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At least 19 recordsLinked to original sources
PubMed2001

Chromosome 9 alterations and trisomy 22 in central chondrosarcoma: a cytogenetic and DNA flow cytometric analysis of chondrosarcoma subtypes.

Chondrosarcomas are malignant cartilaginous tumors. Most are located in the medullar cavity (central chondrosarcoma), and a minority develop in a preexisting osteochondroma (peripheral chondrosarcoma). The authors present karyotypes for 37 central, peripheral, juxtacortical, and dedifferentiated chondrosarcomas. Using loss of heterozygosity (LOH) analysis and DNA flow cytometry, the authors previously showed that central and peripheral chondrosarcomas probably evolve by different genetic mechanisms. Peripheral chondrosarcoma is characterized by genetic instability, as was previously shown by a high percentage of LOH and a broad range in DNA ploidy. The authors now show that all peripheral chondrosarcomas tested are aneuploid, combined with many nonspecific chromosomal aberrations. Two juxtacortical chondrosarcomas showed normal chromosome numbers combined with limited structural alterations, substantiating that juxtacortical and peripheral chondrosarcomas are two clinicopathologically different entities with a different genetic background. Central chondrosarcomas were previously found to be peridiploid with limited LOH, most frequent at 9p21. In the current study, chromosome 9 was involved in five of seven central chondrosarcomas compared with only one of four peripheral chondrosarcomas. Three central tumors showed involvement of the 9pl2-22 region, suggesting an important role for chromosome 9 in the oncogenesis of central chondrosarcoma. Moreover, trisomy 22 was found in four central chondrosarcomas only.

Adolescent↗
PubMed1998

De-differentiated chondrosarcoma is not a 'de-differentiated' chondrosarcoma.

AIMS: De-differentiated chondrosarcoma is characterized by the presence of two distinct chondroid and nonchondroid tumour portions. The aim of our study was to investigate the distribution of extracellular matrix components in this tumour entity and thus to shed light on its histogenetic origin. METHODS AND RESULTS: Histochemical and immunohistochemical analyses were performed for collagen subtypes I, II, III and VI and cartilage proteoglycans in three samples of de-differentiated as well as conventional chondrosarcomas (various grades). In the chondroid tumour areas of de-differentiated chondrosarcoma, typical cartilage matrix components could be detected similar to chondroid areas of grade 1 and 2 conventional chondrosarcomas. In contrast, the tumour matrix of the nonchondroid portions of de-differentiated chondrosarcomas contained matrix molecules which are typical for fibroblastic tissue. This matrix composition was not identical with less differentiated (nonchondroid) areas of grades 2 and 3 conventional chondrosarcomas. CONCLUSIONS: Our results confirm the chondroid nature of the differentiated portion of de-differentiated chondrosarcoma and indicate a nonchondrocytic nature of the nonchondroid portion. De-differentiated chondrosarcoma should not be considered as a 'de'-differentiated chondrosarcoma (grade 4 neoplasm), but as a tumour entity showing two types of mesenchymal differentiation.

Adult↗
PubMed2000

Up-regulation of PTHrP and Bcl-2 expression characterizes the progression of osteochondroma towards peripheral chondrosarcoma and is a late event in central chondrosarcoma.

Chondrosarcomas are malignant cartilage-forming tumors arising centrally in bone (central chondrosarcoma) or within the cartilaginous cap of osteochondroma (peripheral chondrosarcoma). For hereditary multiple osteochondromas, two responsible genes, EXT1 and EXT2, have been cloned. Their recently elucidated role in heparan sulfate biosynthesis and Hedgehog diffusion leads to the hypothesis that EXT inactivation affects fibroblast growth factor (FGF) and Indian Hedgehog (IHh)/parathyroid hormone-related peptide (PTHrP) signaling, two important pathways in chondrocyte proliferation and differentiation. The expression of PTHrP, PTHrP-receptor, Bcl-2, FGF2, FGFR1, FGFR3, and p21 is investigated by immunohistochemistry in osteochondromas (n = 24) and peripheral (n = 29) and central (n = 20) chondrosarcomas. IHh/PTHrP and FGF signaling molecules are mostly absent in osteochondromas. Although no somatic EXT mutations were found in sporadic osteochondromas, the putative EXT downstream targets are affected similarly in sporadic and hereditary tumors. In chondrosarcomas, re-expression of FGF2, FGFR1, PTHrP, Bcl-2, and p21 is found. Expression levels increase with increasing histological grade. Up-regulation of PTHrP and Bcl-2 characterizes malignant transformation of osteochondroma because PTHrP and Bcl-2 expression is significantly higher in borderline and grade I peripheral chondrosarcomas compared with osteochondromas. In contrast, up-regulation of PTHrP and Bcl-2 seems to be a late event in central cartilaginous tumorigenesis because expression is mainly restricted to high-grade central tumors.

Adolescent↗
PubMed1992

Laryngeal chondrosarcomas: a clinicopathologic study of 11 cases, including two "dedifferentiated" chondrosarcomas.

Laryngeal chondrosarcomas are uncommon, and those that contain a distinct, nonchondroid, high-grade spindle cell sarcoma (the so-called "dedifferentiated" chondrosarcoma or chondrosarcoma with additional malignant mesenchymal component [CAMMC]) are extremely rare. Laryngeal CAMMC merit special attention, as CAMMC in other sites portends a poor prognosis. Eleven patients with laryngeal chondrosarcomas are reported on; 2 of these patients had CAMMC. On follow-up, 3 of the 11 patients had recurrences. The first had recurrence 4 and 11 years after tumor enucleation; that patient died disease free 2 years after salvage total laryngectomy. The second had recurrence 2 years after partial laryngectomy and was lost to follow-up after salvage total laryngectomy. The last patient recurred 13 years after partial laryngectomy and underwent salvage total laryngectomy; that patient was one of the two who developed CAMMC, and he also developed stomal recurrence of the "dedifferentiated" component 3 years after total laryngectomy. The other 8 patients are disease free after partial laryngectomy (6) or total laryngectomy (2) 10 months to 12 years later (mean: 51 months). This includes the 1 other patient with CAMMC, who is disease free 60 months after total laryngectomy. Laryngeal CAMMC has been shown, in at least one of the two patients, to be associated with a poor outcome. Patients with recurrent laryngeal chondrosarcomas do not have a poorer outcome after salvage total laryngectomy. The authors advocate partial laryngectomy if technically feasible.

Adult↗
PubMedDate not supplied

[A new variety of chondrosarcoma : so-called "chondro-blastic" sarcomas or "clear cell" chondrosarcomas. Pathology and electron microscopy in 5 cases (author's transl)].

The authors report the pathological and clinical findings in five cases of chondrosarcoma resembling chondroblastic chondrosarcomas as described by Lichtenstein and Bernstein in 1959 and, above all, clear cell chondrosarcomas as described by Unni et al. (1976). These chondrosarcomas have a double peculiarity:--topographic, for their site is often epiphyseal :--anatomica, for they associate, in the usual chondrosarcomatous sectors, areas rich in clear cells, with a rich blood supply, and in some cases a diffuse reactional osteogenesis. Although the long term prognosis is similar to that of all chondrosarcomas, their course is sometimes very slow. One should thus distinguish carefully these tumours from chondroblastomas in spite of certain topographic and radiological similarities.

Adult↗
PubMed1996

Juxtacortical dedifferentiated chondrosarcoma from a primary periosteal chondrosarcoma.

In this report, we describe dedifferentiation in a primary periosteal chondrosarcoma in the proximal tibia of a 73-year-old man. The diagnosis of primary periosteal chondrosarcoma was made by the characteristic radiographic, gross, and microscopic features of the tumor. The presence of dedifferentiation, however, in the form of malignant fibrous histiocytoma, was an unexpected histologic finding. Although rare, dedifferentiation is a well-recognized occurrence in secondary chondrosarcomas arising from precursor osteochondromas. This event has not been previously documented in the setting of primary periosteal chondrosarcoma.

Aged↗
PubMed1999

Is dedifferentiated chondrosarcoma a 'de-differentiated' chondrosarcoma?

Since its first description 30 years ago, dedifferentiated chondrosarcoma has been the prototype of all dedifferentiated sarcomas. The presence of two tumour portions of different mesenchymal differentiation lineages in these neoplasms gives rise to three key questions, which are on the way to being resolved. Does it split up? And if so, how does it split up and when does it split up? Accumulating data provide evidence for a common monoclonal origin of both tumour portions and suggest that dedifferentiated chondrosarcoma is a paradigmatic neoplasm of mesenchymal transdifferentiaton in vivo. Two categories emerge of dedifferentiated chondrosarcomas with different cell biology: the classical one, with a low-grade chondroid component splitting up late, and a second type, with a high-grade chondroid component splitting up early in tumour development.

Bone Neoplasms↗
PubMed1986

Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). II. An immunohistochemical and electron microscopic study.

Light microscopic, immunocytochemical and ultrastructural studies were performed on chondrosarcomas which contained a second, noncartilagenous mesenchymal component. Attention was focused on the nonchondroid portion of each tumor in an attempt to elucidate the histogenesis of this mixed variant of chondrosarcoma. The immunoreactivity of 20 tumors was studied using antisera for S-100 protein, alpha-1-antitrypsin, alpha-1-antichymotrypsin, smooth muscle myosin, desmin, and myoglobin. Cells of the nonchondroid portion stained for alpha-1-antichymotrypsin in 12 of 20 cases, and these were predominantly tumors that had been classified as fibrosarcoma or malignant fibrous histiocytoma by conventional light microscopic study. Staining for S-100 protein was consistently negative, whereas the chondrosarcoma component stained in 14 cases. Six tumors stained for desmin, and four of the six were positive for myoglobin and two for smooth muscle myosin; in four, a rhabdomyosarcomatous component was identified in the hematoxylin and eosin-stained sections. Electron microscopic study was performed on ten tumors and there was a good correlation between the immunohistochemical and ultrastructural findings. Three of the ten were pure rhabdomyosarcomas while the others displayed a range of ultrastructural appearances that can be seen in fibrosarcomas and malignant fibrous histiocytomas. The findings from this study support the view that the tumors are formed by the synchronous differentiation of two separate clones of cells.

Adult↗
PubMed1997

Possible monocytic origin of chondrosarcoma: in vitro transdifferentiation of HLA-DR blood monocyte-like cells from a patient with chondrosarcoma, into neo-fibroblasts and chondrocyte-like cells.

Nodules and multilayered areas composed of fibroblasts and chondrocyte-like cells embedded in an abundant extracellular matrix appeared spontaneously in in vitro culture of mononucleated blood cells taken from a patient with chondrosarcoma. Using specific antibodies it was demonstrated that the neo-fibroblasts which developed in the culture resulted from a direct transdifferentiation of monocytes expressing HLA-DR specificity. The experiment was carried out twice, once before surgery and then two years later. In both cases the spontaneous transdifferentiation of HLA-DR monocytes into neo-fibroblasts was observed. Previously it was shown that normal monocytes were also able to give rise in vitro to neo-fibroblasts. However, the latter are normally rapidly destroyed by cell-cell contact with T-cells. Normal T-cells adhere to normal neo-fibroblasts by which they are finally engulfed. As a result, the neo-fibroblasts lose their fibroblastic shape, no longer adhere to their support and die. Therefore the abnormal proliferation and persistence of neo-fibroblasts in pathological situations such as the present case may result either from an intrinsic defect in monocytes, T-cells or both. The question is whether or not this transdifferentiation process observed in vitro accounts for the development of chondrosarcoma in vivo. The present results suggest that in vivo chondrosarcoma may start in a necrotic zone (resulting for instance from trauma) and attract HLA-DR monocytes, where they accumulate and transdifferentiate into neo-fibroblasts and chondrocyte-like cells. The uncontrolled transdifferentiation of these HLA-DR monocytes resulting from a dysregulation of the immune system is probably linked to the malignant process which may have a retroviral origin. The question is raised regarding the embryologic origin of this special sub-population of blood monocytes in which pluripotential capabilities are retained; its origin may differ from that of the other circulating monocytes.

Cartilage↗
PubMed1988

Chondrosarcoma. II. Chondrosarcoma of the jaws: analysis of 37 cases.

Analysis was made of 37 cases of chondrosarcoma of the maxilla or the mandible. The most common complaint at initial presentation was the presence of a mass. Pain was a presenting symptom in less than half of the patients. The usual roentgenographic appearance was an ill-defined radiolucency with mottled areas of calcification. When teeth were involved, some cases showed a widened periodontal membrane space on periapical dental radiographs. Histologically, the tumors usually showed recognizable cartilage and a lobular growth pattern, but cellular evidence of malignancy was often subtle. Radical surgery was the treatment of choice. Prognosis appears best when such surgery is performed soon after the onset of symptoms. Also, patients presenting with mandibular tumors and with tumors of the better differentiated histologic grades enjoy a better survival time. Neck dissection is probably not warranted for chondrosarcoma of the jaws. Contrary to the relationship between these two tumors in other skeletal sites, the overall prognosis for chondrosarcoma of the jaws does not appear to be as good as that for osteosarcoma of the jaws.

Adolescent↗
PubMed1987

[A rare variant of chondrosarcoma--mesenchymal chondrosarcoma of the scapula].

A rarely occurring tumor, mesenchymal chondrosarcoma of the shoulder blade, is described. The tumor, found in a 19-year-old male, consisted of two components; a typical chondrosarcoma showing a moderate degree of anaplasia, and a tumor tissue composed of poorly differentiated cells with large numbers of thin-walled vessels. The two tissues were separated from each other by connective-tissue elements, though cells of the second component were seen in places to pass into the tissue of hyalin cartilage. This variant of chondrosarcoma proved to be highly sensitive to chemotherapy, allowing considerable prolongation of the patient's survival.

Adult↗
PubMed1986

Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathologic study of 26 cases.

During a 37-year period, 26 patients were seen who had chondrosarcoma with additional mesenchymal components ("dedifferentiated low-grade chondrosarcoma"). Sixteen were men and 10 were women aged 30 to 85 years (median, 61 years). The tumors' chondroid areas were of borderline or low-grade malignancy. The additional mesenchymal component was histologically classified as malignant fibrous histiocytoma (16), rhabdomyosarcoma (4), low-grade fibrosarcoma (3), osteosarcoma (2), and undifferentiated sarcoma (1). Preferred locations were pelvis (10) and femur (8). Symptoms had been present for 1 year or less in most cases. Pain was the most common symptom. In 15 of 26, major amputation was the primary treatment. Twelve patients received chemotherapy, usually after developing metastatic disease, but only one achieved a partial response. Median disease-free interval after diagnosis was 4 months, median survival was 6 months, and 19 patients died within 1 year. Of 4 who survived longer than 18 months, 3 presented with a low-grade fibrosarcoma. Survival and development of metastasis appeared unrelated to cell type, initial treatment, or chemotherapy, except when the tumor's initial nonchondroid component was low-grade fibrosarcoma.

Adult↗
PubMed1982

Ultrastructural study of conventional chondrosarcomas and myxoid- and mesenchymal-chondrosarcomas.

Five cases of conventional chondrosarcomas (CS.) of graded malignancy, 3 cases of myxoid CS. and 2 cases of mesenchymal CS. were studied by electron-microscopy. The chondrocyte like tumor cells of conventional CS. were characterized by: an ovoid shape, eccentric nucleus, abundant endoplasmic reticulum with dilated cisternae of RER; cytoplasmic glycogen, lipid droplets, and filaments plus numerous thin cytoplasmic projections. The histologically high grade tumors showed fewer cytoplasmic organelles, bizarre nuclei and more prominent nucleoli than the better differentiated ones. The tumor cells of myxoid CS. were chiefly fusiform. The cells frequently presented a pattern of rows with good cellular cohesion, and scanty cytoplasmic projections. The most prominent cytoplasmic feature was a conspicuous RER. Abundant cytoplasmic filaments and cytoplasmic glycogen were also observed. The undifferentiated areas of the mesenchymal CS. showed primitive mesenchymal cells with rounded nuclei, and scanty cytoplasm which was poor in organelles and glycogen. The cytoplasmic membranes were very cohesive and cytoplasmic projections were not present. Scanty cytoplasmic filaments and conspicuous desmosome like junctions were observed. The intercellular matrix of conventional and myxoid CS. consisted of fibrils, glycosaminoglycan granules and collagen fibers. In the undifferentiated zones of the mesenchymal CS. the intercellular matrix was very scanty and did not contain collagen fibrils. The more immature cells correspond to the small undifferentiated cells of mesenchymal chondrosarcoma.

Adult↗
PubMed1988

Chondrosarcoma of the larynx with additional malignant mesenchymal component (dedifferentiated chondrosarcoma).

Chondrosarcoma of the larynx is a rare tumor that usually is low grade both in histology and biologic aggressiveness; local ablative surgery is curative in most cases. We present a case of this tumor with additional malignant mesenchymal component, so-called dedifferentiated chondrosarcoma--an entity which, when found in other areas, has carried a much poorer prognosis.

Cell Differentiation↗
PubMed1986

Chondrosarcoma of the ethmoidal and sphenoidal sinuses. A case of chondrosarcoma arising from postparanasal sinuses.

A case of chondrosarcoma originating within the postethmoidal and sphenoidal sinuses with intracranial invasion will be reported. The patient, a 54-year-old female, presented severe headaches and diminished visual acuity of the right eye. Angiographic findings showed both the left carotid artery to be completely occluded and the right inner carotid artery to be narrowed at the site of the carotid bifurcation. Computerized tomography scan findings showed a tumor, located mainly in the frontal base and temporal fossa, that had invaded the cranium through the skull base. Several tumor excisions were performed using a combined extranasal and transmaxillary approach. The diagnosis 'chondroma' was changed to 'chondrosarcoma' during autopsy.

Brain Neoplasms↗