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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 tumors (60%) (P = 0.04). Four patients developed pulmonary metastasis only, 5 developed both pulmonary metastasis 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.

Cytomegalovirus↗

Primary malignant mesenchymoma of bone: case report, literature review, and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma.

The seventh case of primary malignant mesenchymoma of bone is reported. It is compared to the other six cases in the literature and clinicopathologic pattern of this entity is developed. Primary malignant mesenchymoma of bone is compared to mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Differences as well as similarities are discussed and a more current classification scheme for malignant mesenchymoma of bone is proposed.

Bone Neoplasms↗

Mesenchymal chondrosarcoma: a series of 23 cases.

A total of 23 cases of mesenchymal chondrosarcoma were studied from the histopathology records spanning 23 years. There were 16 men and 7 women afflicted with this tumor and their mean age was 28.1 years. The radiological features, histology and treatment outcomes have been studied. Osseous and extra-osseous mesenchymal chondrosarcomas are compared and the differential diagnosis discussed.

Adolescent↗

Radiologic diagnosis of chondroma and chondrosarcoma of the larynx.

The radiographic findings in chondroma and chondrosarcoma of the larynx basically comprise a discreet soft tissue mass on plain lateral and anteroposterior film, a smooth mucosal outline to the lesion, calcification, with either peripheral or central stippling and ossification. Secondary signs are also noted. Definition of these criteria are illustrated in order to apply these findings to the radiographic diagnosis of chondroma and chondrosarcoma of the larynx.

Aged↗

Frequent loss of heterozygosity for markers on chromosome arm 10q in chondrosarcomas.

Many tumors exhibit loss of heterozygosity (LOH) for polymorphic markers in regions of the genome that contain genes whose normal function can suppress tumor growth. Mapping of regions of LOH can help identify putative tumor suppressor loci that play a role in the pathogenesis of a disease. We evaluated 18 chondrosarcomas for LOH at 17 short tandem-repeat polymorphism loci on chromosome 10. Sixty-seven percent of the tumors (12/18) showed LOH for at least one marker and in most of these tumors the region of loss spanned all or large portions of the chromosome. By determining the smallest segment consistently involved, we identified a 7-12 cM critical region for LOH in the proximal long arm. This genomic region contains the RET oncogene, which has been implicated in the pathogenesis of multiple endocrine neoplasia types 2A and 2B, Hirschsprung disease, and medullary and papillary thyroid carcinomas. LOH on chromosome arm 10q was found in early-stage chondrosarcomas and did not correlate with grade or prognosis. Inactivation or alteration of a gene located at this site may be an early event in the development of these tumors.

Bone Neoplasms↗

Electrophoretic and serologic characterization of 56 kDa antigen (M56) with autologous serum derived from a chondrosarcoma patient: a shared antigen of immunoresponses in cancer and autoimmune diseases.

We investigated whether antibodies specific to autologous cancer cells are produced in the peripheral blood of patients with chondrosarcoma. There have been few reports on the investigation of the immune responses, such as autologous antibody production, to chondrosarcoma. Here, tumor-associated antigens were separated by sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis and detected by immunoenzymatic amplification. A 56 kDa molecule (M56) was detected in the serum from patients' peripheral blood. M56 is ubiquitously expressed in various kinds of tissue-derived cells. However, the molecule seemed to be retained mostly in the cytosolic compartment of lymphoid cells, while it was expressed on the cell surface of nonlymphoid cancer cells. Furthermore, the antibodies reactive to the 56 kDa molecule were frequently observed in sera derived from patients with other cancers and autoimmune diseases as compared to the sera from healthy control donors, suggesting that M56 is a common target molecule of immune responses in patients with various cancers and autoimmune diseases.

Animals↗

Extraskeletal myxoid chondrosarcoma: a clinicopathological study.

Extraskeletal myxoid chondrosarcoma is a rare tumor of cartilagenous origin, with only 41 previously reported cases. It has a distinctive histologic pattern and should be distinguished from chondrosarcoma of bone because of its different behavioral pattern. The light microscopic, histo-chemical and electron microscopic features of such a tumor are reported.

Adult↗

Meningeal mesenchymal chondrosarcoma: report of 8 cases with review of the literature.

This paper reviews 8 personally examined cases of primary meningeal mesenchymal chondrosarcoma and 4 similar cases previously reported by others. The clinicopathologic features of these extraosseous intracranial and intraspinal examples are similar to those of other extraskeletal mesenchymal chondrosarcomas. The tumor occurred most often in the second and third decades, showed a moderate tendency to local recurrence (5 of 12 cases) and occasionally metastasized to the lungs (1 case). Both intracranial and intraspinal tumors occurred with equal frequency, but the former, probably due to the later onset of symptoms, had the worse prognosis. Microscopically, they are composed of primitive undifferentiated mesenchymal cells and frequently well-defined islands of hyaline cartilage. There is an apparent correlation between the frequency of mitotic figures and the likelihood of recurrence and metastasis. Electron microscopic study of one example revealed morphologic features similar to those previously described by others and supports the conclusion that the neoplastic cells represent primitive precartilaginous mesenchyme displaying focal cartilaginous differentiation.

Adolescent↗

Chondrosarcoma arising in osteochondroma.

Secondary chondrosarcomas are rare. An analysis of 75 cases of chondrosarcomas secondary to osteochondroma (42 lesions were from the Mayo Clinic files and 33 were from consultation files) revealed that 40 of the patients had single exostosis and 35 had multiple lesions. More males than females were affected, and most of the patients were age 20 to 40 years. The tumors involved various bones. Malignant change was manifested radiologically by fuzzy margins of the cartilage cap and by the presence of lucent zones within the lesion. Grossly, the surface of the thickened cartilage cap is irregular. Microscopically, most of the tumors were well-differentiated (Grade 1). Treatment was surgical. Simple excision of the tumor resulted in a large recurrence rate. Resection and amputation were generally curative. Only 12 of the patients have died of their tumor. Most patients died of local recurrence. Only two of the 75 patients had definite evidence of metastatic disease.

Adolescent↗

Mesenchymal chondrosarcoma of bone and soft tissues.

Mesenchymal chondrosarcoma of bone and soft tissues treated at the Istituto Ortopedico Rizzoli are reviewed. The skeletal locations were prominent in five cases. Only two cases were in the soft tissues. Radiographic picture in the bone shows an aggressive osteolysis, with soft tissues invasion. Histologic picture is the same in bone and soft tissues, and is highly distinctive: islands of well-differentiated chondrosarcoma embedded in undifferentiated mesenchymal cells with high malignancy characteristics. The cases showed a poor prognosis, and no patient survived more than four years.

Adult↗

Dedifferentiated chondrosarcoma. An ultrastructural study.

The light and electron microscopic features of a well-differentiated chondrosarcoma with dedifferentiated foci (dedifferentiated chondrosarcoma) is presented. Ultrastructurally, the cells of the dedifferentiated portion were embedded in a matrix containing numerous short bundles of collagen fibers; these cells showed dilated rough endoplasmic reticulum containing stippled material as well as poorly formed cell junctions and resembled the cells in the cartilaginous component. These findings contrast with those reported previously.

Aged↗

Multiple chondrosarcomas in dyschondroplasia (Ollier's disease).

The relationship between dyschondroplasia (Ollier's Disease) and the possibility of malignant change is well known. The authors describe a patient who developed four primary chondrosarcomas over 11 years. These were treated by local wide excision and, in two sites, prosthetic replacement to preserve the limb. To the authors' knowledge, no patient with more than two primary chondrosarcomas has previously been described.

Adult↗

Chondrosarcomas of the synovium.

Chondrosarcoma of the synovium, either primary or secondary to synovial chondromatosis, is rare. Ten cases of synovial chondrosarcoma were studied (four from the Mayo Clinic files and six from the authors' consultation files). Two were considered primary. In five cases there was evidence of preexisting synovial chondromatosis, and in the remaining three, there was a suggestion of preexisting disease. Several histologic features were found that were helpful to diagnose malignancy. The most important ones were loss of the "clustering" growth pattern typical of synovial chondromatosis, myxoid change in the matrix, areas of necrosis, and spindling at the periphery of chondroid lobules. Pulmonary metastasis developed in five of nine patients; three of these patients died.

Adult↗

Extraskeletal myxoid chondrosarcoma. A clinicopathologic study of ten patients with long-term follow-up.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma that has been reported to have a relatively good prognosis. The authors report ten patients with EMC on whom there was a minimum follow-up of 10 years. The patients' ages ranged from 31 to 72 years (mean, 57 years); there were six men and four women (seven white, three black). The tumor locations included the knee area and thigh (three patients each), the shoulder (two patients), and the leg and foot (one patient each). The tumors ranged from 3.5 to 18 cm in greatest dimension (median, 11.5 cm). All cases had typical histologic features. Lung metastases developed in all patients but one, and three patients had extrathoracic metastases (one in regional lymph nodes; one in subcutis; and one widespread). Four of the patients who had metastases also had local recurrence, as did the only patient without known metastases. Seven patients died of tumor at 4, 5, 8, 10, 15, 16, and 17 years, respectively, and the three remaining patients were alive with metastatic disease at latest follow-up of 13, 14, and 16 years. The authors' results are distinctly different from those previously reported in series with shorter follow-up times. The authors conclude that extraskeletal myxoid chondrosarcomas are indolent but resilient and capricious tumors with a high potential for metastasis, especially to the lungs, over the long-term.

Adult↗

Extraskeletal mesenchymal chondrosarcoma of the orbit.

Mesenchymal chondrosarcoma, an uncommon lesion of bone and extraskeletal tissue, is rare in the orbit. A case of extraskeletal mesenchymal chondrosarcoma of the orbit in a 28-year-old woman presenting with proptosis and diminution of vision in the left eye is reported. Histologically, the tumor showed areas of undifferentiated mesenchymal cells with islands of cartilage. Immunohistochemical analysis for S-100 protein showed focal positivity in chondroid areas. The eye was preserved and the patient has good vision after 2 years of follow-up.

Adult↗

Cytopathology of mesenchymal chondrosarcomas: a report and comparison of four patients.

BACKGROUND: Mesenchymal chondrosarcoma (MC) is an infrequent neoplasm, representing approximately 1% of all chondrosarcomas. Cytologic descriptions of MCs have been confined to rare case reports. In the current report, the authors describe their experience with the cytologic features of four MCs: two primary tumors and two metastatic lesions. METHODS: Four patients were diagnosed with MC at the authors' institution from 1994 to 2002. Three of four patients underwent fine-needle aspiration (FNA) biopsy as part of their diagnosis; in the fourth patient, imprint cytology was performed. Each tumor also received histologic confirmation. RESULTS: The patients studied included three females and one male. In three patients, the tumor presented initially as a soft tissue mass; whereas, in the remaining patient, the MC presented in the tibia. FNA results demonstrated small, oval-to-spindled cells with high nuclear-to-cytoplasmic ratios. Cells occurred singly and in clumps in a background of basophilic extracellular matrix. Histologic examination of each lesion demonstrated biphasic tumors, including focal areas of relatively mature cartilage formation as well as a small cell population. CONCLUSIONS: MC is a rare soft tissue tumor that occurs frequently in extraskeletal locations. FNA of these tumors can be diagnostic if the tumor is sampled appropriately and of critical features, such as the background extracellular matrix, are recognized. Given the propensity of these tumors to metastasize and the poor prognosis of patients with MC, early identification by FNA biopsy may allow earlier, more aggressive interventions.

Adolescent↗

Dedifferentiated central chondrosarcoma.

BACKGROUND: The prognosis for patients who develop dedifferentiation of central chondrosarcoma traditionally has been poor. Because not much has been reported about this rare lesion, many uncertainties remain about prognostic factors. METHODS: In this retrospective study, the clinical, radiographic, and histologic features and the treatments in 123 patients from the Rizzoli Institute were reviewed in an attempt to define which factors may be related to outcome in patients with dedifferentiated central chondrosarcoma. RESULTS: Among 123 patients who were included in this study, 109 patients were treated at the Rizzoli Institute, and 14 patients were seen in consultation. There were 66 males and 57 females, and their average age was 59 years. The femur (62 patients), pelvis (28 patients), and humerus (20 patients) were the most common locations. Radiographically, a soft tissue mass was present in 87% of patients, and a bimorphic pattern was appreciated in 53% of patients. Histologically, the cartilaginous component was considered Grade 1 in 63% of patients and Grade 2 in 37% of patients. In most patients, the dedifferentiated component showed the features of an osteosarcoma (92 patients), followed by fibrosarcoma (19 patients), and malignant fibrous histiocytoma (9 patients). For 101 patients, surgery was a component of their definitive management. In 25 patients, surgery was combined with chemotherapy. The 2-year and 5-year survival rates were 34% and 24%, respectively. The median survival was 13 months (95% confidence interval, 9-17 months). CONCLUSIONS: Metastatic disease at diagnosis, malignant fibrous histiocytoma dedifferentiation, and a high percentage of dedifferentiated component were related to poorer outcomes. There was no statistical evidence of any beneficial effect from adjuvant chemotherapy.

Adult↗

Diagnosis and grading of chondrosarcomas on FNA biopsy material.

A consecutive series of 47 chondrosarcomas was reviewed to identify the most useful criteria for cytological diagnosis, and grading in comparison to histology grade. This series includes 39 evaluable cases, including 2 patients with Ollier's disease and 2 with extraskeletal chondrosarcomas. Eight cases were excluded because of lack of histologic confirmation (3). nondiagnostic cytology (4). and one inconclusive histologic diagnosis. The cytologic diagnosis of these tumors was based on the identification of chondrocytes and a chondromyxoid stroma. The chondrocytes often showed double nuclei (34/39) and macronucleoli (20/39). Nuclear atypia in terms of distinct variation in size was less common (16/39). Cytoplasmic granules and vacuoles were often seen (34/39 and 29/39, respectively). In smears, chondroid fragments and a chondromyxoid background were seen in a majority of cases (36/39 and 30/39, respectively). By grade, concordance with histology was present in 4/7 grade I tumors, 26/29 grades II-III, and 5/5 grade IV, respectively.

Adolescent↗