information, even better than that obtained by conventional histopathologic grading. In this study, diploid chondrosarcomas were found to be associated with a significantly more favorable clinical course than were hyperploid chondrosarcomas. The prognostic relevance of these ploidy determination was further confirmed by analyzing the ploidy level of selected cases characterized bydifferent clinical courses. Thus, the tumors from 13 (93%) of 14 patients with a highly malignant clinical course (death within two years) and the tumors at the primary site in 17 (81%) of 21 metastasizing chondrosarcomas were found to be hyperploid. Direct measurements on four metastases revealed that all were hyperploid.

Adolescent↗

PubMed1993

Clear cell chondrosarcoma of bone. A report of three cases with immunohistochemical and affinity histochemical observations.

Clear cell chondrosarcoma, a subtype and separate entity from the traditional chondrosarcoma, is characterized by its special histologic features, site of predilection, slow growth and better prognosis. Three cases are presented with elucidation of clinicopathologic correlation and detection by the ABC immunohistochemical method using several antibodies. The observation of positive reaction to S-100 protein (S-100), vimentin (Vim), alpha 1-antichymotrypsin (AACT) and lysozyme (Lyso) by the tumor cells of clear cell chondrosarcoma, similar to traditional chondrosarcoma and chondroblastoma, proves that this tumor has its origin in the cartilaginous tissue. It was found for the first time that the clear cell chondrosarcoma was positive for wheat germ agglutinin (WGA) and concanavalin A (Con A). The authors believe that clear cell chondrosarcoma may result from the anaplastic changes of chondroblastoma cells into another subtype of that tumor. The osteoclast-like multinucleated giant cells (MGC), retaining the antigens of phagocytes, are not considered to be neoplastic.

Adult↗

PubMed1997

Expression of plasminogen activators and plasminogen activator inhibitor 1 in dedifferentiated chondrosarcoma.

BACKGROUND: The plasminogen activator system plays an important role in different malignant tumors. These enzymes participate in the destruction of intercellular matrices and basement membranes and/or can modulate the growth potency of tumor cells and may even promote metastases. In this study, the expression of three glycoproteins that play a role in the plasminogen activator system as activators of proteolysis-urokinase type plasminogen activator (u-PA), tissue type plasminogen activator (t-PA), and plasminogen activator inhibitor type 1 (PAI-1) were studied in various components of dedifferentiated chondrosarcomas of bone. METHODS: The expression of u-PA, t-PA, and PAI-1 was investigated in 10 dedifferentiated chondrosarcomas and 14 conventional chondrosarcomas. The plasminogen activator/inhibitor glycoproteins were visualized immunohistochemically on paraffin sections and the levels of expression were assessed semiquantitatively. RESULTS: In dedifferentiated chondrosarcoma, high grade dedifferentiated components displayed strong, diffuse coexpression of u-PA, t-PA, and PAI-1. For all glycoproteins studied, the immunoreactivity was significantly increased compared with the reactions in the low grade cartilaginous component of the same tumor and conventional chondrosarcoma. In the latter, u-PA, t-PA, and PAI-1 expression was found to be enhanced at invasive foci and in regions of endochondral ossification. CONCLUSIONS: The current study documents the overexpression of u-PA, t-PA, and PAI-1 in dedifferentiated chondrosarcoma and suggests involvement of the plasminogen activator system in the biology of these tumors.

Bone Neoplasms↗

PubMed1990

Chondrosarcoma of the larynx. Diagnosis with magnetic resonance imaging and computed tomography.

Chondrosarcomas are the most common sarcomas of the larynx. They are generally slow-growing lesions with insidious onset of symptoms. We are presenting a case of chondrosarcoma that recurred 10 years after excision of a presumed chondroma. Computed tomographic and magnetic resonance imaging (MRI) scans were performed. Both studies delineated the lesion nicely. To the best of our knowledge, this is the first report on MRI characteristics of chondrosarcoma of the larynx. A review of the literature regarding the use of computed tomography in the management of chondrosarcoma is presented. The MRI characteristics are discussed and the two scanning modalities are compared. Computed tomography is an excellent study in its ability to demonstrate the extent of the lesion within the laryngeal skeleton. Furthermore, it is more highly sensitive than plain roentgenograms in the detection of calcifications that are highly suggestive of chondrosarcoma. Alternatively, MRI can also demonstrate the lesion within the larynx but it has the added advantage of superior contrast resolution of the tumor and paralaryngeal tissues. When the three-dimensional imaging capacity of MRI is considered as well, MRI seems to be the superior study.

Aged↗

PubMed1998

Mesenchymal chondrosarcoma of the jaw bones: clinicopathologic study of 19 cases.

BACKGROUND: Patients with mesenchymal chondrosarcomas in general have a 5-year survival rate ranging from 42-54.6% and a 10-year survival rate of 28%. Nineteen cases of mesenchymal chondrosarcoma of the jaw bones were reviewed to study their clinicopathologic features and to compare their clinical behavior with that of mesenchymal chondrosarcomas of other locations. METHODS: The patients were 10 males and 9 females (age range, 2-51 years). Sixteen patients were age < 30 years, and the average age at presentation was 19 years. Eleven tumors involved the mandible and eight involved the maxilla. RESULTS: Histologically, the classic picture of a bimorphic tumor, composed of islands of well differentiated hyaline cartilage juxtaposed to a small cell undifferentiated malignancy, was present in every case. Resection, including hemimandibulectomy and hemimaxillectomy, was the main treatment in 16 patients. Seven patients had local recurrence, and five patients developed distant metastases. Six patients died of disease, and the 5-year and 10-year survival rates were 82% and 56%, respectively. CONCLUSIONS: Mesenchymal chondrosarcoma of the jaw bones appears to have a more indolent course than mesenchymal chondrosarcoma of other anatomic

Adolescent↗

PubMed1998

Altered p53 is associated with aggressive behavior of chondrosarcoma: a long term follow-up study.

BACKGROUND: p53 is a major tumor suppressor gene that has been implicated in the biology of a variety of human neoplasms, including some that affect the skeleton. Recent studies based on small numbers of cases have shown that overexpression or alteration of the p53 gene is frequently present in high grade, clinically aggressive chondrosarcomas of bone. In this study, the authors addressed the relation between overexpression and alteration of the p53 gene and the clinical aggressiveness of chondrosarcoma in a large series of patients for whom long term follow-up data were available. METHODS: The authors analyzed the expression and/or alteration of the p53 gene in 158 cases of chondrosarcoma of bone using immunohistochemistry, single-strand conformation polymorphism, and direct sequencing. They then related the findings to various clinicopathologic parameters and long term follow-up data. RESULTS: The presence of overexpression and/or structural alterations of the p53 gene was documented in 38.1% of chondrosarcomas of bone. A statistically significant correlation was observed between overexpression or alteration of the p53 gene and both the histologic grade of the tumor and the presence of metastasis. The probability of local recurrence free, metastasis free, and overall survival was significantly higher for patients with no overexpression or alteration of p53 than for patients with p53 overexpression or alteration. CONCLUSIONS: Overexpression or alteration of the p53 gene is an important predictor of aggressive clinical behavior in chondrosarcoma of bone.

Adolescent↗

PubMed1999

Chondrosarcoma of the phalanx: a locally aggressive lesion with minimal metastatic potential: a report of 35 cases and a review of the literature.

BACKGROUND: Enchondroma is the most common primary benign bone tumor of the hand, especially the phalanges, whereas chondrosarcoma is uncommon at this site. Although phalangeal chondrosarcoma may have ominous histologic features, its biologic behavior is relatively indolent. METHODS: Thirty-five cases of phalangeal lesions previously diagnosed as chondrosarcoma were studied. Histologic and tumor-biologic parameters (Ki-67 and p53 immunohistochemistry) were investigated and correlated with clinical behavior. RESULTS: All cases were characterized by unequivocal malignant histologic features (Grade 2 or higher) or Grade 1 malignant histologic features combined with the presence of cortical destruction and soft tissue extension. The median age of the patients at the time of diagnosis was 67 years (range 21-87 years), with a slight female predominance. Occurrence in the hand was more common than in the foot, with the proximal phalanx affected most often. Treatment varied from local therapy (curettage or local excision) in 16 patients to amputation or exarticulation in 19 cases. Follow-up ranged from 8-432 months for 28 patients. Ten of 15 tumors treated by local therapy recurred whereas none of 13 tumors treated by radical surgery recurred. The median survival was 20.8 years; none of 28 patients developed metastases nor died of disease. Both the type of treatment and localization in the proximal phalanx were associated independently with local recurrence. CONCLUSIONS: Phalangeal chondrosarcoma behaves as a locally aggressive lesion and, in contrast to chondrosarcomas located elsewhere, rarely metastasizes. Treatment is indicated only because of its locally destructive growth. The authors believe that given the excellent survival data, curettage with adequate follow-up should be considered as the treatment of choice if technically feasible, especially in those cases in which amputation would lead to a significant loss of hand function.

Adult↗

PubMed1982

Prognostic factors in chondrosarcoma: a comparative study of cellular DNA content and clinicopathologic features.

In a retrospective study of 45 surgically treated chondrosarcomas, the prognostic significance of the cellular DNA content and different clinicopathologic factors was analyzed with separately and in combination. The relationship between these parameters was also investigated. Diploid (normal DNA content) chondrosarcomas were associated with as significantly (P less than 0.001) higher ten-year survival rate than hyperploid (abnormally increased DNA content) chondrosarcomas. Apart from ploidy, factors such as tumor size, tumor location and tumor grade also proved to be of prognostic importance. However, patients with diploid tumors had a better prognosis than those with hyperploid tumors, almost regardless of location, size, grade or even treatment. Nevertheless, additional prognostic information could be obtained by combining ploidy with size, location and grade of the tumors. Ploidy and treatment were significantly related to the ten-year recurrence rate. No statistical relationship could be demonstrated between ploidy and other prognostic factors. A strong correlation (P less than 0.001) was, however, found between location and size of the tumors. Thus, distal tumors were significantly smaller and, moreover associated with a better prognosis than axial tumors. The results indicate that ploidy probably is the best predictor of the clinical course in chondrosarcoma. While tumor location and size may be assumed to determine the conditions for radical surgery, ploidy and grade seem to reflect the inherent biological malignancy in chondrosarcoma. Consideration of these factors seems to provide not only valuable prognostic information, but also guidelines for treatment.

Adolescent↗

PubMed1989

Dedifferentiated peripheral chondrosarcomas. A report of seven cases.

Peripheral dedifferentiated chondrosarcoma (CS) is an exceedingly rare variant of the highly malignant entity of dedifferentiated chondrosarcoma. Only five such cases have previously been reported. Seven cases are analyzed and evaluated for the presentation and natural history of this highly malignant lesion when it arises in a tumor that was previously an osteochondroma. Both peripheral and central dedifferentiated chondrosarcomas are high-grade malignant lesions and require wide or radical surgical margins for adequate treatment. Despite adequate resection, survival is poor; five of the seven cases presented herein died of metastatic spread of their disease. These cases of the peripheral variant of dedifferentiated chondrosarcoma occurred in patients who were younger than patients with central dedifferentiated chondrosarcomas and may be present with longer duration of symptoms because they occur in previously long-standing benign osteochondromas. As such, they may be easily overlooked clinically and radiographically. Therefore, careful histologic analysis of all cartilage lesions arising on the surface of bone is essential to prevent overlooking foci of high-grade sarcomatous dedifferentiation.

Adult↗

PubMed1990

A common cytogenetic abnormality and DNA content alterations in dedifferentiated chondrosarcoma.

Dedifferentiated chondrosarcoma is an uncommon and aggressive variant of chondrosarcoma. The authors report the flow cytometric characteristics and cytogenetic findings in culture of two cases of dedifferentiated chondrosarcoma. The first case was DNA diploid by flow cytometry but had cytogenetic abnormalities consisting of breaks in the short arms of both chromosomes 1, resulting in deletion in one homolog and recombination in the other. In addition, cells from this tumor showed a balanced translocation between chromosomes 4 and 5, deletion of chromosome 9, and monosomy for chromosome 10. The second case was DNA aneuploid and more complex cytogenetically but had, in common with the first case, rearrangement and translocation at the same band on chromosome 1. These cytogenetic changes are compared with abnormalities previously reported for chondrosarcoma. Possible relationships between the nonrandom chromosomal abnormalities and subclassification among chondrosarcomas are discussed.

Aged↗

PubMed1990

Chondrosarcoma of bone in children.

Chondrosarcoma of bone rarely occurs in children. This report analyzes the clinicopathologic features in a series of 47 patients with conventional chondrosarcoma who were less than 17 years of age. Of the 47 cases, 14 were from the Mayo Clinic files, and 33 were from our consultation files. Most of the lesions occurred in the trunk and upper ends of the long bones, with the humerus being the most frequent skeletal site. Twelve of the 47 tumors were secondary. The radiographic findings were the same as those seen in adult chondrosarcoma. Pathologically, the tumors were low grade. En bloc resection is the treatment of choice because of the high incidence of local recurrence with lesser surgical margins. Prognosis in childhood chondrosarcoma is no different from that in adult chondrosarcoma. None of the patients with follow-up data had metastasis.

Adolescent↗

PubMed1994

Mesenchymal chondrosarcoma of the orbit. Report of three new cases and review of the literature.

BACKGROUND: Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchymal chondrosarcoma have been reported. METHODS: The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed to determine clinical characteristics and appropriate therapy. RESULTS: Nine of the 10 patients were female; age of onset ranged from 10 to 35 years. Of eight patients with at least 2 years of observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct histologic types that might guide therapy. Presenting symptoms were typical of symptoms of an orbital mass: proptosis, pain, diplopia, change in visual acuity, ptosis, and tearing. CONCLUSION: The small number of reported cases of mesenchymal chondrosarcoma of the orbit prevents definitive conclusions, but it appears that resection is adequate therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of the orbit may have a better prognosis than tumors in other sites.

Adolescent↗