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[A case of chondrosarcoma of the thoracic vertebra].

A case of chondrosarcoma of the thoracic vertebra in a 47-year-old female is described. Chest X-ray, CT and MRI disclosed an abnormal mass lesion in the posterior mediastinum. The lesion was surgically removed through right thoracotomy. The operative findings revealed that the lesion, hard and measuring 6.5 x 4.5 x 4.5 cm in diameter, was originated from the 7th and 8th thoracic vertebra. The lesion was histopathologically diagnosed chondrosarcoma. The chondrosarcoma arising from the thoracic vertebra is very rare, and only 10 cases have hitherto been reported in the Japanese literature.

Chondrosarcoma↗

Laryngeal chondrosarcomas.

Four cases of laryngeal chondrosarcoma are presented. The patients' ages ranged from 60 to 75 years; one was a man and three were women. Two of the tumors arose in the thyroid cartilage, one in the cricoid cartilage, and one in the left arytenoid cartilage. A rapid fatal course with pulmonary and cerebral metastases was observed for the tumor arising in the arytenoid cartilage that histologically corresponded to a myxoid chondrosarcoma. The other three cases corresponded to low-grade, well-differentiated chondrosarcomas of the hyaline type, and the patients were alive and free of recurrence or metastases 3 to 6 years following total and partial laryngectomy. A review of the literature on the topic and the importance of assessing histologic grade, size, and location in the evaluation of these neoplasms is discussed.

Aged↗

[Dedifferentiated chondrosarcoma with maxillary involvement. An anatomo-clinical study, surgical treatment and prosthetic rehabilitation. Apropos of a case].

A 19 year old girl presented with a dedifferentiated chondrosarcoma of maxilla. The recurring tumor, diagnosed initially from the provoked pain, appeared on the scan image as a heterogeneous mass containing numerous calcifications. Histology showed, within a well differentiated chondrosarcoma, an anaplastic sarcomatous zone of the malignant fibrous histiocytoma (MFH) type. This histologic appearance is consistent with a poorer clinical prognosis. Radical surgery of the 4th tumor recurrence was combined with maxillofacial prosthetic rehabilitation. This appears to be first reported case of a dedifferentiated chondrosarcoma of the MFH type located in the maxilla.

Adult↗

[Chondrosarcoma of the trachea--report of a case].

A case of chondrosarcoma of the trachea in a 64-year-old man was reported. He was pointed out of benign tracheal tumor in September 1988. He was transferred to our hospital in September 1991 with respiratory distress attributable to asthma-like attack after bronchoscopic examination. Under right posterolateral thoracotomy, 1.7 cm length (3 rings) of the mediastinal trachea was resected and the trachea was reconstructed by end-to-end anastomosis with 3-0 Maxon interrupted sutures. The pathological diagnosis was a moderately well differentiated chondrosarcoma (grade II) of the trachea. Postoperative clinical course was uneventful without serious anastomotic complications. No local recurrence was seen 1 year after operation. It is necessary to follow meticulously the patients with chondrosarcoma of the trachea after surgery because of its low grade malignancy.

Chondrosarcoma↗

Low-grade myxoid chondrosarcoma of the temporal bone: differential diagnosis and report of two cases.

Skull base chondrosarcoma and chordoma are rare tumors that generally have a poor prognosis. In 1973, Heffelfinger et al described a chondroid variant of chordoma, called chondroid chordoma that was found to have a significantly better prognosis than classic chordoma. However, recent evidence suggests that many of the tumors diagnosed as chondroid chordoma may, in fact, be low-grade myxoid chondrosarcomas. This report presents the diagnosis and treatment of two cases of skull base tumor that were diagnosed preoperatively as schwannoma because they were thought initially to be centred on the jugular foramen. Initial histologic evaluation suggested chondroid chordoma, but immunohistologic techniques and a review of the literature led to a diagnosis of low-grade myxoid chondrosarcoma.

Adult↗

[Chondrosarcoma of the skull base associated with fatal intratumoral hemorrhage : report of an autopsy case and review of the literature].

We report a case of intracranial chondrosarcoma of the skull base with fatal intra- and peritumoral hemorrhage. A 75-year-old woman complained of right blepharoptosis and diplopia in 1989. An initial diagnosis of Tolosa-Hunt syndrome was made, and the patient was treated with steroid hormone therapy at a local hospital. Because the symptoms had not been relieved, she was admitted to our hospital. Computed tomography (CT) scan and magnetic resonance (MR) images demonstrated a large mass extending from the right side of the clivus to the parasellar region and petrous apex. The mass was partially calcified and had destroyed the base of the middle cranial fossa. The lesion had homogeneous enhancement with contrast medium. Preoperative diagnosis was chordoma or chondroma. A biopsy of the tumor was made. The pathological diagnosis of biopsy specimen was chondroid chordoma. The patient was followed up but no palliative treatment such as radiotherapy was given. On June 25, 1991, she suffered from cerebral infarction. On June 29, 1993, she died of sudden respiratory failure. Autopsy was performed. It revealed intra- and peritumoral hematoma compressing the medulla oblongata, pons and midbrain. Histologically immature chondroid cells proliferated in a myxoid-rich extracellular matrix. The tumor cells were composed of hyperchromatic nuclei and eosinophilic cytoplasm, but there was no evidence of notochordal differentiation. Compared with biopsy findings, the tumor showed high cellular density. Immunohistochemically, the tumor cells reacted positively for S - 100 protein, vimentin and cytokeratin, but negatively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). In view of these histopathological findings, the diagnosis of low-grade myxoid chondrosarcoma was established. Intratumoral hemorrhage often occurs in malignant brain tumors such as glioblastoma and metastatic brain tumor, but chondroid tumors rarely develop a fatal type of intratumoral hemorrhage. Only 8 cases have been reported in detail to date. We discuss the immunohistochemical features and spontaneous intratumoral hemorrhage of chondrosarcoma.

Aged↗

Metastasis of chondrosarcoma.

This study was undertaken to analyse metastases of patients with intermediate- or high-grade chondrosarcomas. Out of 24 intermediate-grade tumours, 5 (21%) developed metastases, as did 6 of 10 high-grade tumours (60%) (P = 0.04). Four patients developed pulmonary metastasis only, 5 developed both pulmonary metastases and metastases of the other sites. Two patients showed a rare metastatic pattern: bone metastases only. The metastasis rate in the primary chondrosarcoma (42%) was higher than that in the secondary chondrosarcomas (0%) (P = 0.03). The metastasis rate was higher in patients with local recurrence (86%) than in those without local recurrence (19%) (P = 0.01). In 5 of 6 patients who had a local relapse and metastasis, the interval between the two relapses was a few months.

Adult↗

The importance of initial presentation and treatment upon the survival of patients with chondrosarcoma.

The effect of initial presenting symptoms, degree of tumor differentiation and type of surgical procedure on survival was evaluated in a 20 year review of 42 patients with chondrosarcoma. Therapy of patients presenting initially with a painless mass resulted in a median survival period longer than 13 years versus less than three years of the initial symptom was pain. Median survival time of patients with well differentiated chondrosarcomas was more than ten years compared with 1.2 years if the tumor was less well differentiated. Even if the sarcoma was nonresectable, these factors influenced the survival time. Since chondrosarcomas tended to recur locally rather than to metastasize, a number of patients who had a recurrence after the initial surgical excision could be saved by a more radical secondary operation.

Adolescent↗

Mesenchymal chondrosarcoma in anterior skull base: case report.

We report the case of a 16-year-old boy who presented with mesenchymal chondrosarcoma involving the ethmoid sinus, right orbit, nasal cavity, and anterior cranial fossa. The tumor was totally resected by craniofacial surgery; the patient's postoperative course was uneventful. Microscopically, the tumor was composed of highly cellular undifferentiated small cells, alternating with zones of cartilaginous tissues. Mesenchymal chondrosarcoma is a rare, aggressive variant of chondrosarcoma, which might be encountered more frequently due to the advances in neuroimaging and surgical procedures.

Adolescent↗

Primary chondrosarcoma of the heart: a case report.

Chondrosarcoma involving the heart has been previously reported but is extremely rare. Most chondrosarcoma described in the literature are secondary, and the primary site can be identified. We present an unusual case of primary cardiac chondrosarcoma which originated in the left atrium. Severe pulmonary edema was the first presentation. The sarcoma subsequently metastasized to the mandible and finally recurred in the right heart causing severe pulmonary emboli and myocardial failure.

Adult↗

Chondrosarcoma of the nasal cavity, paranasal sinuses, and nasopharynx.

Thirteen patients, six men and seven women, were seen at the Mayo Clinic with chondrosarcomas of the nasal cavity, paranasal sinuses, or nasopharynx in a 25-year period. Nasal obstruction, discharge, and bleeding were the major symptoms and a nasal mass was the most common sign. The typical chondrosarcoma is low in grade but malignant and it arises in the nasal cavity as a large, pale, glistening mass. Local excision was employed initially in seven patients and five had local recurrence. Definitive block excision cured four of six patients and the two others had a protracted clinical course and ultimately died of the disease. Long-term follow-up shows that chondrosarcomas are insidious, locally progressive tumors. Radiotherapy, used for palliation after recurrence, failed to produce any cures. Lateral rhinotomy and block excision are advocated as the primary treatment.

Adolescent↗

Effect of intraperitoneally, intravenously and intralesionally administered monoclonal anti-beta-FGF antibodies on rat chondrosarcoma tumor vascularization and growth.

The growth and vascularization of many tumours has been reported to be associated with the overexpression of the potent mitogenic and angiogenic polypeptide basic fibroblast growth factor (beta-FGF). Consequently, it has been proposed that inhibition of beta-FGF action would prevent the growth of beta-FGF-dependent tumours. In this study, cell culture assays were established to assess the ability of mouse monoclonal DG-2 anti-beta-FGF antibodies to inhibit the mitogenic action of beta-FGF in vitro. Following in vitro characterisation, the monoclonal DG-2 antibodies were used to evaluate the role of beta-FGF in promoting the vascularization and growth of rat chondrosarcoma tumours. The effect the monoclonal anti-B-FGF antibodies had on tumour vascularization and growth in vivo were monitored using a 99m Technetium (99mTc)-labelled red blood cell procedure. The characterization studies confirmed that the DG-2 monoclonal antibody recognised beta-FGF and inhibited its mitogenic action on mouse Balb/c cells and bovine endothelial cells in vitro. When examined in vivo, intralesional administration of mouse monoclonal DG-2 antibody significantly inhibited rat chondrosarcoma growth and vascularization. However when the monoclonal DG-2 antibody was administered intraperitoneally or intravenously no attenuation of rat chondrosarcoma tumour vascularization or growth was observed. This report has confirmed the potential effectiveness of anti-beta-FGF antibodies in the regulation of tumour growth. It has also demonstrated that further studies on the pharmacokinetics of administered antibodies and their mode of delivery are required so that the effectiveness of such anti-growth factor immunotherapy can be assured.

3T3 Cells↗

Chondrosarcoma: clear-cell variant. A report of sixteen cases.

Of sixteen adult patients with clear-cell chondrosarcoma, ten were men. The tumor usually involved the proximal part of the femur or humerus. Roentgenographically, the lesion was usually well defined and indistinguishable from chondroblastoma. Serial roentgenograms occasionally showed malignant progression of the lesion. Histologically, benign giant cells and cells with clear cytoplasm were always present, and areas of conventional chondrosarcoma were seen in about half the cases. Excision usually resulted in recurrence; resection seemed to be curative. Four of the sixteen patients died as a result of the tumor. Clear-cell chondrosarcoma should be distinguished from chondroblastoma and treated more aggressively.

Adult↗

Association between ratio of matrix metalloproteinase-1 to tissue inhibitor of metalloproteinase-1 and local recurrence, metastasis, and survival in human chondrosarcoma.

Chondrosarcoma, a malignant cartilage-forming mesenchymal tumor, displays a wide range of clinical behavior that can be difficult to predict with histological analysis. Matrix metalloproteinases contribute to the processes of local invasion and metastasis by controlling the ability of a tumor to transverse tissue boundaries. The specificity of matrix metalloproteinase-1 (interstitial collagenase) for fibrillar collagen may be central to those processes. Matrix metalloproteinase-2 facilitates invasion by degradation of such basement-membrane structures as type-IV collagen. The balance between the activity of tissue inhibitors of metalloproteinase and the activity of matrix metalloproteinase determines the proteolytic activity and may, in part, determine the overall invasiveness and potential for metastasis. The measurement of the ratio of matrix metalloproteinase to tissue inhibitor of metalloproteinase may have prognostic value for determining whether individual chondrosarcomas are locally invasive or will metastasize. Furthermore, there may be a specific pattern of expression of matrix metalloproteinase and tissue inhibitor of metalloproteinase in chondrosarcomas that is related to local invasion and probability of metastasis. Sixteen paraffin-embedded archival specimens of tumors were examined. Six twenty-micrometer-thick sections were cut from each tumor, and the amounts of cDNA formed from the mRNA were determined with reverse transcription-polymerase chain reaction with use of novel primers for matrix metalloproteinase-1, matrix metalloproteinase-2, tissue inhibitor of metalloproteinase-1, and tissue inhibitor of metalloproteinase-2. The amounts of cDNA for the matrix metalloproteinases and their inhibitors were determined by chemiluminescence and band densitometry. The ratio of the amount of cDNA for matrix metalloproteinase-1 to that for its tissue inhibitor and the ratio of the amount of cDNA for matrix metalloproteinase-2 to that for its tissue inhibitor were calculated, and the results were compared with use of the Student t test, enabling log-rank analysis of Kaplan-Meier survival curves. These ratios as well as the age and gender of the patient; the grade, size, and location of the tumor; the type of adjuvant therapy; and the operative margins were examined for significance with use of stepwise logistic-regression analysis. The patients who had recurrent disease had a significantly higher (p < 0.003) ratio of matrix metalloproteinase-1 to tissue inhibitor of metalloproteinase-1 (mean, 0.939; range, 0.647 to 1.101) than the patients who were free of disease (mean, 0.703; range, 0.629 to 0.772). Moreover, there was a striking difference between the Kaplan-Meier survival curve associated with a high ratio (more than 0.8) and that associated with a low ratio (p = 0.0015). The mean ratio of matrix metalloproteinase-2 to tissue inhibitor of metalloproteinase-2 was 1.814 (range, 1.206 to 3.77) in the patients who had recurrent disease compared with 1.473 (range, 1.073 to 2.390) in those who were free of disease; this difference was not found to be significant, with the numbers available. Analysis of the survival curves indicated that a worse prognosis was associated with a high ratio, but again this relationship was not found to be significant. Regression analysis revealed that a high ratio of matrix metalloproteinase-1 to its tissue inhibitor was a moderately significant independent predictor of a poor outcome (alpha = 0.07).

Adult↗

[Clear cell chondrosarcoma (CCCS) of the nasal septum].

INTRODUCTION: Chondrosarcomas belong to the most common malignomas of the skeletal system. The vast majority is located at the epiphysis of the long bones or in the pelvis. Less than 10% of chondrosarcomas are located in the head and neck area, most common in the bony viscerocranium and the larynx. Five histological variants can be differentiated: conventional, mesenchymal, myxoid, dedifferentiated and in less than 5% clear cell chondrosarcomas (CCCS). This case report gives the first description of a CCCS originating from the nasal septum. CASE REPORT: A 79 year old female complaining about a 6 month history of nasal obstruction revealed a huge ballooning of the basal septum in anterior rhinoscopy. Computed tomography exhibited a septal tumor with amorphous calcification. Histology of the tumor resected via lateral rhinotomy confirmed a diagnosis of CCCS. Because of clear surgical margins no radiation therapy was performed. The patient stayed free of recurrence during follow-up of sixteen months. DISCUSSION: The patient presented with typical symptoms of nasoseptal tumors as well as representative CT-scans and histologic findings of CCCS. Because of the complete resection and the CCCS-subtype, the patient is expected to have a positive prognosis.

Aged↗

Maffucci's Syndrome and Intracranial Chondrosarcoma.

Maffucci's syndrome is a rare, congenital mesenchymal dysplasia characterized by multiple enchondromata and hemangimata, both of which may undergo malignant degeneration. Intracranial involvement is uncommon. A literature review yielded only six cases of Maffucci's syndrome with intracranial chondrosarcoma and two cases of Ollier's disease (enchondromata alone) with intracranial chondrosarcoma. We report the seventh case of Maffucci's syndrome in a patient with a very large and extensive skull base chondrosarcoma requiring staged operations for removal.

Case Reports↗

Chondrosarcoma of the jugular foramen.

Chondrosarcoma, although uncommon, must be included in the differential diagnosis of skull base tumors. The potential for morbidity and mortality, similar to other lesions located at this anatomic site, is related to compromise of adjacent vital neurovascular structures. Optimal surgical management is controversial and reliant on adequate tumor exposure. A case of myxoid chondrosarcoma of the jugular foramen is presented with a review of the common presenting signs and symptoms along with the radiographic and histologic features of these lesions. The surgical approach to chondrosarcoma and other lesions at this location has undergone an evolution in technique in recent years. This report details the approach used by the authors, which allows for reconstruction of the skull base with subsequent normal anatomic contour and functional hearing. The technique employs hydroxylapatite for reconstruction, which has not been previously described in skull base applications.

Adult↗