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Expression of the intermediate filament peripherin in extraskeletal myxoid chondrosarcoma.

The embryologic histogenesis of cartilage is not well characterized. While cranial cartilage is believed to be derived from pluripotential precursor cells of the neural crest, chondrocytes found elsewhere in the body are thought to be derived from mesoderm. As such, soft tissue tumors with cartilaginous differentiation may be related to neural crest or mesoderm. Peripherin is an intermediate filament encoded on chromosome 12, involved in growth and development of the peripheral nervous system. Peripherin is apparently expressed exclusively in cells derived from the neural crest and neural tube. A group of six soft tissue tumor types was selected because they are either of controversial differentiation or cytogenetically related to chromosome 12. A total of 41 cases was evaluated with antibodies against the intermediate filament peripherin. A panel of neural and neuroendocrine differentiation markers was used in selected cases. Three of five extraskeletal myxoid chondrosarcomas showed strong cytoplasmic reactivity with anti-peripherin. No peripherin expression was noted in any of eleven epithelioid sarcomas, eight liposarcomas, seven conventional chondrosarcomas, four neurothekeomas, three alveolar soft part sarcomas, or three clear cell sarcomas. The finding of peripherin expression in some extraskeletal myxoid chondrosarcomas may suggest the ability of some tumors to demonstrate both neural and chondroid differentiation.

Adolescent↗

Translocation der(13;21)(q10;q10) in skeletal and extraskeletal mesenchymal chondrosarcoma.

Cytogenetic studies of mesenchymal chondrosarcoma are few and to date, no specific or recurrent aberrations have been found. In this investigation, the cytogenetic and molecular cytogenetic (spectral karyotypic and fluorescence in situ hybridization) findings for two mesenchymal chondrosarcomas, one arising skeletally and the other extraskeletally, are reported. An identical Robertsonian translocation involving chromosomes 13 and 21 [der(13;21)(q10;q10)] was detected in both cases, possibly representing a characteristic rearrangement for this histopathologic entity. Both cases also exhibited loss of all or a portion of chromosomes 8 and 20 and gain of all or a portion of chromosome 12. The observation of similar chromosomal abnormalities in both skeletal and extraskeletal mesenchymal chondrosarcoma supports a genetic as well as histopathologic relationship between these anatomically distinct neoplasms.

Adult↗

Chondrosarcoma of bone complicating Ollier's disease: report of a favourable response to radiotherapy.

Because chondrosarcoma of bone is traditionally thought to be a radioresistant malignancy, it is usually managed surgically. We report a case of multifocal chondrosarcoma arising in Ollier's disease for which the patient declined surgery. He was given a course of radical radiotherapy that resulted in symptom palliation and a radiologically confirmed response before he died of disseminated disease. In patients with inoperable chondrosarcoma, radiotherapy can provide palliative benefit.

Bone Neoplasms↗

Chondrosarcoma of the distal phalanx of the second toe: a case report.

Although chondrosarcoma is a relatively common malignant bone lesion, these lesions rarely affect the foot. This article presents the case of a 32-year-old woman with a chondrosarcoma in the distal phalanx of the right second toe. Radiography and computed tomography showed cortical disruption and soft-tissue expansion. Histologically, the tumor was classified as grade 2 chondrosarcoma according to the American Joint Committee of Cancer pathologic staging system. Amputation at the distal half of the second proximal phalanx was performed to obtain clear surgical margins. A literature review and discussion of salient diagnostic and treatment issues is included.

Adult↗

Enchondroma and chondrosarcoma.

Enchondroma and chondrosarcoma are two of the most commonly encountered primary bone lesions in the typical radiology practice. The purpose of this article is to review the clinical, radiological, and pathological features that distinguish conventional chondrosarcoma from enchondroma. Chondrosarcoma is almost always associated with pain and tends to present in the axial skeleton of middle aged adults. Enchondroma tends to present in young adults in the appendicular skeleton, particularly the hands, and is often an incidental finding. Although both lesions have characteristic radiographic appearances, difficulty separating these two entities most often occurs when a lesion is seen in the long tubular bones. The judicious use of computed tomography, magnetic resonance imaging, and nuclear medicine in conjunction with appropriate clinical data allows the radiologist to establish the correct diagnosis of benign or malignant medullary chondroid lesion in the majority of cases.

Bone Diseases↗

[Chondrosarcoma of the sphenoethmoid complex: Case report].

BACKGROUND: Chondrosarcomas are rare malignant mesenchymal tumors of unknown etiology. Only 5 to 10 % appear in the head and neck region. PATIENT AND RESULTS: We report the case of a 63 year old woman with a chondrosarcoma of the sphenoethmoidal complex extending into the anterior cranial fossa. The tumor was subtotally resected via an osteoplastic lateral rhinotomy. Postoperatively, a fractionated radiation therapy with carbon ions (Schwerionen) was applied. There was no evidence of recurrence one year after therapy. CONCLUSION: Recent prognostic improvements of skull base chondrosarcomas by combination of skull base surgery and innovative radiation therapy are discussed and the literature is presented.

Cell Division↗

Imaging of chondrosarcoma with histopathological and prognostic correlation. An analysis of 49 cases mainly based on plain film radiography.

PURPOSE: To investigate the course of chondrosarcoma in relation to radiographic and histopathologic findings. MATERIAL AND METHODS: 49 consecutive patients seen during an 11-year-period were analysed, including re-evaluation of their radiographic and histopathologic material. RESULTS: Forty-two patients had radiographic changes typical for cartilaginous tumours, in 37 with malignant stigmata. Seven patients had malignant changes not typical for chondrosarcoma. By histopathologic grading 16 patients had grade I, 17 grade II and 16 grade III tumours. Six of the grade II-III tumours were histopathologic variants (mesenchymal, dedifferentiated or myxoid chondrosarcomas). Surgical removal of the tumour was performed in 42 patients, 41 of whom were followed up for 0.4-11.4 years (median 3.8 years). Local recurrence occurred in 7 patients, and 11 patients developed metastases. Ten patients, 4 with local recurrence and metastases, and 6 with metastases only were dead at the end of the follow-up. The actuarial 5-year overall survival rate was 64%. The occurrence of local recurrence, metastases and death was found to be related to the histopathologic grades II and III. Atypical radiographic features only occurred in grade II-III tumours and were related to metastases and death, but not to local recurrence. CONCLUSION: Atypical radiographic findings were related to high-grade malignancy and poor prognosis.

Adolescent↗

[Mesenchymal chondrosarcoma of the maxilla].

Mesenchymal chondrosarcoma is a variant that shows some peculiarities in comparison with classical chondrosarcomas. On the basis of a case report on a young man with a mesenchymal chondrosarcoma of the maxilla, the clinical symptomatology and diagnostics, morphological findings and actual therapy are discussed. In addition, problems of differential diagnosis are also discussed.

Adult↗

[Clear cell chondrosarcoma--clinico-pathological report of an additional case].

On account of the rarity and its histologic features, the clear-cell chondrosarcoma is a particular entity. Up to the present, 98 cases have been reported in the literature. In the present report one case located in the proximal femur will be discussed. Approximately 60% of these tumors are situated in the mentioned region. Including one referred case, which will not be described in detail, 100 cases have been recognized. Clear-cell chondrosarcoma is radiographically indistinguishable from benign lesions or chondroblastoma. Therefore the histologic examination is crucial for the diagnosis. The histologic characteristics preclude the diagnosis of chondroblastoma. Clear-cell chondrosarcoma is characterized by regions of chondroid matrix and areas of closely packed, glycogen-rich large tumor cells with distinct boundaries. The centrally located nuclei are large with marked polymorphism, surrounded by abundant clear PAS positive cytoplasm. The chondroid matrix is lacking. Osteoid formation and multinucleated giant cells are present in clear-cell areas. In spite of the low malignancy there is still a metastatic potential. Therefore the type of surgical treatment is very important. En bloc excision with wide margins is the treatment of choice. The knowledge about this tumor is crucial for adequate surgical therapy.

Adult↗

Mesenchymal chondrosarcoma originating from the femoral vein.

Mesenchymal chondrosarcoma is a rare variant of chondrosarcomas characterized by a bimorphic pattern with areas of the undifferentiated malignant small cells and well differentiated cartilaginous islands.(1) It occurs most commonly in the bone but can also occur in the extraskeletal soft tissues, the brain, and the meninges. This type of tumor has also been described in the eyelids, parapharyngeal space, mediastinum, and the kidney.(1-5) An origin from the large vessels has not been reported in the medical literature. The authors report a case of mesenchymal chondrosarcoma originating from the femoral vein in a 28-year-old female patient, treated by the wide-margin resection.

Adult↗

Laryngeal chondrosarcoma: a 24-year experience at the Royal National Throat, Nose and Ear Hospital.

This paper presents a review of the experience of 12 patients with chondrosarcoma of the larynx treated at The Royal National Throat, Nose and Ear Hospital, London, over the last 24 years. This represents the largest European series and the third largest in the world. Chondrosarcoma is the most frequent non-epithelial malignant tumor of the larynx, with 83.3% of cases arising from the cricoid cartilage in our series. We have shown that deep wedge biopsy with a CO2 laser, aided by computerized tomography scanning, gives the most reliable diagnosis. Laryngeal chondrosarcoma is characterized by indolent growth, a potential for local recurrence and, infrequently, by metastases. The treatment details of our patients are discussed. Adequate partial resection is often successful and use of CO2 laser as the initial treatment in five of these cases is presented.

Adult↗

Mesenchymal chondrosarcoma: an immunohistochemical study of 10 cases examining prognostic significance of proliferative activity and cellular differentiation.

AIMS: Mesenchymal chondrosarcoma is a rare malignant chondrogenic neoplasm that tends to affect young adults and teenagers. The prognosis is unpredictable, and the identification of prognostic markers that could aid in determining the behaviour of this tumour would be helpful. There are few studies in the literature that have attempted to address this issue. METHODS AND RESULTS: In this study, we explored the prognostic significance of three different parameters: (1) tissue morphology of small cell areas, (2) the expression of tumour differentiation marker genes, and (3) the proliferation rate. Our results did not show a correlation of prognosis with the histological features of the neoplastic small cell areas or the expression of tumour differentiation genes. However, the proliferative activity of the tumour cells appeared to have some prognostic significance as related to patient survival. CONCLUSION: Mesenchymal chondrosarcoma is a rare tumour with a wide clinical range of behaviour. Therefore, it is difficult to obtain reliable prognostic parameters. Nevertheless, our study suggests that proliferative activity may be a useful prognostic parameter for mesenchymal chondrosarcomas.

Adolescent↗

Meningeal chondrosarcomas, a review of 31 patients.

We reviewed the literature to study the clinical features, the management and the outcome of meningeal chondrosarcomas. We included 31 patients in this review: 22 were mesenchymal and nine were non-mesenchymal. The mean age was 27 years and 64% arose from the cranial meninges. The treatment was mainly total surgical excision. Adjuvant therapy was given to 36% of patients. Spinal meningeal chondrosarcomas had a better prognosis (81% 1-year survival and 45% 3-year survival). There were no pathognomonic clinical or radiological features. We concluded that the best management of meningeal chondrosarcomas is total surgical excision whenever possible, followed by combined course of radiotherapy and chemotherapy as soon as possible.

Adult↗

Clear cell chondrosarcoma of rib following repetitive low-impact trauma.

A case of clear cell chondrosarcoma of the rib in a 30-year-old man is described. The tumor developed at the site of repetitive low-impact trauma (on the arm of a wheelchair) over a period of 24 years. An association between chondrosarcoma and external trauma has not been previously reported. Chondrosarcoma has been described in previously irradiated bone and in Paget's disease. In both these examples there is an increase in new bone formation associated with necrosis of bone and increased resorption of bone, respectively. A possible mechanism in our case is increased new bone formation elicited by repeated microfractures of trabeculae of rib bone.

Adult↗

Mesenchymal chondrosarcoma with secondary orbital invasion.

Orbital involvement with mesenchymal chondrosarcoma is rare. Until recently, despite the young age of the affected population, exenteration was the recommended management. We report a patient with orbital invasion by mesenchymal chondrosarcoma managed surgically without exenteration. Adjuvant chemotherapy and radiotherapy were administered. There is no evidence of local or distant tumor recurrence with 30 months of postoperative follow-up. This and other recent case reports suggest that exenteration may not be necessary for local tumor control of mesenchymal chondrosarcoma in the orbit.

Adolescent↗

Utility of planar bone scintigraphy to distinguish benign osteochondromas from malignant chondrosarcomas.

PURPOSE: The current study was designed to evaluate the role of planar bone scintigraphy in the diagnosis of sarcomatous change in osteochondromas (cartilaginous exostoses). MATERIALS AND METHODS: Histologically verified cases of chondrosarcoma and osteochondroma, in which Tc-99m bone scintigraphy was performed, were reviewed in a retrospective study. RESULTS: Twenty-two cases were evaluated. Of 11 cases with chondrosarcomas, 8 (73%) had clearly increased tracer uptake, whereas the remaining cases had normal or decreased tracer uptake. Of the 11 cases with osteochondromas, 8 (73%) had clearly increased tracer uptake, whereas the remaining cases had normal or decreased tracer uptake. No significant difference in the distribution of scintigraphic results was noted between the two pathoanatomic groups (p = 1). CONCLUSION: In conclusion, single standing planar bone scintigraphy has no value in distinguishing benign osteochondromas from malignant chondrosarcomas.

Adult↗

Chondrosarcoma of the pelvis.

Thirty-one patients with chondrosarcoma of the pelvis were reviewed. The median followup period of the surviving patients was 66 months. Thirteen of 23 tumors after surgery with inadequate margins (57%) and 1 of 8 after surgery with adequate margins (13%) relapsed locally. Four of 10 patients had local recurrence, 2 of 2 patients had metastasis, and 4 of 4 patients who had local recurrence and metastasis died. A cumulative 10-year overall survival of patients with pelvic chondrosarcoma was 54%. Patients who had primary or high grade chondrosarcoma had a poor prognosis. Multivariate analysis (Cox proportional hazard model) showed that tumor type was the most important prognostic factor. Eight patients underwent hindquarter amputation, 6 patients had resection alone, and 17 patients had resection and reconstruction as follows: 3 interpubic arthrodeses, 2 iliosacral arthrodeses, 3 ischiofemoral arthrodeses, 8 hip prostheses (3 prostheses alone and 5 prostheses with allograft), and 1 hip transposition. Failure of reconstruction was attributed to infection in 5 patients, local recurrence of tumor in 5, and local recurrence and fracture of implant in 1 patient.

Adolescent↗

Marshall Urist Award. Interstitial collagenase gene expression correlates with in vitro invasion in human chondrosarcoma.

Matrix metalloproteinases contribute to the processes of local invasion and metastasis by providing cells with the ability to traverse tissue boundaries. The levels of gene expression were quantitated for matrix metalloproteinases-1 and tissue inhibitors of metalloproteinases-1 in human chondrosarcoma cell lines, and the results were correlated with cell differentiation, collagenase activity, and in vitro invasion. Three well characterized human cell lines were used in this study, with the level of chondrocytic differentiation confirmed to be JJ012, FS090, and 105KC in increasing order on the basis of aggrecan and collagen gene expression. The matrix metalloproteinases-1/tissue inhibitors of metalloproteinases-1 ratio correlated with the level of differentiation in an inverse fashion. Collagenase activity paralleled matrix metalloproteinases-1/tissue inhibitors of metalloproteinases-1 gene expression and was associated with a more invasive phenotype in an in vitro assay. In this report, matrix metalloproteinase-1 and tissue inhibitors of metalloproteinases-1 expression in human chondrosarcoma tumor cell lines were quantitated, and it was shown that interstitial collagenase gene expression correlates inversely with chondrocytic differentiation. Differences in collagenase activity and in vitro invasion correlate inversely with the level of differentiation. These findings are consistent with the hypothesis that collagenase activity is associated with a poorer prognosis in chondrosarcoma by facilitating cell egress from the tumor matrix.

Antineoplastic Agents↗