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Low-grade myxoid chondrosarcoma of the base of the skull: CT, MR, and histopathology.

A retrospective CT, MR, and histopathologic study was performed in five patients with histologically verified low-grade myxoid chondrosarcoma of the base of the skull. In four patients, the tumor originated off the midline and was associated with bone destruction at the petrous apex near the petrooccipital fissure. Tumor extent included the cerebellopontine angle in three patients and the parasellar area in two patients. The fifth tumor originated on the midline and was associated with destruction at the dorsum sellae. Three tumors contained calcifications, whereas two lesions were mostly isodense with brain on CT scan. All tumors were hypointense on T1-weighted MR images and very intense on T2-weighted images except for areas of signal void consistent with calcifications. Light microscopy revealed islands of mature hyaline cartilage in an abundant myxoid ground substance. Histology and immunocytochemical analysis were consistent with a low-grade myxoid chondrosarcoma. The CT and MR features of low-grade myxoid chondrosarcoma are comparable with those of chordoma. Chordoma usually arises from the midline, but cases with origin from the lateral portion of the clivus or the petrous apex have been described. Low-grade myxoid chondrosarcoma has distinct histologic and immunocytochemical features and includes lesions formerly called "chondroid chordomas."

Adolescent↗

The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base.

Despite conventional multimodality treatment (surgery and fractionated radiation therapy), recurrence and clinical progression of cranial base chordomas and chondrosarcomas are common. The malignant behavior of these tumors is a result of their critical location, locally aggressive nature, and high recurrence rate. To explore the role of radiosurgery in the treatment of these skull base neoplasms, we assessed its use in four patients with chordoma and two with chondrosarcoma. In five of the patients, radiosurgery was used as adjuvant therapy for residual or recurrent tumors after surgical debulking, and in one patient with a chordoma, it was the primary treatment. No patient received fractionated external beam radiotherapy. All tumors were less than 30 mm in diameter and were treated with 20 Gy to the tumor margin. Skull base computed tomography and magnetic resonance images were essential to define the anatomic relationships between tumor and adjacent basal structures. During follow-up (mean, 22 mo; range, 8-36 mo), we found no progression of the treated tumor volume in any patient. Neurological deficits before treatment improved in three patients; the other three patients remained in stable neurological condition. Serial follow-up imaging studies demonstrated that two patients showed reduction in tumor size and four patients had no tumor growth. In one patient, a metastatic parietal lobe chondrosarcoma developed and was treated by microsurgery. Another patient showed tumor progression outside of the radiosurgical treatment volume. Our results attest to the value of stereotactic radiosurgery as an adjuvant or primary treatment for selected patients with chordoma or chondrosarcoma and demonstrate its potential advantages over standard fractionated irradiation. Analysis of the long-term clinical and imaging effects after radiosurgery is warranted.

Adult↗

Chondrosarcoma of the mobile spine: report on 22 cases.

STUDY DESIGN: A retrospective review of 22 cases of chondrosarcoma arising from the mobile spine. OBJECTIVE: To evaluate the role of oncologic and surgical staging in correlating management and outcome of chondrosarcoma involving the spine. SUMMARY OF BACKGROUND DATA: Approximately 10% of chondrosarcomas arise from the mobile spine, occurring mainly in adults, particularly elderly men. The course of the disease depends on the aggressiveness of the tumor, but also is influenced by the management. Intralesional surgery is followed almost constantly by local recurrence even with adjuvant therapy. METHODS: All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical staging of the reviewed cases. According to Enneking criteria, the surgical procedures were defined as curettage (piecemeal excision) or en blocexcision. The margins were submitted to histologic study and reported as intralesional, marginal, or wide. - As primary management, 10 intralesional curettages (follow-up period, 2-119 months; average, 61 months) and 12 en bloc excisions (follow-up period, 39-207 months; average, 97 months) were performed. A total of 33 procedures were performed, including the management of the recurrences (18 curettages and 15 en bloc excisions: one for soft tissue recurrence). A clinical and radiographic follow-up period of of 2 to 236 months (average, 81 months; minimal follow-up period for survivors, 30 months; average follow-up period for survivors, 115 months) was available for all the patients. RESULTS: Three recurrences occurred in 14 patients treated by en bloc excision at onset or for recurrence, two in cases of histologically proven contaminated or intralesional margins. All but one patient were alive at final follow-up evaluation. Conversely, all the patients treated by one or more curettages (with or without adjuvant radiation therapy) had at least one recurrence, and 8 of 10 of these patients died of the disease. At final follow-up evaluation, nine patients had died of the disease; nine were continuously disease free (but one had died of another unrelated malignancy); and four were symptom free after management for recurrences (one was found alive 155 months after a soft-tissue metastasis en-bloc excision). CONCLUSIONS: En bloc excision, with wide or marginal histologic margins, is the suggested management for chondrosarcomas of the spine. Early diagnosis and careful surgical staging and planning are necessary for conducting adequate management. However, tumor contamination of the specimen margins, even in a small area, or spreading of the tumor myxoid content can worsen the prognosis.

Adult↗

Primary nasal chondrosarcoma. The pediatric experience.

Chondrosarcomas are infrequently found in the head and neck region. In a review of nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx, only 4% were chondrosarcomas. The peak incidence occurs in the fourth decade of life. Only four cases of head or neck chondrosarcoma in children less than or equal to 10 years of age have been described in the literature, and only two of these were primary nasal cavity tumors. We present in this report a case of a 5-year-old boy treated with adjuvant chemotherapy after complete resection of a nasal cavity chondrosarcoma, and a review of the literature on this topic.

Age Factors↗

Secondary chondrosarcoma in osteochondroma: report of 107 patients.

Secondary chondrosarcomas are rare; recognition and diagnosis are difficult. Slow growth and late recurrence require long-term followup to understand the clinical course. In the current study, 107 patients had secondary chondrosarcoma arising in a solitary osteochondroma (61 patients) or multiple exostoses (46 patients). All histologic slides were reviewed without knowledge of the outcome, and radiologic studies were available for review in 71 cases. Patients with secondary chondrosarcoma were one to two decades younger than those with primary chondrosarcoma. Male preponderance and a predilection for flat bones were observed. The radiologic signs of sarcomatous degeneration included irregularity of the margin, inhomogeneous mineralization, and an associated soft tissue mass. The tumors generally were well-differentiated. Only 10 tumors were classified as Grade 2. Five-year and 10-year local recurrence rates were 15.9% and 17.5%, respectively, and 5- and 10-year mortality rates were 1.6% and 4.8% for patients having initial treatment at the authors' institution. Metastasis developed in five patients: in the lung in four patients and in the groin region in one patient. Most patients who died of tumor died of local recurrence. Wide excision had the lowest local recurrence rate. With successful surgical treatment, patients may have long-term disease-free survival.

Adolescent↗

Chondrosarcoma of the chest wall: a retrospective clinical analysis.

Primary chondrosarcoma of the chest wall is uncommon, and reports of treatment from one institution are limited. Treatment of this lesion is difficult because of the anatomic location, which is in close proximity to surrounding neurovascular and visceral structures. The purpose of this study was to review the outcome of surgical resection of chondrosarcoma of the chest wall, and to delineate the prognostic factors related to local recurrence and survival. Twenty-four patients with chondrosarcoma of the chest wall were treated at our institution between 1986 and 2000. There were 14 males and 10 females, with an average age of 54 years (range, 11-76 years). Patients were observed for a minimum of 3 years or until death. The median followup was 71 months. The anatomic locations of chondrosarcoma of the chest wall were the rib in 16 patients, the ribs and sternum in two patients, the ribs and spine in three patients, and the sternum only in three patients. Histologically, 17 patients had Grade 1 disease and seven patients had Grade 2 disease. At followup, 17 patients were alive without disease, two were alive with disease, two died without disease, and three died secondary to progressive disease. The overall survival estimate at 5 years was 92%. The recurrence rate for patients with adequate surgical margins was 10%, compared with 75% for patients with inadequate margins. The 5-year survival rate for patients with adequate surgical margins was 100%, compared with 50% in patients with inadequate surgical margins. An inadequate margin of resection was associated with a significantly worse overall survival and a higher chance of having local recurrence develop.

Adolescent↗

An institutional review of clear cell chondrosarcoma.

UNLABELLED: Clear cell chondrosarcoma is a rare bone neoplasm with a slow progressive clinical course and infrequent metastasis, but with a high local recurrence rate. We sought to ascertain the long-term outcome of patients with this neoplasm and to identify possible factors predicting survival. Sixteen patients with clear cell chondrosarcomas treated at one institution and who had long-term clinical followup were identified. All patients were treated by surgical resection of the tumor, which was classified as clear (> 2 mm) in 10 patients and marginal or intralesional in six patients. Three patients had local recurrence after a median of 1.7 years. Metastatic disease developed in four patients with a median time to diagnosis of 8.1 years. Ten-year overall survival of patients with clear cell chondrosarcomas was 89%, and disease-free survival was 68%. Patients with surgical resections comprising clear margins had longer disease-free survival compared with patients with marginal and intralesional tumors. Inadequate surgical resection of clear cell chondrosarcoma leads to risk of local recurrence and metastatic disease. The malignancy has a tendency to metastasize relatively late, therefore, followup of patients is necessary after the generally accepted 5-year period. LEVEL OF EVIDENCE: Prognostic study, Level III (case control study). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗

Mesenchymal chondrosarcoma treated with total en bloc spondylectomy for 2 consecutive lumbar vertebrae resulted in continuous disease-free survival for more than 5 years: case report.

STUDY DESIGN: A case report of an extremely rare malignant spinal tumor successfully treated with total en bloc spondylectomy and chemotherapy. OBJECTIVE: To describe points for consideration when an osteogenic lesion in the spine is diagnosed and treated. SUMMARY OF BACKGROUND DATA: Primary mesenchymal chondrosarcoma in the spine is extremely rare. There were no reports of this tumor being treated with spondylectomy to achieve total surgical resection with a wide margin followed by chemotherapy. METHODS: A 44-year-old female presented with low back pain and left flank pain. Magnetic resonance imaging and computerized tomography showed an osteosclerotic tumor of the lumbar vertebrae. Tc-99m HMDP bone scintigraphy was positive, but thallium-201 scintigraphy and gallium scintigraphy were negative. The patient was diagnosed as having chondrosarcoma based on biopsy findings. RESULTS: To resect the tumor completely, total en bloc spondylectomy for 2 consecutive lumbar vertebrae was performed. However, the postoperative pathologic diagnosis was extremely difficult because the patient was initially suspected to have osteosarcoma, but the final diagnosis was mesenchymal chondrosarcoma. Five years after surgery, there have not been any signs of local recurrence or distant metastasis, and the patient has remained continuously disease free. CONCLUSIONS: To our knowledge, we reported the first case of mesenchymal chondrosarcoma occurring from the lumbar spine treated with total en bloc spondylectomy and chemotherapy. Successful radical resection of the tumor could be accomplished. Although the effect of chemotherapy on the final results could not be clearly determined, considering that at least continuous disease-free survival was achieved, it is highly likely that chemotherapy contributed to the favorable results.

Adult↗

Mesenchymal chondrosarcoma of the orbit.

PURPOSE: To describe a patient with mesenchymal chondrosarcoma of the orbit, review clinical and imaging findings, and refine the differential diagnosis from other tumors of the orbit. METHODS: Interventional case report and literature review. RESULTS: A 21-year-old woman with a 6-month history of progressive proptosis presented with left-sided visual loss of recent onset. CT revealed a clearly outlined heterogeneous mass with calcified foci, whereas MRI showed an isointense signal to gray matter on T1- and T2-weighted images. The patient was diagnosed with mesenchymal chondrosarcoma of the orbit only after orbitotomy and subsequent histopathologic study. After surgery, she received adjuvant therapy with irradiation of the orbit. CONCLUSIONS: Mesenchymal chondrosarcoma is a rare malignant tumor of the orbit capable of mimicking several other lesions. Early diagnosis requires a high level of suspicion, particularly with regard to internally calcified lesions. The treatment of choice is complete surgical resection, or, when the tumor is not resectable or residual mass is present after surgery, chemotherapy and radiotherapy. Prognosis for patients with orbital mesenchymal chondrosarcoma is tentative at best, in part because of the rarity of the lesion.

Adult↗

Establishment and characterization of a continuous human chondrosarcoma cell line, ch-2879: comparative histologic and genetic studies with its tumor of origin.

Chondrosarcomas are malignant cartilage-forming tumors that represent the second most common malignant solid tumor of bone. These biologically poorly understood neoplasms vary considerably in clinical presentation and biologic behavior. Chemotherapy and radiation therapy are generally ineffective. Here we describe the establishment and characterization of a new human chondrosarcoma cell line named ch-2879, and we compare the cell line with its tumor of origin. The cell line was established from a recurrent grade 3 chondrosarcoma of the chest wall and characterized by growth kinetics and morphologic studies. Immunocytochemistry and RT-PCR were performed to examine the expression of cartilage-specific phenotypes. Genetic characterization was performed using cytogenetics, fluorescence in situ hybridization, flow cytometry, and molecular techniques for analysis of the genes implicated in cell cycle control, amplification of MDM2, CDK4, and Cyclin D1, and mutations in the p53 gene. ch-2879 cells were subcultured for more than 80 passages. They expressed vimentin, HNK-1, HBA-71, Ki-67, cyclin D1, Fli-1, S-100, p21, p27, and p53 and were negative for cytokeratin, EMA, p14, p16, MDM2, Rb, and c-erb-b2 antigens. Cytogenetically the recurrent tumor showed a hyperhaploid karyotype with clonal numerical and structural abnormalities. The sole structural abnormality was a chromosome derivative of a t(1;21) translocation. The cell line at passage 3 showed two populations: the hyperhaploid and an exactly duplicated, hypotriploid population. After the 18th passage, only the hypotriploid population was present. The cells expressed collagen 2. Molecular comparison of the primary and recurrent tumor evidenced an in vivo molecular change consisting of a deletion of 9p21 genes in the recurrence, probably caused by a selection process. Because of its gene expression profile, including expression of genes implicated in chondrogenesis in uncoated plastic dishes, this cell line may prove useful for cellular and molecular studies as well as studies of chondrosarcoma characterization and treatment.

Bone Neoplasms↗

Clear cell chondrosarcoma. A case report with topographic analysis.

A case of clear cell chondrosarcoma located in the right proximal end of the femur is presented. Radiologically, the tumor appeared as a large osteolytic lesion with mottled calcification resembling chondroblastoma. Histologically, the greater portion was made up of tumor cells with abundant clear cytoplasm and usually distinct boundaries, associated with areas of conventional chondrosarcoma. The proportion of the clear cell areas was about 60%, and they were mainly distributed along the tumor periphery. Moreover, myxoid chondrosarcoma-like areas, a feature not previously mentioned in clear cell chondrosarcoma, were observed, occupying about 10% of the tumor area.

Adult↗

True pulmonary carcinosarcoma (squamous cell carcinoma and chondrosarcoma). A case report.

True pulmonary carcinosarcoma (squamous cell carcinoma and chondrosarcoma) originating in the right lower lobe in a 62-year-old Japanese male is reported. The tumor, measuring 5.5 x 3.5 x 3.5 cm, was markedly necrotic and its apex protruded into the bronchial lumen. Light microscopy showed that the tumor was composed of squamous cell carcinoma with sarcomatous spindle or polygonal cell proliferation and true chondrosarcoma. Immunohistochemically, the cytoplasm of numerous cells of the squamous cell carcinoma component was stained with anti-cytokeratin (PKK 1) and the cytoplasmic membrane with anti-epithelial membrane antigen (EMA). Although sarcomatous regions were stained with anti-vimentin (vimentin) and no tumor cells were reactive for EMA, a few tumor cells were positive for PKK 1. The cytoplasm of numerous chondrosarcoma cells was positively stained for vimentin and S-100 protein. Based on these findings, we concluded that the present tumor was a true carcinosarcoma composed of squamous cell carcinoma with sarcomatous regions and true chondrosarcoma.

Aged↗

Dedifferentiated chondrosarcoma with telangiectatic osteosarcoma-like features.

A 35-year-old Japanese man was admitted to the National Cancer Center, Tokyo, Japan, in December 2000, with a 2-month history of pain around the left thigh. Radiographs showed a poorly demarcated osteolytic lesion with focal mineralisation and endosteal scalloping in the left proximal femur. Biopsy showed a proliferation of highly anaplastic cells without any cartilaginous component. A wide excision of the left proximal femur with a replacement by endoprosthesis was carried out in February 2001 after treatment with methotrexate and 20 Gy radiation therapy. Pathological examination of the surgical specimen showed a focus of low-grade chondrosarcoma and the coexistence of telangiectatic osteosarcoma-like features. The patient was diagnosed with dedifferentiated chondrosarcoma with telangiectatic osteosarcoma-like features. Lung metastasis appeared in July 2001 despite an adjuvant chemotherapy including methotrexate, cis-platinum and doxorubicin. The latest follow-up study in June 2004 showed multiple lung metastases. Establishing a definitive diagnosis of dedifferentiated chondrosarcoma may be difficult with limited small biopsy specimens. Dedifferentiated chondrosarcoma should be included in the differential diagnosis of osteolytic tumours with focal calcification and endosteal scalloping even if an extraosseous tumour component is not identified.

Adult↗

High quality RNA isolation from tumours with low cellularity and high extracellular matrix component for cDNA microarrays: application to chondrosarcoma.

AIMS: High quality RNA isolation from cartilaginous tissue is considered difficult because of relatively low cellularity and the abundance of extracellular matrix rich in glycosaminoglycans and collagens. Given the growing interest and technical possibilities to study RNA expression at a high throughput level, research on tissue with these characteristics is hampered by the lack of an efficient method for obtaining sufficient amounts of high quality RNA. METHODS: This paper presents a robust protocol combining two RNA isolation procedures, based on a combination of Trizol and RNA specific columns, which has been developed to obtain high molecular weight RNA from fresh frozen and stored tissue of normal cartilage and cartilaginous tumours. Using this method, RNA was isolated from normal cartilage, peripheral chondrosarcoma, and central chondrosarcoma. RESULTS: The yields ranged from 0.1 to 0.5 microg RNA/mg tissue. RNA isolated with this method was stable and of high molecular weight. RNA samples from normal cartilage and from two chondrosarcomas isolated using this method were applied successfully in cDNA microarray experiments. The number of genes that give interpretable results was in the range of what would be expected from microarray results obtained on chondrosarcoma cell line RNA. Signal to noise ratios were good and differential expression between tumour and normal cartilage was detectable for a large number of genes. CONCLUSION: With this newly developed isolation method, high quality RNA can be obtained from low cellular tissue with a high extracellular matrix component. These procedures can also be applied to other tumour material.

Bone Neoplasms↗

Radiographic hallmarks of peripheral chondrosarcoma.

Ten peripheral chondrosarcomas, nine arising in the cap of an osteochondroma and one developing as a primary periosteal tumor, are reported. The radiological and histological features of these tumors are compared with those of 203 benign osteochondromas and two soft-tissue chondrosarcomas. In 3 of the 10 cases, cytological evidence of malignancy could not be demonstrated, although the size of the tumors and the presence of extension into adjacent soft tissues indicated that they were low-grade chondrosarcomas. In all of the 10 cases there was radiological evidence of scattered calcifications in the cartilaginous part of the tumor, and this is regarded as an important radiological indication of malignancy. Histological studies indicate that calcification of tumor cartilage, followed by replacement of the calcified cartilage by endochrondral bone, is responsible for the opacities that, in clinical radiographs, are characteristic of chondrosarcoma.

Adult↗

Radiographic, CT, and MR imaging features of dedifferentiated chondrosarcomas: a retrospective review of 174 de novo cases.

Up to 11% of chondrosarcomas may undergo regional anaplastic change, resulting in a high-grade noncartilaginous sarcoma arising within a typically low-grade chondrosarcoma. Known as dedifferentiated chondrosarcomas, these tumors are highly malignant with a very poor prognosis. The most important factor affecting survival is an accurate preoperative diagnosis. Therefore, the ability to predict the possibility of dedifferentiation in a malignant cartilage tumor on the basis of imaging findings is critical to ensure adequate tumor sampling at the time of biopsy. Imaging findings at radiography, computed tomography (CT), and magnetic resonance (MR) imaging in 174 patients with dedifferentiated chondrosarcoma were reviewed to determine whether there are radiologic features that can help predict dedifferentiation. On approximately one-third of the radiographs, one-third of the MR images, and one-half of the CT scans, the tumors demonstrated bimorphic features (ie, distinctly different tumor features juxtaposed within the lesion), most frequently a dominant lytic area adjacent to a mineralized tumor at radiography and a large, unmineralized soft-tissue mass associated with an intraosseous chondroid-containing tumor at CT and MR imaging. In the initial evaluation of patients with a primary bone tumor, thorough evaluation of the radiologic features of the entire tumor is critical.

Adolescent↗

Functional treatment of a large laryngeal chondrosarcoma by tracheal autotransplantation.

Chondrosarcoma of the cricoid cartilage poses difficult therapeutic challenges. The tumor necessitates resection of parts of the cricoid cartilage with a resulting defect that is difficult to repair. We wanted to improve the functional outcome after resection of a lateralized chondrosarcoma by applying the technique of tracheal autotransplantation. The technique involves a 2-stage procedure, because the trachea needs at least 2 weeks for revascularization. Tracheal revascularization is the first stage, and is accomplished by wrapping the trachea in vascularized fascia (radial forearm). The second stage, performed after 14 days, consists of a hemilaryngectomy with tracheal autotransplantation. A case of a large unilateral chondrosarcoma of the cricoid cartilage with involvement of 1 cricoarytenoid joint is reported. The tumor was removed by hemicricohemilaryngectomy. After 2 weeks of tracheal revascularization, the cervical trachea was used to repair the laryngeal defect. The tumor was completely resected, and all laryngeal functions (swallowing, voice, respiration without tracheostomy) were restored. The patient remains tumor-free after a follow-up period of 3 years. Tracheal autotransplantation improves the functional treatment of lateralized chondrosarcomas of the cricoid cartilage.

Chondrosarcoma↗

Hormone responsiveness of a transplantable rat chondrosarcoma. II. Evidence for in vivo hormone dependence.

We have previously demonstrated that the Swarm rat chondrosarcoma responds to GH-dependent serum factors in vitro by increasing amino acid transport and macromolecular synthesis. The question of in vivo hormone dependence was evaluated by studying the growth of the tumor in hypophysectomized rats. Tumor-bearing hypophysectomized rats were treated with saline, T4, cortisone, bovine GH (bGH), or combinations of these hormones. Tumor growth was assessed in terms of tumor weight. Histological appearance was studied to ascertain the viability of the tumors and the relative contributions of cellularity vs. cartilage matrix to the weight of the chondrosarcoma. The weight of tumors grown in saline-treated hypophysectomized rats was less than 10% of the weight attained by tumors grown in normal rats for a comparable period of time. There was a greater relative cellularity in tumors grown in normal and hormone-treated hypophysectomized rats compared to that in tumors grown in saline-treated hypophysectomized rats. Tumors from saline-treated hypophysectomized rats had an atrophic appearance. Treatment of the hypophysectomized rats with bGH or cortisone increased the rate of tumor growth 5- to 6-fold and restored the histological appearance toward that of tumors grown in normal rats. A greater rate of tumor growth was effected by treatment with a combination of bGH and cortisone. T4 by itself did not stimulate chondrosarcoma growth in the hypophysectomized rat. Withdrawal of bGH and cortisone treatment from hypophysectomized rats after tumors were hormone stimulated caused a loss of the apparent exponential rate of growth observed in hypophysectomized rats treated through the full 38-day observation period. These data indicate that bGH and cortisone act in concert to stimulate the growth of the Swarm rat chondrosarcoma in vivo and that the tumor is hormone dependent.

Animals↗