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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↗
PubMed1977

Chondrosarcoma of spermatic cord.

A case of extraosseous chondrosarcoma arising from the spermatic cord is reported and the literature reviewed. Chondrosarcoma arising from nonosseous tissue is rare, and only a handful of cases have been reported. However, unlike chondrosarcoma of bone, extraosseous myxoid chondrosarcoma behaves in a less aggressive fashion making the distinction between the two entities prognostically significant.

Aged↗
PubMed1979

Chondrosarcoma of the larynx.

A chondrosarcoma arising in the posterior cricoid plate is presented. The tumour gave rise to increasing inspiratory stridor: laryngectomy was performed. The tumour consisted of loose cartilaginous tissue with great predominance of highly differentiated cartilage cells and only small areas with nuclear polymorphism. This complicated the differential diagnosis between chondroma and highly differentiated chondrosarcoma. From the fact that the patient died from massive pulmonary metastases 3 1/2 years later, it is evident that the degree of malignancy of cartilaginous tumours should be determined even on small polymorphic foci. The DNA histogram for the foci of the laryngeal tumour with atypia differed distinctly from those for benign chondroma and normal cartilage, but resembled those of the pulmonary and splenic metastases and of a nasal chondrosarcoma. Photometric examination may provide an aid in the difficult differential diagnosis between chondroma and highly differentiated chondrosarcoma.

Chondrosarcoma↗
PubMed1979

A review of the behaviour of chondrosarcoma of bone.

Sixty-two cases of chondrosarcoma of bone were reviewed and histologically graded as low, medium or high-grade tumours. After excluding patients dead from unrelated causes or lost to follow-up, forty cases were available for ten-year follow-up and fifty-eight for five-year follow-up. The rates of survival, recurrence and metastasis were analysed according to the histological grading. Recurrence was further analysed according to the adequacy of treatment. The results were compared with those previously reported in the literature. There was a ten-year survival rate of 58 per cent. Recurrence developed in 58 per cent and was uncontrollable in 29 per cent. The recurrence rate was 87 per cent with inadequate treatment and 15 per cent with adequate treatment. Recurrences outside the limb bones usually proved uncontrollable; recurrences in the limb bones were amenable to further, and if necessary repeated, operations. High-grade chondrosarcoma had a metastatic risk of 75 per cent and eventual mortality of 88 per cent. Medium-grade chondrosarcoma had a metastatic risk of 14 per cent and a mortality of 60 per cent. Low-grade chondrosarcoma had a metastatic risk of 5 per cent and a mortality of 29 per cent.

Adult↗
PubMed1979

Laryngeal chondrosarcoma in Sweden.

Between 1958 and 1972, 5 cases of posterior cricoid chondrosarcoma were reported to the Swedish Cancer Registry. These cases, and one recently observed case of laryngeal chondrosarcoma, are presented in this study of one female and five male patients. The tumours grow slowly and recur locally, but have not metastasized, and none of the patients has died from tumour disease. The clinical and morphological findings indicate that these tumours represent secondary chondrosarcomas developing from pre-existing benign ecchondromas. The reason for the uniformity as to localization is not known. The microscopic appearance varied in different parts of the same tumour. The presently obtained data and review of the literature indicate that laryngeal chondrosarcomas are extremely rare, locally invasive, but usually not metastasizing tumours. Conservative laryngeal operation is suggested as primary treatment. Total laryngectomy is necessary only after local recurrences involving a major portion of the cricoid cartilage.

Aged↗
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

Clear-cell chondrosarcoma: a report of five cases including ultrastructural study.

Five cases of clear-cell variant of chondrosarcoma (Unni et al.) are reported. The tumors occurred in the epiphyseal region of long bones; three in the femoral head. Roentgenographically, the lesion was usually a well-defined and benign appearing one, either purely lytic (3 cases) or with central radiodensity (2 cases). Histologically, all five cases had areas of conventional chondrosarcoma; however, the greater portion of the tumor was made up of sheets of clear-cells intermixed with nonneoplastic bone trabeculae but devoid of chondroid matrix. Electron microscopic studies showed that these clear-cells possess cytoplasmic microvilli, abundant glycogen particles and prominent golgi complexes, like normal or tumorous chondroid cells usually have. In our experience, the best treatment seemed to be en bloc resection with joint replacement; indeed, despite the fact that they are true chondrosarcomas, these tumors usually have a very slow rate of growth.

Adolescent↗
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↗
PubMed1979

Mesenchymal chondrosarcomas of the head and neck.

Mesenchymal chondrosarcoma, in both osseous and extra-skeletal forms, has a decided predilection for the head and neck region. The two cases presented in this report affirm this tendency and also illustrate the capricious biologic behavior of the neoplasms as manifested by the 30 additional cases recorded in the literature. Histopathologic confusion with hemangiopericytoma is avoided by identification of the cartilage component in the mesenchymal chondrosarcoma and by the undifferentiated nature of the cellular (non-cartilaginous) component.

Adolescent↗
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↗