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Multifocal (dimelic) chondrosarcoma--report of a case.

Estraosseous origin, implantation, and multifocal origin are some of the peculiar behaviour of chondrosarcoma. A case is presented of osseous chondrosarcoma arising from multiple sites in the same patient to illustrate the problem involved in the treatment of such a case.

Adolescent↗

[Intra-and extracranial chondrosarcoma].

A 17-year-old female, who had diplopia and progressive gait disturbance, had been diagnosed as having a right parasellar mass lesion in 1986. Initial CT scan failed to show a parasellar space occupying lesion, although it was well demonstrated on MRI. No surgery was carried out at that time. Seven years later, she was hospitalized because of progressive gait disturbance, lower cranial nerves palsy, and cerebellar sign as well as the known right abducence palsy. At this time, CT scan exhibited a huge low density area with a marginal high density area suggesting calcification, which extended from the right parasellar area to the ventral portion of the midbrain. She underwent surgery under the diagnosis of cartilaginous tumor or chordoma. The intracranial lesion was removed via combined translabyrinthine approach and epidural subtemporal approach. The tumor was hitologically diagnosed as well-differentiated chondrosarcoma. Electron-microscopy demonstrated tumor cells with features of chondrocytes. Since the original site of the tumor, strategies for treatment and prognosis are different from each other, distinction between chondrosarcoma and chordoma is important. MRI seemed very useful for making this distinction as a first step.

Adult↗

[Chondrosarcoma originating in the rib: a case report].

Chondrosarcoma originating in the rib is a rare disease. Although its pathological characteristics are believed to be mild, it is apt to recur and prognosis deteriorates in the absence of appropriate excision. The authors experienced a patient with a chondrosarcoma in the right 6th rib. The patient was a 49-year-old man who visited our hospital with a mass in the right anterior chest wall and chest pain. The patient underwent radical excision of the bony chest wall including the right 5th to 7th ribs. The resulting defect in the chest wall was restored with double-overlapping Marlex mesh. There has been no sign of recurrence to date, 6 years and 1 month postoperatively.

Bone Neoplasms↗

Chondrosarcoma complicating synovial chondromatosis: findings with magnetic resonance imaging.

A 64-year-old man with a tender mass at the ankle was found to have chondrosarcoma complicating synovial chondromatosis. Malignancy was suspected on the basis of the clinical presentation rather than the findings in plain radiographs or magnetic resonance images. Chondrosarcoma arising in synovial chondromatosis is very rare. Misinterpreting the latter condition as malignant and the former as benign can be problematic, and the importance of distinguishing the two entities is discussed.

Ankle Joint↗

[Chondrosarcoma of the larynx. Needle biopsy diagnosis by computed tomography].

Chondrosarcoma of the larynx is the most frequent sarcoma at this site. The growth rate is very slow, so that the first symptoms, dyspnea and hoarseness, may be insidious. Chondrosarcomas are usually of low-grade malignance and rarely metasize. Surgical resection is the treatment of choice. We present a case of diagnostic FNA using computed tomography.

Aged↗

The use of cryosurgery in the treatment of low and medium grade chondrosarcoma. A preliminary report.

Eighteen chondrosarcoma (Grade I and Grade II) have been treated with cryosurgery. One patient subsequently developed a metastasis; three later had to have an en bloc resection (in two of these there was no tumor in the specimen) and three underwent amputation (one of these had tumor in the specimen). In general, cryosurgery for low grade chondrosarcoma is encouraging as long as the limb is protected postoperatively from fracture -- using various orthopedic appliances to allow bone regeneration (i.e. ischial weight bearing brace). Other complications such as nerve paralysis have been transient. One air embolis death occurred (in another disease) where the exit of nitrogen gas was blocked. This is preventable by allowing free exit of gas from the bone. Approximately 500 cases of primary bone have been treated with cryosurgery as of this date; long term follow-up results will be reported later.

Adolescent↗

Dedifferentiation of low grade chondrosarcomas.

In four unusual cases where a low-grade chondrosarcoma suddenly dedifferentiated into a florid, highly-malignant fibrosarcoma, the average survival was 12 months (range 5-18 months). The surgical treatment of chondrosarcoma consists of a primary excisional biopsy, and if dedifferentiation to fibrosarcoma is seen, radical excision and chemotherapy or irradiation or both.

Bone Neoplasms↗

Chondrosarcoma of the larynx: CT features.

PURPOSE: To define the typical CT features of chondrosarcoma of the larynx. PATIENTS AND METHODS: Results of CT studies, performed on 10 patients with pathologically proved chondrosarcoma of the larynx, were retrospectively reviewed and correlated with clinical presentation. RESULTS: In all patients, the mass was detected on CT. The most frequent site of origin of the tumor was the cricoid cartilage (nine cases) followed by the thyroid cartilage (one case). Coarse or stippled calcification within the tumor was the most helpful radiologic finding and was seen in every case. In eight patients, the tumor had both an endolaryngeal and an extralaryngeal growth pattern, whereas in two patients the tumor was entirely endolaryngeal. Hoarseness, dyspnea, and dysphagia were the most common symptoms. In all patients presenting with dyspnea, the tumor exhibited endolaryngeal components. In patients presenting with hoarseness, three tumors had endolaryngeal and extralaryngeal components and two tumors were entirely endolaryngeal. CONCLUSION: Cross-sectional imaging afforded excellent evaluation of the airway as well as the extralaryngeal component of the tumor.

Aged↗

Myxoid chondrosarcoma of the abdominal aorta.

In this case report we present a 64 year old man who underwent resection of his infrarenal abdominal aortic aneurysm. Thrombus of unusual color, texture and consistency was noted within this aneurysm which upon histopathologic analysis had the characteristics of a myxoid chondrosarcoma. Primary myxoid chondrosarcoma of the abdominal aorta is very rare and to our knowledge this report represents the first recorded case. The clinical presentation of our patient was dominated by hypertension and severe lower extremity claudication associated with microembolization to the left fourth toe.

Aorta, Abdominal↗

[Primary chondrosarcoma of the pulmonary artery].

A 69-year-old woman who had progressive dyspnea, edema and oliguria was diagnosed as primary sarcoma arising in the pulmonary trunk by preoperative examination. She received thoracotomy and total resection of the tumor in the pulmonary trunk utilizing cardiopulmonary bypass. A microscopical examination revealed that the tumor had the features of the chondrosarcoma originating in the pulmonary artery. There have been very few reports on chondrosarcoma in the pulmonary artery. It is reported that the prognosis of the sarcoma in the pulmonary artery is less favorable, however, this case had uneventfully postoperative course and no signs of recurrence one year after operation.

Aged↗

[Clinical aspects and treatment of chondrosarcoma in children and adolescents].

The clinical cours and treatment of chondrosarcoma in 9 children, aged from 8 to 16 years, are analysed. Only provisional and not persistant effect was gained after roentgenotherapy. Resection of the bone with tumor removal also failed to provide radical intervention in all patients, but one, and recurrences were noted 3-12 months following the resection. However, chondrosarcoma in children is continuously observed to be a local process and is characterized by late metastasization, due to that its prognosis is more favourable than in other malignant bone tumors.

Adolescent↗

Fusion of the EWS gene to CHN, a member of the steroid/thyroid receptor gene superfamily, in a human myxoid chondrosarcoma.

The specific chromosomal translocation t(9;22)(q22-31;q11-12) has been observed in the myxoid variant of human chondrosarcoma. In agreement with this observation we report that the EWS gene located at chromosome band 22q12 becomes fused to CHN, a member of the steroid/thyroid receptor gene superfamily located at 9q22-31, in a skeletal myxoid chondrosarcoma. CHN appears to be the human homologue of the rat gene NOR1, which was recently identified as a sequence overexpressed in rat brain cells undergoing apoptosis. Our results also indicate that the chimaeric EWS-CHN gene encodes a EWS-CHN fusion protein in which the C-terminal RNA-binding domain of EWS is replaced by the entire CHN protein, comprising a long N-terminal domain, a central DNA binding domain and a C-terminal ligand-binding/dimerisation domain.

Amino Acid Sequence↗

Dedifferentiated chondrosarcoma in childhood: report of a case.

Dedifferentiated chondrosarcoma is an unconventional chondrosarcoma of distinctive pathology. The tumor, not previously reported in childhood, is characterized by a very poor prognosis with an average survival of only 6 months. Imaging features include a lytic lesion, focal calcifications, and a soft tissue mass.

Child↗

[A case of dumbbell-shaped chondrosarcoma of the rib in the posterior mediastinum].

A 43-year-old man was pointed out an abnormal shadow in the left hilar lesion with no symptom. Chest CT and MRI showed that the mass might be a dumbbell-shaped neurogenic mediastinal tumor next to the 8th thoracic intervertebral foramen. The resection of the chest wall and the thoracic vertebrae including the tumor was carried out, so it was revealed chondrosarcoma arising from the 8th rib by the operative findings and histological examination. The dumbbell-shaped chondrosarcoma of the rib in the posterior mediastinum is very rare, and a brief review was made from the literature.

Adult↗

Loss of heterozygosity and tumor suppressor gene mutations in chondrosarcomas.

Loss of heterozygosity (LOH) of chromosomes 13q and 17p and mutations of the retinoblastoma (Rb) and p53 gene were studied in 28 tumors from 22 patients with chondrosarcomas. Allele loss at polymorphic loci on 13q was found in 36% of tumors and the frequency was much higher in grade II (56%) or high grade (40%) tumors than in grade I tumors (18%). LOH of 17p, which was detected in 25% of all tumors, was of low frequency in low grade tumors (8% in grade I and 20% in grade II), whereas 80% of tumors with high grade tumors were positive for LOH. These observations may imply that LOH on 13q and 17p contributes to the tumor development at different stages. In low grade cases (grade I and II), tumor recurrence was observed more frequently in primary tumors with LOH on 13q (86%) than those without (8%), suggesting the significance of LOH analysis in the assessment of biological behavior of tumors. Structural alteration of the Rb gene was found in one dedifferentiated tumor, and point mutations of the p53 gene were found in all of five high grade tumors, indicating that high grade chondrosarcomas were genetically equivalent to other high grade sarcomas such as osteosarcomas.

Adolescent↗

[A case of chondrosarcoma of the low grade malignancy originated in rib].

A case with chondrosarcoma of rib, which is relatively uncommon in Japan, in a 61-year-old man is reported. Chest X-ray examination revealed an abnormal shadow in the right of the chest wall. Chest CT scan and MRI disclosed the tumor to have been arosen from the right 5th rib protruding into the thoracic cavity. Surgical resections of the tumor with parts of the 4th and 5th ribs and parietal pleura were performed. The defect of the chest wall was repaired with muscle. The tumor measured 3.0 x 3.0 x 3.5 cm in size and was a chondrosarcoma of the low grade malignancy, histologically. Post operative course was uneventful with no adjuvant therapy.

Bone Neoplasms↗

Chondrosarcoma: a case report with left atrial involvement and systemic embolization.

A 26-year-old white man underwent amputation of the right lower extremity for a chondrosarcoma of the distal femur. Eleven years later, after a long symptom-free interval, he was hospitalized for rapidly progressive dyspnea, pleuritic chest pain, and hemoptysis resulting from a large pulmonary metastasis that had extended directly to the left atrium via the pulmonary vein. Within 24 hours of hospitalization, obstruction of the left commom iliac artery by tumor embolus necessitated embolectomy. This represents the second report of a metastatic chondrosarcoma involving the left atrium. The case presented clinically as an atrial myxoma and disseminated via the systemic circulation with a rapidly downhill course therafter.

Adult↗