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PubMed1998

[Chondrosarcoma in nasal fossae and Maffucci's syndrome].

INTRODUCTION: Maffucci's syndrome is a congenital non-hereditary disease very similar to Ollier's disease and associates multiple cutaneous hemangiomas, dyschondroplasia and often enchondromas. EXEGESIS: We report a unique case involving synchronous localization of chondrosarcoma in nasal fossae and anterior chest wall, disclosing Maffucci's syndrome. CONCLUSION: Atypical chondrosarcoma localization must lead to further investigation of potential multiple enchondromatosis.

Adult↗
PubMed2002

Mutation analyses of the NFAT1 gene in chondrosarcomas and enchondromas.

Mice lacking nuclear factor of activated T cell 1 (NFAT1) showed an abnormal proliferation of chondrocytes in articular cartilage and formed an extraosseous cartilaginous mass resembling a neoplastic lesion, suggesting that the NFAT1 gene is a tumor suppressor gene in cartilaginous neoplasms. Here we performed mutation analyses of the NFAT1 gene in human cartilaginous tumors including 30 chondrosarcomas and 15 enchondromas. Reverse transcription-polymerase chain reaction (PCR) analysis revealed the expression of the NFAT1 gene in 15/15 chondrosarcomas and 12/13 enchondromas. To find subtle alterations, the genomic structure of the NFAT1 gene was determined using human genome draft sequences, and a mutation analysis was performed using the exon-by-exon PCR-single-strand conformation polymorphism method. Two heterozygous missense mutations, A1557T (His446Leu) and C2859T (Pro880Leu), were found in eight tumor samples, but the same mutation was also present in the constitutional cells of corresponding patients. The incidence of the mutant alleles in the patient and control groups showed no significant difference, suggesting that these mutations are rare single nucleotide polymorphisms unrelated with tumorigenesis. These results suggest that the NFAT1 gene is not likely to be a tumor suppressor gene in human cartilaginous tumors.

Adolescent↗
PubMed2003

Monoclonal antibody to parathyroid hormone-related protein induces differentiation and apoptosis of chondrosarcoma cells.

We investigated the effects of treatment with anti-parathyroid hormone-related protein (1-34) monoclonal murine antibody (anti-PTHrP MoAb) on apoptosis and the differentiation of chondrosarcoma HTB-94 cells. Treatment with anti-PTHrP MoAb accelerated apoptosis of HTB-94 cells in a dose-dependent manner, and anti-PTHrP MoAb also promoted the chondrogenic differentiation of HTB-94 cells. The induction of apoptosis by anti-PTHrP MoAb via imbalance of Bcl-2/Bax ratio and activation of caspase-3 may provide a mechanistic explanation for its potential antitumor effects. Our results suggest the possibility that anti-PTHrP MoAb may be beneficial as a new treatment for chondrosarcoma.

Antibodies, Monoclonal↗
PubMed1998

Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences.

PURPOSE: Skull base chordomas and chondrosarcomas pose management challenges owing to their critical location, locally aggressive nature, and high recurrence rate despite multimodality treatment. We used stereotactic radiosurgery as primary or adjuvant therapy to achieve safe and effective therapeutic irradiation. METHODS AND MATERIALS: At an average of 4 years (range 1-7), we evaluated 15 patients (nine with chordomas and six with chondrosarcomas) who had gamma-knife radiosurgery as an adjunct (13 patients) or as an alternative to microsurgical resection (two patients). Patient age varied from 7 to 70 years (mean 38). There was a distinct male preponderance (2:1). Thirteen patients had undergone between one and four resections. Using conformal radiosurgical planning, a maximum tumor dose of 24-40 Gy (mean 36) and a tumor margin dose of 12-20 Gy (mean 18) was given to a mean tumor volume of 4.6 ml. RESULTS: Eight patients showed clinical improvement, three remained stable, and four died. Two of the four patients who died had tumor progression remote from the radiosurgery volume; two patients died of unrelated disorders. Among 11 surviving patients, follow-up imaging showed a reduction in tumor size in five, no further tumor growth in five, and an increase in the size of the tumor in one. The patient with further tumor growth after radiosurgery subsequently underwent repeat resection. CONCLUSION: Despite the formidable management challenge posed by these neoplasms, our long-term evaluation has shown that radiosurgery is a safe and effective treatment for patients with small volume tumors.

Adolescent↗
PubMed1996

Primary cardiac chondrosarcoma.

A case of primary cardiac chondrosarcoma in a 41-year-old woman who presented with cardiac tamponade and cardiac intracavitary obstruction is described. The tumor originated from the right atrium and invaded the adjacent right ventricular wall and interatrial septum. Primary cardiac chondrosarcoma is extremely rare, and its clinical, computed tomographic, echocardiographic, and magnetic resonance imaging findings are described.

Adult↗
PubMed1995

Chondrosarcoma of bone. A clinical and DNA flow cytometric study.

Fifty-two patients with chondrosarcoma were treated in our hospital between 1981 and 1992. Tumours from 21 of the patients were histologically grade I, 19 were grade II, 10 were grade III and two were grade IV. Forty-seven patients were treated surgically and samples for flow cytometric DNA analysis were taken from 38 tumours with a successful measurement in 37 cases. The mean follow-up time was 40 months and during this period 17 of the patients treated surgically developed a local recurrence or metastasis. In one case, a highly anaplastic sarcoma developed after radiation treatment. Only two of the 19 diploid tumours recurred, whereas eight of the 18 aneuploid tumours showed a recurrence. These results indicate that DNA flow cytometry is of prognostic significance in chondrosarcoma and may aid in planning the treatment.

Adult↗
PubMed1997

Pelvic girdle chondrosarcoma and inferior vena cava thrombosis.

Pelvic girdle chondrosarcomas in children and adolescents are extremely rare. These high grade malignant chondrosarcomas with a locally aggressive growth pattern have a high tendency for thrombus formations in the afferent tumour vessels, associated with an increased risk of inferior vena cava thrombosis, pulmonary embolism and metastatic spread to the lungs. This tumour location is generally non-resectable for cure and, since adjuvant chemotherapy is ineffective, patients die from the tumour thrombus formation causing tumour emboli and metastatic disease to the lungs. Effective local tumour control can be achieved with high-dose hyperfractionated radiation therapy.

Adolescent↗
PubMedDate not supplied

Parosteal chondrosarcoma, a very rare condition of the mandibular condyle.

We present a rare case of parosteal chondrosarcoma of the madibular condyle. The patient was referred for a functional limitation of the left temporo-mandibular joint. CT and MRI examinations demonstrated a 3.5-cm cystic mass with a peripheral rim of contrast enhancement located in the left pterygo-maxillary space. The mass had partial intraarticular spread causing deformation and focal cortical erosion of the medial aspect of the condylar head. The lesion was surgically removed; the histological diagnosis was of low-grade chondrosarcoma.

Bone Neoplasms↗
PubMedDate not supplied

MR imaging of dedifferentiated chondrosarcoma.

MR imaging of a dedifferentiated chondrosarcoma of the humerus is presented and correlated with gross and microscopic histology from the surgical specimen. The classification of chondrosarcoma and specific MR signal characteristics that suggest dedifferentiation are discussed.

Bone Neoplasms↗
PubMed1998

Parosteal chondrosarcoma attached to the hyoid bone. A case report.

A parosteal chondrosarcoma with low-grade malignancy attached to the hyoid bone in a 66-year-old man is reported. The tumor was considered to present a low-grade malignancy because slight invasion into the surrounding soft tissues and rapid growth were observed. Four years after initial surgery he had a local recurrence. To the best of our knowledge, this is the first reported case of parosteal chondrosarcoma in this location.

Aged↗
PubMed1998

Mesenchymal chondrosarcoma of the jaws. Report of four cases.

Mesenchymal chondrosarcoma (MCS), described in 1959 by Lichtenstein & Bernstein, is a very rare malignant tumour. Only 46 cases have been previously reported in the jaws. Over a period of 30 years, four chondrosarcomas of mesenchymal type (of which one has already been published in 1987 with a three-month follow up) were treated in our department. Two patients have been followed for more than 17 years, of whom one has died of an intracranial extension and the other is still alive after surgical treatment of numerous recurrences and metastases. The two others are tumour-free with a follow up of 18 months and 11 years respectively. Histological, epidemiological and clinical features and treatment of MCS are discussed relevant to the data published in the literature.

Adolescent↗
PubMed1988

Chondrosarcoma of the mandible. Report of case and a survey of 23 cases in the Japanese literature.

A huge chondrosarcoma of the mandible (80 X 95 X 100 mm in size) with extension into the infratemporal fossa is described. The tumour was successfully treated by surgical removal and postoperative irradiation. A survey of the Japanese literature revealed 23 cases of chondrosarcoma with involvement of the mandible. The tumours occurred equally in males and females whose mean age was 38 years. The molar region was the site of predilection. The most common symptom was swelling and it was accompanied by pain in 7 cases and paraesthesia in 5 cases. Radiographically, the lesions were quite variable and with the exception of 3 cases in which information was not available, they consisted of a combination of irregular radiopacity and radiolucency in 9 cases, whereas the predominant feature was radiopacity in 6 cases and radiolucency in 4 cases. There was no radiographical abnormality in 2 cases. Root resorption of adjacent teeth was noted in 3 of 6 cases where information existed. Computed tomography was thought to be quite valuable in determining the nature and extent of the tumour. Although an elevation of serum alkaline phosphatase was observed in our case, results of laboratory tests were mostly of no diagnostic significance. Surgical removal was employed in 22 cases alone or in conjunction with irradiation and/or chemotherapy. Of 14 cases on whom information was available, local recurrence occurred in 6 cases in which radiotherapy was not given and distant metastasis in 2 of 10 cases on whom information was available. Of 20 patients on whom information was available on the postoperative course, 7 patients died 5 months to 6 years after the primary treatment.

Adult↗
PubMed1999

Chondroma and chondrosarcoma.

A chondrosarcoma is the most common primary malignant tumor of the chest wall. It usually presents on the anterior chest wall arising from the costochondral arches or sternum. It may occur as the result of malignant degeneration of a benign chondroma. Both chondrosarcomas and benign chondromas present as painful, slow-growing, hard, fixed, and nontender anterior chest wall masses. Microscopic differentiation between the two tumors is difficult and the treatment for both tumors is wide excision with a margin of at least 4 cm. Chemotherapy is ineffective and radiation therapy is used only for patients with tumors that are either not amenable to surgical resection or have positive resection margins.

Bone Neoplasms↗
PubMedDate not supplied

Chondrosarcoma of the bones of the feet.

Twelve chondrosarcomas of the bones of the feet from 11 patients in the Scottish Bone Tumor Registry were reviewed. One patient with diaphyseal aclasis (osteochondromatosis) developed 2 chondrosarcomas. The mean age of patients was 52.3 years (range, 17 to 83 years). Men were predominantly affected. Four tumors affected the tarsal bones; the rest involved the short tubular bones. The usual clinical presentation was a painful, progressively enlarging swelling. Radiologically, most showed some bone expansion, cortical destruction with indistinct margins, and soft-tissue extension. Histologically, the majority were middle-grade tumors. Treatment included curettage or local excision for 4 tumors and amputation or ray resection for 8 tumors. Follow-up varied from 6 months to 18 years (average, 5.8 years). Local recurrence after surgery was seen in 3 patients. All 3 died because of metastases to the lungs or brain.

Adolescent↗
PubMed2001

[Chondrosarcoma of the hand. Diagnostic and therapeutic discussion (three case reports)].

Chondrosarcoma of the hand is very rare. We report three observations which enable us to make a review of its pathology. There were two men and one woman aged 72, 38 and 63 years respectively. Localisation was phalangeal in two cases and metacarpal in one case. Two cases were central and one peripheral. Radiographs were characterised by the presence of lytic areas with intralesional calcifications and soft tissue extension. Histological diagnosis was made by biopsy in two cases and after amputation of the finger for lesional spread in one case. Treatment consisted of amputation of the finger in two cases. In the case of metacarpal localisation, wide resection of two rays was followed by recurrence after ten months which required amputation of the hand. We agree with other authors who believe that chondrosarcoma in the hand is due to malignant change in a pre-existing chondroma. Conservative treatment is difficult due to the small size of the hand which allows easy spread of the tumour from compartment to compartment.

Adult↗
PubMed1978

Chondrosarcoma of the thyroid cartilage.

A TUMOUR which had been present for nine years in the thyroid cartilage of a 45-year-old man, was shown on microscopy to be a chondrosarcoma. This tumour probably took its origin in a pre-existing benign tumour at this site. The literature on similar previously documented tumours is reviewed, and the classification, diagnosis and management of these rare tumours is briefly discussed. Primary cartilaginous laryngeal tumours are uncommon (Hyams and Rubuzzi, 1970; Zizmor et al., 1975). A tumour of the thyroid cartilage which had been present for about nine years was shown histologically to be a low-grade chondrosarcoma probably arising from a pre-existing chondroma at this site. The clinical and pathological features of this case are presented and discussed in the light of the available literature.

Chondrosarcoma↗
PubMed1979

Mesenchymal chondrosarcomas of the head and neck.

Mesenchymal chondrosarcoma, in both osseous and extra-skeletal forms, has a decided predilection for the head and neck region. The two cases presented in this report affirm this tendency and also illustrate the capricious biologic behavior of the neoplasms as manifested by the 30 additional cases recorded in the literature. Histopathologic confusion with hemangiopericytoma is avoided by identification of the cartilage component in the mesenchymal chondrosarcoma and by the undifferentiated nature of the cellular (non-cartilaginous) component.

Adolescent↗
PubMed1982

Chondrosarcoma of the nasal septum.

The case history is presented of a patient with a massive chondrosarcoma arising from the posterior part of the nasal septum, extending into the base of the skull to involve the right middle cranial fossa. Removal of the main bulk of the tumour was accomplished by means of a cranio-facial resection, and it is hoped that useful palliation has thus been achieved. The literature relating to this rare tumour is reviewed: only seven cases have been previously described in which the tumour is believed to have arisen from the nasal septum. It is generally accepted that chondrosarcomas are radio-insensitive, and that surgical excision is the only effective form of treatment.

Adult↗