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[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↗
PubMed1978

[Clear-celled chondrosarcoma or chondroblastic sarcoma. A new type of chondrosarcoma (author's transl)].

The authors report five cases treated by wide resection. Pathological studies were made of the whole of the lesion. Three tumours were situated at the upper end of the femur, one at the upper end of the humerus and one at the level of the tibial plateau. The specific features were localisation in an epiphysis and histological areas of clear cells whose cytoplasm contained glycogen. From the anatomical and radiological points of view, these tumours were like chondroblastomata but their progress was more like that of chondrosarcomata. The author concludes that they should be distinguished from both types of tumour.

Adult↗
PubMed2025

An Integrated Clinical Genomic and Transcriptomic Subgrouping of Central Chondrosarcoma.

Central conventional chondrosarcoma, a malignant cartilage-producing bone tumor, is the second most common bone sarcoma. Chondrosarcomas are histologically graded, which is so far the best predictor of survival. Early mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2 genes are frequent, leading to the production of the oncometabolite D-2-hydroxyglutarate, which affects DNA methylation, resulting in a preferred chondrogenic differentiation over osteogenic differentiation of mesenchymal stem cells, which are currently considered the precursor cells of chondrosarcoma. DNA methylation profiling has previously revealed distinct profiles between IDH-mutant and IDH-wild-type chondrosarcomas, but the presence of further DNA methylation subgroups indicates that classification based solely on IDH status is too simplistic. In this study, we aim to identify biological subgroups in a total of 116 chondrosarcomas by integrating clinical data, IDH mutation status, gene expression, and genome-wide loss of heterozygosity (LOH). Clinical associations were observed between several factors, including sex and histological grade, as well as tumor site and IDH mutation status. RNA sequencing and genome-wide LOH confirmed the distinction between IDH-wild-type and IDH-mutant chondrosarcomas, where the number of chromosome arms affected by LOH was significantly higher in IDH-wild-type tumors than in IDH-mutant tumors. However, no clear subgroups emerged within each IDH group. Further clustering on RNA expression of differentiation markers identified subgroups characterized by chondrogenic, osteogenic, resting chondrocyte, or dedifferentiated profiles. These different subgroups showed a specific clinical presentation and suggest different precursor cells. Instead of a simple dichotomy between IDH-mutant and IDH-wild-type, our integrated approach highlights interconnected clinical, genomic, and transcriptomic patterns that offer a more nuanced view of chondrosarcoma biology and might potentially guide treatment stratification.

Humans↗
PubMed1976

Effect of aromatic retinoids on rat chondrosarcoma glycosaminoglycan biosynthesis.

Synthetic aromatic analogs of retinoic acid were administered i.p. and p.o. to Fischer F344 rats bearing a transplantable chondrosarcoma. 35CO4 incorporation into glycosaminoglycans were compared for neoplastic and normal cartilage explants after removal from animals given various analogs. There was a direct relationship between [35S]glycosaminoglycan synthesis by chondrosarcoma chondrocytes and inhibition of tumor growth. The degree of inhibition of [35S]glycosaminoglycan synthesis in the neoplastic cartilage was dependent on the dose of the retinoid administered. At 20-mg/kg/day doses of retinoid for 4 weeks, 35SO4 incorporated into glycosaminoglycan by treated tumor explants was reduced as much as 95%. There was no reduction of [35S] glycosaminoglycan produced in normal costal cartilage of the same animals. Retinoid treatment of 20-mg/kg/day doses for 4 weeks resulted in a 75% reduction in glycosaminoglycan per mg of chondrosarcoma; there was no reduction in costal cartilage glycosaminoglycan. Retinoid (10- to 20-mg/kg/day doses) elevated collagen levels per mg of chondrosarcoma but had no effect on costal cartilage collagen. Combined in vitro and in vivo studies showed that retinoid administration modified neoplastic chondrocyte function but had no measurable effect on normal chondrocyte function.

Animals↗
PubMed1979

Chondrosarcoma of the first metatarsal--primary or secondary to enchondroma?

Enchondromas are said to be extremely rare precursors of secondary chondrosarcomas. Peripheral enchondromas of the hand and foot may be considered as benign, even when the histomorphological study reveals pleomorphic features with atypical nuclei. Our case deals with a 56 year old woman who had an enchondroma of the first metatarsal. Its distinct histopathological signs of focal malignant transformation were only seen in later review. Nine months after curettage and autologous bone-grafting a chondrosarcoma was diagnosed. The enbloc-resection of the medial foot provided adequate surgical treatment of the chondrosarcoma.

Bone Transplantation↗
PubMed1977

Juxtacortical chondrosarcoma.

The features are described of seven cases of "juxtacortical" chondrosarcoma, the term introduced by Jaffe for a rare but distinct entity and now accepted in the World Health Organisation classification as preferable to the term "periosteal" chondrosarcoma. In all cases the lesion involved the shaft of a long bone, most often the femur, and in two cases two different long bones were affected. Six of the seven patients were male and all were in the second decade. The characteristic appearance was that of a small tumour adjacent to the cortex with areas of spotty calcification often accompanied by radiating bone spicules perpendicular to the bone shaft and a typical Codman's triangle. Histologically all the tumours showed a cartilaginous lobular pattern, well limited on the surface and seldom infiltrating the cortex; areas of spotty calcification and enchondral ossification were often present but tumour osteoid and bone were conspicuously absent. Despite the ominous histological aspect, the prognosis proved to be relatively favourable compared with the usual central chondrosarcoma of a similar grade of malignancy.

Adolescent↗
PubMed1975

Isolation and characterization of proteoglycans from the swarm rat chondrosarcoma.

Proteoglycan monomer (D1) and aggregate (A1) preparations were isolated from 4 M guanidinium chloride extracts of the Swarm rat chondrosarcoma. When EDTA, 6-aminohexanoic acid, and benzamidine were present in the solutions, the D1 preparation contained a single component (SO = 23 S), and the A1 preparation contained 30% monomer (SO = 23 S) and 70 percent aggregate (SO = 111 S). In the absence of EDTA, 6-aminohexanoic acid, and benzamidine, the A1 preparations contained only small proteoglycan fragments, indicating that extensive enzymatic degradation had occurred. The composition of the proteoglycan monomer was different from that of proteoglycan monomer preparations from normal hyaline cartilages in that it did not contain keratan sulfate and chondroitin 6-sulfate; only chondroitin 4-sulfate was found. The A1 preparation from the chondrosarcoma contained only one link protein, which was like the smaller (molecular weight of 40,000) of the two link proteins present in A1 preparations from bovine nasal cartilage. When the A1 preparation from the chondrosarcoma was treated with chondroitinase ABC and trypsin and the digest was chromatographed on Sepharose 2B, a complex was isolated which contained the link protein and the segments of the protein core from the hyaluronic acid-binding region of the proteoglycan molecules.

Amino Acids↗
PubMed1977

[Chondrosarcoma of the upper jaw. Apropos of a clinical case].

Initially it has been a tendency to place chondrosarcoma in the general category of osteogenic sarcoma. Jaffe et Lichtenstein claimed that the concept of chondrosarcoma as a separate entity has a firm clinical and histological basis. Chondrosarcomas may arise in peripheral long and flat bones. The maxillary location is very rare, it represents about 0,7% of the whole body. 40 well documented instances are yet counted in the literature. Reporting a personnal case, the author proceeds to a comparative study between the three varieties of maxillary sarcomas, evokes the special histogenesis in this location, he reminds the interest of bone electrocoagulation as curative treatment, and discusses the difficulty in making a prognosis 3 years after surgery without local recurrence or metastasis in this case.

Adult↗
PubMed1977

Chondrosarcoma of the jaws and facial skeleton: a review of the Japanese literature.

From the 35 cases of chondrosarcoma, the maxilla was the most frequently affected site (35.5%) and the mandible was the next (29.4%). Painless swelling is the conspicuous symptom for chondrosarcoma of the lower jaw and nasal complications are often recorded with the lesions of the upper jaw. Several kinds of histologically malignant featurs coexisted which made diagnosis complicated. The three-year survival rate was about 50% for chondrosarcoma of the lower jaw and much lower for the upper jaw.

Adolescent↗
PubMed1978

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↗
PubMed1979

Chondrosarcoma of the jaws in Ibadan, Nigeria--a report of four cases.

Four cases of chondrosarcoma of the jaws seen in our clinics within a period of 15 years are described. In all, the lesions were initially benign-looking making early diagnosis difficult. The histogenesis of chondrosarcoma of the jaws is briefly discussed. In our environment, the prognosis of this disease is very poor.

Adult↗
PubMed1976

Chondrosarcoma of the esophagus.

A 46-year-old man had a large noncalcified tumor in the wall of the thoracic esophagus narrowing its lumen by projection of numerous firm nodular masses. The pathologic diagnosis was chondrosarcoma. Malignant degeneration of a tracheobronchial cartilaginous remnant is the main consideration in this first recorded case of esophageal chondrosarcoma.

Chondrosarcoma↗
PubMed1979

Hormone-dependent growth of a rat chondrosarcoma in vivo.

The importance of various hormonal factors in the growth of a transplantable chondrosarcoma has been studied in vivo. Tumor growth was reduced by 95% in adrenalectomized or hypophysectomized rats as compared to normal animals. The number of tumors developing in either adrenalectomized or hypophysectomized rats was reduced more in male than in female rats. However, ovariectomy or orchiectomy did not alter the growth of the tumor. The inhibition of tumor growth in adrenalectomized and hypophysectomized animals was only observed after the first 10 days following inoculation. Cortisone (4-pregnen-17 alpha,21-diol-3,11,20-trione) administration fully restored tumor growth in adrenalectomized animals while adrenocorticotropic hormone or growth hormone were only partially effective in supporting tumor growth in hypophysectomized animals. High-affinity glucocorticoid receptors (7 to 10S) were present in the cytosls prepared from the tumor cells and were found to be increased in tumors from adrenalectomized animals. These results indicate that the growth of this chondrosarcoma is strongly dependent upon endocrine factors of adrenal and pituitary origin.

Adrenal Glands↗
PubMed1978

Mandibular metastasis of an extraskeletal myxoid chondrosarcoma arising on the plantar surface of the foot: report of case.

This paper presented a discussion of the initial appearance and atypical clinical course, histopathology, treatment, and prognosis of a case of extraskeletal myxoid chondrosarcoma that originated on the plantar surface of the left foot and that metastasized to the mandible. The clinical behavior of this neoplasm, as reported, is distinctively less aggressive and therefore thought to have a better prognosis than chondrosarcoma of bone. Contrary to the biologic behavior reported in the literature, however, our patient had a less than six-year remission from the neoplasm.

Adult↗
PubMed1979

Chondrosarcoma of the maxilla: report of case.

Chondrosarcomas of the jaws are rare tumors; an additional case in the maxilla is described. The literature that discusses the pathogenesis of the tumor, its biological behavior, and the preferred treatment is reviewed. The histologic differentiation between chondrosarcoma and benign cartilagenous tumors is discussed.

Adult↗
PubMed1979

Combination of radiotherapy and razoxane (ICRF 159) for chondrosarcoma.

Eight patients with 12 chondrosarcomas were treated with radiation and razoxane (ICRF 159). Two tumors in 1 patient progressed unequivocally, 3 tumors in 3 patients showed no change, and 7 tumors in 5 patients had complete or partial (more than 50%) regressions. At least 2 complete regressions have responded for more than 2 1/2 years at the present time.

Adult↗
PubMed1977

Mesenchymal chondrosarcoma of the rectus sheath case report with ultrasonic findings.

The ultrasonic findings in a 54 year old white male with a rare tumor, mesenchymal chondrosarcoma, involving the rectus sheath are described. The sonogram confirmed the tumor's location in the anterior abdominal wall and provided useful information regarding its extent. An interesting feature was the lesion's relative sonolucency, apparently due to its myxoid content.

Abdominal Muscles↗
PubMed1979

Pseudo-chondrosarcoma: solitary osseous metastases from atypical bronchogenic carcinoma.

A patient with bronchogenic carcinoma with a solitary metastases to the femur in whom the clinical, radiologic, and pathologic setting suggested that the primary tumor was the bone lesion (chondrosarcoma) and the pulmonary lesion was a solitary metastasis is presented. This case is added to the limited literature indicating that mucin-secreting tumors metastatic to bone may simulate primary bone tumors radiographically.

Adenocarcinoma, Mucinous↗