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PubMed2003

[Dedifferentiated chondrosarcoma of bone--report of 5 cases and literature review with emphasis on histogenesis].

Five cases of dedifferentiated chondrosarcoma of bone are presented. Three women and two men of age ranging from 57-72 years (median 63 years) suffered from tumors located in femur (2), scapula (1), humerus (1) and pelvis (1). They were all bulky tumors destroying the bones and infiltrating into the adjacent soft tissues in maximum diameter from 5-20 cm (median 12 cm). Both the short clinical histories lasting from 2-24 months (median 5 months) and persistent pains unrelieved by acylpirin were constant findings in all the cases. Four patients died of metastatic disease (mainly to lungs) with median survival of 8 months. All cases were the so-called "de novo" dedifferentiated chondrosarcomas without clinical or morphological evidence of the primarily benign or malignant chondroid lesions. The tumors displayed a typical dual composition with an abrupt interface between chondroid and nonchondroid components. The chondroid tissue resembled G1 chondrosarcoma in three cases, G2-3 chondrosarcoma in two cases. The second nonchondroid mesenchymal portion had the appearance of undifferentiated pleomorphic sarcoma in three cases, rhabdoid and giant cell tumor-like in one case. The new data and evidence provided by molecular biology dramatically changed the opinions of histogenesis of this unique neoplasm. At present, there is a generally accepted idea of common origin of both portions from a primitive stem cell. Therefore, this peculiar tumors should not be viewed merely as dedifferentiated conventional chondrosarcomas.

Aged↗

PubMed2003

Primary intraspinal mesenchymal chondrosarcoma: a case report and literature review.

Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas, especially those that arise in the central nervous system, are even rarer. Most of those described were intracranially located, with only a very few cases having been reported in an intraspinal region. Therapeutic experience with primary spinal mesenchymal chondrosarcomas is also extremely limited. We report on a case of a 21-year-old man with back pain and bilateral progressive weakness and numbness of the lower extremities. A T1-weighted magnetic resonance image revealed a hypointense tumor located at the T8 level. The tumor was completely excised through a posterior approach. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. Spinal irradiation and chemotherapy were also administered for prevention of local recurrence and metastasis. The patient has been symptom-free for 1 year after surgery. Herein, we review the 22 cases of primary intraspinal mesenchymal chondrosarcomas in the literature and discuss their clinical presentations, pathology, imaging studies, treatments, and outcomes.

Adult↗

PubMed2003

The expression of selected immunocytochemical diagnostic markers in the case of chondrosarcoma with a mesenchymal component.

The case of 52-year-old man is presented, who had suffered from pains in his right brachial region and in whom, upon admission to the Lower Silesia Centre of Oncology, a tumour of 20 cm in diameter and restricted mobility was disclosed in the right brachial region and proximal 1/3 of his right arm. Radiograms of his right humerus disclosed non-uniform restructuring of the osseous tissue, dominated by osteosclerosis in the upper half of his right humerus, while in the surrounding dift tissues of the proximal portion non-uniform shades were seen of calcified appearance. The patient was qualified to surgical biopsy and histopathological examination of the sample disclosed Chondrosarcoma G II. Following amputation of the right upper extremity together with the scapula, the tumour was subjected to histopathological and immunocytochemical examination in the Department of Pathomorphology, Lower Silesia Centre of Oncology. In numerous samples of the tumour dedifferentiated chondrosarcoma was diagnosed with a dominating component of malignant fibrous histiocytoma (MFH), which was confirmed by detecting a high expression of alpha-1-antichymotrypsin (ACT) within the spindle-shaped cell component of the chondrosarcoma. Spindle-shaped elements in dedifferentiated chondrosarcoma (DChSa) may represent patterns of fibrosarcoma, osteosarcoma or the malignant fibrohistiocytoma (MFH) type and they are present as if in the form of restricted fields with no reciprocal infiltrates. Moreover, due to the worse prognosis in the case of the MFH component in dedifferentiated chondrosarcoman as compared to that in classical chondrosarcoma, common evaluation of the material was made by a surgeon, radiologist and histopathologist, accompanied by a minimum panel of immunocytochemical tests (ACT, Vimentyna, Desmina, S-100). This enabled a final diagnosis for all tumour components to be established and prognosis to be made regarding the further fate of the patient.

Biomarkers, Tumor↗

PubMed1992

Histological investigation for comparison of cartilaginous tumors of unknown biological course with unequivocal chondrosarcomas.

Morphological findings recorded from chondromatous tumors of unknown biological behaviour (n = 40) were compared with those obtained from unequivocal chondrosarcomas (n = 27), and the histological results were evaluated by clinical outcome. The morphological details were analysed by histological set up and summarized by Mirra et al. (1985): Two benign patterns, the enchondroma encasement pattern and the island of cartilage patterns, were the most common findings in cartilaginous tumors of unknown biological behaviour. Malignant patterns, such as chondrosarcoma permeation, invasion of the Haversian system, soft tissue mass and bands of fibrosis patterns were detected only in unequivocal chondrosarcomas. However, the enchondroma encasement pattern could be focally demonstrated also in central chondrosarcomas (n = 3), a phenomenon, which in our opinion could not be interpreted as being indicative of a preexisting benign lesion. Morphological classification of chondromatous tumors of unknown biological behaviour, as a group of benign cartilaginous tumors, enables their discrimination from unequivocal chondrosarcomas. This was also confirmed by the significant difference (p = 0.0065; Mantel-Cox) among survival curves after Kaplan and Meier (1958). We conclude that the above criteria, when applied with due caution, are helpful in classifying chondromatous tumors.

Bone Neoplasms↗

PubMed2004

Heterogeneity in growth properties of the rat Swarm chondrosarcoma.

Chondrosarcoma remains one of the most difficult clinical conundrums of orthopaedic pathology, with wide variation in clinical course. The natural history of chondrosarcoma ranges from slow indolent growth without metastasis over years to rapid proliferation and lethal metastasis. Molecular regulatory events in the growth of these neoplasms are poorly understood. Of the Swarm rat chondrosarcoma, originating from a single neoplasm in a Sprague-Dawley rat more than thirty-five years ago, two populations were identified with different growth properties. These two Swarm chondrosarcoma lines were characterized for growth properties, histomorphometric and ultrastructural integrity, and the ability for proteoglycans to form aggregates with hyaluronan. After careful comparison, no obvious clues to the variation in growth rate were noted. Further molecular analyses may lead to better understanding of the differential growth properties of these cell lines. Understanding the mechanisms involved in differential growth rates may lead to clinically applicable clues to predict clinical behavior of chondrosarcomas in humans.

Animals↗

PubMed2005

Central nervous system mesenchymal chondrosarcoma.

Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival.

Adolescent↗

PubMedDate not supplied

Combined radiotherapy and razoxane in the treatment of chondrosarcomas and chordomas.

BACKGROUND: Chondrosarcomas and chordomas are reported to have low radio-sensitivity. Therefore, a study was undertaken to explore the radioresponsiveness of these tumours using the sensitising agent razoxane. PATIENTS AND METHODS: Thirteen chondrosarcomas and five chordomas were irradiated with high-energy photons and razoxane in the period from 1984 to 2003. The median tumour dose was 60 Gy in the chondrosarcomas and 63 Gy in chordomas. Razoxane tablets were given at a dose of 125 mg twice daily starting 5 days before the first irradiation. The drug was continued on radiation days. RESULTS: Eight out of the 13 chondrosarcomas had unresectable or recurrent measurable disease. There were one complete and five partial responses, while two tumours remained unchanged (response rate 75%). The median duration of response was 22 months. Three out of four patients without clear surgical margins and one patient with clear margins had locally controlled disease. Overall, local control was achieved in seven out of twelve patients who were not radically resected. All five patients with chordomas survived 5 years and remained locally controlled at that time. Among four measurable tumours, two complete and one partial regression were noted. Razoxane was well tolerated; the dose limiting toxicity was leukopenia. CONCLUSION: Photon irradiation together with razoxane induces major responses in a majority of patients with chondrosarcomas and chordomas. This combination therapy seems to be more effective than photon irradiation alone.

Adolescent↗

PubMed1991

Clear cell chondrosarcoma--a report of 2 cases.

Clear cell chondrosarcoma is a form of chondrosarcoma that was first described by Unni in 1976. It is essentially a low-grade chondrosarcoma with many similar clinical, radiological and histological features as the more common chondrosarcomas. However, the distinguishing feature of this variant is in the presence of the characteristic clear cells histologically. We relate two such cases of clear cell chondrosarcoma in Singapore. A short discussion covering the incidence/prevalence of this tumour, its common presenting clinical, radiological and histological features follows. We also review the available world literature, highlight the salient and differentiating aspects of this particular tumour, and touch on its management and eventual prognosis.

Adult↗

PubMed1990

Neutron boost irradiation of soft tissue and chondrosarcomas at the West German Tumour Centre in Essen.

In this retrospective study, the results of postoperative neutron boost therapy for 40 soft tissue- and 18 chondrosarcomas are presented. In 65.5% and 34.5% of all cases, microscopic to macroscopic tumour residual was left postoperatively. Locally advanced and moderate to highly malignant tumours were found at surgery in 81% and 69%, respectively. Except for seven patients with a whole series of neutrons to 16 Gy, the other cases were treated with initially 40 to 50 Gy photons and a subsequent neutron boost of 4 to 6 Gy. The median follow-up was 29.3 and 36.2 months for the soft tissue- and chondrosarcomas. The actuarial three-year local control and overall survival rates were 69.3% and 91% for soft tissue- vs. 55.6% and 62.9% for chondrosarcomas. With regard to the extension of surgery (microscopic or macroscopic residual), local control rates of 80% and 60% for soft tissue- vs. 62.5% and 40% for chondrosarcomas were observed. The overall local failure rate was 32.8% with a median disease-free interval of ten respectively eleven months for soft tissue and chondrosarcomas. 17 out of 19 local recurrences were due to inadequate irradiation portals for the neutron boost or to insufficient total doses in the initially treated photon target volume.

Actuarial Analysis↗

PubMed1988

A reassessment of "chordoid sarcoma". Ultrastructural and immunohistochemical comparison with chordoma and skeletal myxoid chondrosarcoma.

The relationship of "chordoid sarcoma" (CS) to chordoma and myxoid chondrosarcoma has been debated for several years. In order to reassess this issue, we studied 5 CS, 5 chordomas, and 3 skeletal myxoid chondrosarcomas ultrastructurally and immunohistochemically. By electron microscopy, CS demonstrated smooth cellular outlines, macular intercellular junctions, and cytoplasmic inclusions of matrix-like material. Chordomas displayed a closely similar fine structural appearance, but in addition contained small, membrane-bound, glycogen-containing inclusions. Skeletal myxoid chondrosarcomas resembled CS, except that the former lesions had spiculated cell membranes and lacked intercellular junctions. Immunohistochemically, all CS cases expressed vimentin and lacked cytokeratin (CK). Leu 7 and S100 protein were seen in four cases each of CS, and three of these tumors demonstrated diffuse or focal reactivity for epithelial membrane antigen (EMA). Similar phenotypic features were seen in chordomas, except that all of them stained diffusely for CK, as well as EMA. Skeletal myxoid chondrosarcomas expressed vimentin, S100, and Leu 7 uniformly, but were devoid of epithelial markers. In aggregate, these data support the classification of "chordoid sarcoma" as a form of chondrosarcoma, but reveal that it may exhibit an "epithelial" antigen in some cases.

Adult↗

PubMed1986

Dedifferentiated chondrosarcoma. A report of the clinicopathological features and treatment of seventy-eight cases.

Dedifferentiated chondrosarcoma is a highly malignant variant of chondrosarcoma. Approximately 11 per cent of chondrosarcomas can be expected to dedifferentiate into more anaplastic lesions. In this report, we analyze the clinicopathological features and treatment of seventy-eight lesions of this type. The ages of the patients ranged from nineteen to eighty-two years (average, 54.6 years). The cartilaginous precursor was central in most patients. Eleven of the lesions developed in the site of a previously resected low-grade chondrosarcoma. Dedifferentiation was from low-grade chondrosarcoma to osteosarcoma in forty-two patients, to fibrosarcoma in thirty-three, and to malignant (fibrous) histiocytoma in three. Perforation of the cortex and a soft-tissue mass were found in most of the patients. Widespread metastatic disease within two years after resection was a frequent finding. The over-all five-year-survival rate was 10.5 per cent. Any potential for a "cure" is related to early diagnosis and adequate surgical treatment by amputation or resection.

Adult↗

PubMed1987

[Secondary chondrosarcoma].

A retrospective evaluation of 101 cases of secondary chondrosarcoma was performed. The results of the analysis of X-ray data showed chondrosarcoma to develop from osteocartilaginous exostosis or chondroma (53 and 47 cases, respectively). (One case lacked roentgenologic data). The former pattern of tumor development prevailed in patients under 40 years of age, while the latter one was more frequently observed in the older age group (68% of cases in each age bracket). Secondary chondrosarcoma was most frequently localized to long tubular and flat bones (48 and 34 patients, 47.53 and 33.66%, respectively). Roentgenologic diagnosis of early stage secondary chondrosarcoma was extremely difficult. Histologic examination showed degree I cell anaplasia in most cases. Surgery is the main procedure of treatment of secondary chondrosarcoma. Relapses were observed only in patients who had undergone sparing operations. The smaller the extent of surgery, the higher relapse rate was registered. Fifteen patients (14.87%) died of tumor progression. Sparing procedures were performed in 14 of them (93.3%).

Adolescent↗

PubMed1986

Chondrosarcoma.

Chondrosarcoma is a malignant cartilage-forming tumor that affects predominently the middle-aged population. There are three histologic grades, but differentiation of Grade I tumors from enchondromas can be difficult. The measurement of polypoidy (augmented chromosomal content per cell) appears to offer a method of segregation of benign from low-grade from high-grade tumors. With the exception of the high-grade spindle elements, current chemotherapy does not appear to affect chondrosarcomas. Retinoic acid, however, by enhancing lysosomal enzyme release, may be a potential efficacious therapeutic agent. Enhanced tumor staging as provided by the computed tomographic (CT) scan has demonstrated the boundaries for wide surgical excisions, most notably internal hemipelvectomies for low- and moderate-grade pelvic chondrosarcomas. Currently, low-grade chondrosarcomas have an overall five-year survival of 65%--85% in contradistinction to 15% for the highest grade chondrosarcomas.

Adult↗

PubMed1984

[Clinico-morphological characteristics of bone chondrosarcomas].

One hundred and sixty-two cases of bone chondrosarcoma (86 males and 76 females) were studied. Course development of secondary neoplasms was more favorable than that of primary ones. Tumor size was found to be in correlation with 3-year survival only. No significant correlation was established between tumor size, on the one hand, and 5- and 10-year survival rates, on the other. Histologic type of chondrosarcoma proved to be of the highest prognostic value. The tumor followed 5 patterns: chondrosarcoma with mild (I), moderate (II), and severe (III) anaplasia, mesenchymal chondrosarcoma and the so-called "dedifferentiated" chondrosarcoma.

Adolescent↗

PubMed1984

[Morphological characteristics of mesenchymal chondrosarcoma].

Complex morphological examination of 11 cases of mesenchymal chondrosarcoma is performed. This tumour has sufficiently characteristic clinical and morphological features distinguishing it from a usual chondrosarcoma. Clinically, mesenchymal chondrosarcoma is characterised by a shorter anamnesis of the disease, a rapid course and shorter life-span. It occurs more frequently during the 2nd and 3rd decades of life. Histologically, apart from the structures characteristic of typical chondrosarcoma, poorly differentiated areas resembling hemangiopericytoma occur and sometimes predominate. Electron microscopic examination of the differentiated parts of mesenchymal chondrosarcoma revealed the presence of cells with chondroid direction of the structurally-functional differentiation while in the areas with pericytal structures there were found structurally and functionally undifferentiated cells.

Adolescent↗

PubMed1982

Chondrosarcoma in children and adolescents.

Chondrosarcoma is rare in children. Only 12 patients with a diagnosis of chondrosarcoma were treated at Children's Hospital Medical Center in Boston during the period from 1957 to 1980. Pediatric chondrosarcoma has a relatively rapid onset, manifested by pain, a palpable mass and neurological symptoms with the lesion localized in the spinal column. The pelvis was the most frequent location. Ten patients had primary and two patients had secondary chondrosarcoma. Of the latter two, one developed chondrosarcoma in a preexisting osteochondroma and the other had irradiation for neuroblastoma. The poorest outcomes were in patients who had involvement of the pelvic girdle and spinal column. Grade 2 and 3 lesions had the worst prognoses with metastases occurring within 12 months. Eight patients died at the time of study, and the longest survival time was 80 months. If surgically feasible, the treatment is radical excision. Radiation therapy and chemotherapy should be reserved for recurrences or distant metastasis.

Adolescent↗

PubMedDate not supplied

Chondrosarcoma of the hand.

A case of chondrosarcoma of the hand recurred 4 times over a period of 32 years. Forty-seven cases of hand chondrosarcomas have been found in the literature. Histological diagnosis is difficult in differentiating chondrosarcomas from benign chondromas. Pain and swelling are the main symptoms of recurrence. The diagnosis is established by arteriography and excisional biopsy. Extraskeletal hand chondrosarcomas do not metastasize and other hand chondrosarcomas do so only rarely. Block excision of localized amputation is the treatment of choice.

Aged↗

PubMed1993

[Experience with the surgical management of primary and secondary chondrosarcoma].

Forty eight primary and 20 secondary chondrosarcomas were treated surgically 1975 through 1991. An evaluation of the data of the Bone Tumor Register of the Semmelweis Medical University proved that the incidence of the malignant transformation and development of secondary chondrosarcomas is 3% and 2.6% among solitary osteochondromas. The authors summarize the clinicopathological characteristics of the malignant transformation. A retrospective evaluation of the histological grade of the malignancy proved that 67% of tumors were classified as grade I; 18% as grade II and 15% as grade III. The survival of the patients was mainly determined by the grade of the malignancy. A 95% 5-years survival was found in the grade I group and a 10% survival only in the grade II and III groups. In the cases of highly malignant chondrosarcomas radical surgical intervention i.e. amputation is recommended, considering the most often extra-compartmental location of the tumors. Low malignant and intra-compartmental highly malignant chondrosarcomas should be treated, however, by limb saving surgery. In the cases of large inresecable but low malignant chondrosarcomas debulking surgery is also acceptable.

Adolescent↗