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PubMed1999

Clear cell chondrosarcoma: a pathological and immunohistochemical study.

AIM: Clear cell chondrosarcoma (CCC) is a rare malignant cartilaginous neoplasm of bone. CCC is characterized by clear cells (CCC cells), osteoclasts and osteoblasts. Many important questions concerning the varied histological features of CCC, and the interactions between CCC cells and coexisting osteoclasts and osteoblasts have not been fully investigated and remain controversial. The aim of this study is to clarify and explain the varied histological features and the possible interaction between tumour cells (CCC cells) and stromal cells such as osteoclasts and osteoblasts. METHODS AND RESULTS: Four cases of CCC were histologically and immunohistochemically studied in order to elucidate the biological nature and histological characteristics. A comparative study with chondroblastoma and grade I conventional chondrosarcoma (CC) was also performed. S100 protein and type II collagen were expressed in CCC cells, chondroblastoma cells and CC cells. CD68 and matrix metalloproteinase-9 were expressed in coexisting histiocytes and osteoclasts. Parathyroid hormone-like protein (PTH-LP) was expressed in histiocytes, osteoclasts, osteoblasts, chondroblastoma cells and CCC cells. Platelet-derived growth factor (PDGF) and its receptor (PDGF-R) were observed in osteoblasts, chondroblastoma cells and CCC cells. However, PTH-LP, PDGF and PDGF-R were not expressed in CC cells. PCNA (proliferating-cell nuclear antigen) was expressed more intensely in CCC than in chondroblastoma. CONCLUSION: These observations suggest that CCC cells trigger the varied histological changes in association with several cytokines. The difference of PCNA expression between CCC and chondroblastoma seemed to be related to the biological difference between the two tumours.

Adult↗
PubMed2002

Dedifferentiated chondrosarcoma with a noncartilaginous component mimicking a conventional giant cell tumor of bone.

We report a case of dedifferentiated chondrosarcoma in which the dedifferentiated component of the tumor shows a close histologic resemblance to a conventional giant cell tumor of bone. The tumor affected a 30-year-old woman with a long history of left shoulder discomfort and limitation of motion. Radiographic studies revealed a biphasic destructive lesion in the left proximal humerus composed of high-signal lobulated component on T2-weighted magnetic resonance image accompanied by a low signal intensity component exhibiting destructive growth with extension into soft tissue. Microscopically, two different areas consisting of the chondroid tissue and nonchondroid giant cell-rich lesion resembling conventional giant cell tumor of bone were found. Considering that the prognosis and survival associated with these two entities are very different, it is important to be aware of this variant of dedifferentiated chondrosarcoma to avoid the misdiagnosis of conventional giant cell tumor of bone.

Adult↗
PubMed2001

Successful removal of a giant recurrent chondrosarcoma of the thoracic wall in a patient with hereditary multiple exostoses.

Chondrosarcoma represents the most common malignant tumour of the chest wall, with a tendency for local recurrence after resection. Here we report the successful complete resection of a giant, local recurrent chondrosarcoma of the chest wall (max. diameter 25 cm), in a patient with hereditary multiple exostoses, who had had a wide resection 8 years before. Clinical features and surgical management are described.

Bone Neoplasms↗
PubMedDate not supplied

Chondrosarcoma of the talus: a case report.

We present histopathologic and imaging features, treatment alternatives, and follow-up of a patient with a secondary chondrosarcoma of talus. A 27-year-old man with a chondrosarcoma arising from a preexisting benign tumor was treated by talectomy. At 3 years' follow-up, there is no evidence of recurrence or metastasis. The patient can walk with slight limp without the aid of external support.

Adult↗
PubMed1998

Regulation of nitric oxide production by salicylates and tenidap in human OA-affected cartilage, rat chondrosarcomas and bovine chondrocytes.

OBJECTIVE: To examine the effects of non-steroidal anti-inflammatory drugs (NSAIDS) on nitric oxide (NO) and prostaglandin E2 (PGE2) production in chondrocytes from three different species. METHODS: We have estimated NO production by Griess method, and PGE2 by RIA from the supernatants of articular cartilage obtained from osteoarthritis joints (OA-affected cartilage), rat chondrosarcomas (in ex vivo conditions) and bovine chondrocytes (stimulated with cytokines + endotoxin in vitro conditions) in the presence or absence of aspirin, indomethacin, sodium salicylate, tenidap and glucocorticoids. RESULTS: NO, which was spontaneously released in ex vivo conditions by OA-affected cartilage and rat chondrosarcomas (maintained in vivo), was susceptible to inhibition by pharmacologically relevant concentrations of aspirin, sodium salicylate and tenidap, but not to concentrations of indomethacin or glucocorticoids that significantly inhibited PGE2 production under the same conditions. Similarly, the production of NO by bovine chondrocytes grown in monolayer cultures that had been stimulated with cytokines + endotoxins (in vitro) to release both NO and PGE2 (at 48-72 h post stimulation), were inhibited by aspirin, sodium salicylate and tenidap, but not by indomethacin or glucocorticoids at concentrations sufficient to PGE2 production. Inhibition of NO in the cytokines + endotoxin stimulated bovine chondrocytes (like the human OA-affected cartilage) augmented PGE2 production. CONCLUSION: These experiments demonstrate that NO production by chondrocytes across species show a similar profile of susceptibility to inhibition by selected anti-inflammatory drugs. The insensitivity of NO production to glucocorticoids is an important characteristics of these cells that merits further investigation.

Aged↗
PubMed1990

[Primary chondrosarcoma of the ureter].

A 62-year-old patient had complained of recurring colicky pain in the right renal bed, seven days before he was admitted as an inpatient. Macrohaematuria was an accompanying sign during one of these attacks. Sonography revealed right urinary obstruction of medium severity; the right kidney could not be demonstrated by urography. Since CT did not yield any clue, retrograde ureteropyelography was performed. An obstructive tumour of the right ureter was identified. This tumour, which had a length of 3.5 cm and a width of 1.2 cm, was completely resected by ureteronephrectomy. Examination revealed an extraskeletal mesenchymal chondrosarcoma of predominantly intraluminal growth, and a tumour-free external ureteral wall. A chondrosarcoma at this site has not been reported as yet. Pathogenesis may be ascribed either to degeneration of an existing chorista or to the development of a pluripotent tumour parent cell.

Chondrosarcoma↗
PubMed2003

No improvement in the overall survival of 194 patients with chondrosarcoma in Finland in 1971-1990.

We describe the clinicopathologic profile and survival of 306 patients with chondrosarcoma reported to the Finnish Cancer Registry in 1971-1990. 218 cases were available for reevaluation. Owing to their various clinicopathologic characteristics, we excluded the histologic variants of chondrosarcoma. Therefore, the final study population included 194 patients. The minimum follow-up was 9 years. The study population included 69 grade 1 tumors, 114 grade 2 tumors, and 11 grade 3 tumors. The commonest tumor sites were the chest, pelvis and femur. A local recurrence developed in 25% of the patients and metastatic lesions in 18%. The patients were treated in 31 hospitals (in 22 hospitals during the 1970s and in 26 in the 1980s), and the number of patients biopsied before the referral remained about the same from the 1970s (15%) to the 1980s (18%). The 5-and 10-year disease-specific survival rates were 70% and 57%, respectively. Multivariate analysis showed that the most important independent predictors of shortened survival were high histologic grade, age 50 years or older, and a diagnosis in the 1980s, as compared to the 1970s. Most findings accorded with reports from specialist treatment centers, but to our surprise, the survival rate declined among patients diagnosed in the 1980s versus the 1970s. The failure to improve patient survival is probably due to treatment of the patients in 31 hospitals rather than in a few centers dealing with treatment of cancer.

Adolescent↗
PubMed2001

Primary intracerebral mesenchymal chondrosarcoma with rhabdomyosarcomatous differentiation: case report and literature review.

Intracranial chondrosarcoma (Ch-S) is a slow-growing, locally recurrent, malignant cartilaginous tumour of the skull base. Intracranial mesenchymal chondrosarcoma (MsCh-S) is a rarer, more malignant variant associated with the supratentorial meninges. Only seven cases of Ch-S, and six of MsCh-S, that were primarily intraparenchymal in origin have been reported. Moreover, no case of intracranial Ch-S or MsCh-S has been reported in which rhabdomyosarcomatous differentiation was prominent. A 17-year-old Asian girl presented with a 4-week history of occipital headache, vomiting and paraesthesia in the left hand. She was drowsy with a left hemiparesis and had a dilated right pupil with bilateral papilloedema. CT demonstrated a large, partly calcified, contrast-enhancing mass in the right temporo-parietal region with oedema and midline shift. Through a large craniotomy, a tense brain was encountered with no apparent cortical abnormality. Despite a radical tumour excision, with excellent initial clinical recovery, a local recurrence rapidly occurred within weeks prior to the administration of any radiotherapy. Initial histopathological examination revealed a primary MsCh-S with osseous and rhabdomyosarcomatous differentiation, with an indistinct margin. After a second radical excision, a second recurrence rapidly occurred; however, this proved excessively vascular and inoperable. Radiotherapy was declined and death followed within 3 weeks. This is the seventh case of primary intracerebral MsCh-S to be reported and the first to demonstrate rhabdomyosarcomatous differentiation. It was characterized clinically by rapid, local recurrence with increased vascularity.

Adolescent↗
PubMed2004

Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome--three case reports and review of the literature.

Ollier's disease is a rare disease characterized by constitutional bone dysplasia with multiple enchondromas. The combination of haemangioma and chondromatoses is known as Maffucci's syndrome. Malignant degeneration of bone dysplasia into chondrosarcoma is a well-known complication, but a lesion of the base of the skull is exceptional. It is a slowly growing low-grade malignant tumour. Three new cases of chondrosarcoma of the base of the skull occurring in one patient with Marfucci's syndrome and two patients with Ollier's disease are reported and these cases are discussed in the light of the literature. A multidisciplinary approach, comprising surgery and radiotherapy, achieved good results.

Adult↗
PubMed1998

Chondrosarcoma of the chest wall with Ollier's disease.

A 15 x 15 cm mass in the right hemithorax of a 25-year-old woman was incidentally detected at routine chest radiography. Radical excision of the bony chest wall, including right CII-VI, revealed chondrosarcoma. Large, expansile parosteal chondromas were radiographically visualized in the pelvis and bones of the lower leg, representing Ollier's disease. Chondrosarcoma of the chest wall appears to be rare in Ollier's disease.

Adult↗
PubMed1999

EXT-mutation analysis and loss of heterozygosity in sporadic and hereditary osteochondromas and secondary chondrosarcomas.

Osteochondromas occur as sporadic solitary lesions or as multiple lesions, characterizing the hereditary multiple exostoses syndrome (EXT). Approximately 15% of all chondrosarcomas arise within the cartilaginous cap of an osteochondroma. EXT is genetically heterogeneous, and two genes, EXT1 and EXT2, located on 8q24 and 11p11-p12, respectively, have been cloned. It is still unclear whether osteochondroma is a developmental disorder or a true neoplasm. Furthermore, it is unclear whether inactivation of both alleles of an EXT gene, according to the tumor-suppressor model, is required for osteochondroma development, or whether a single EXT germline mutation acts in a dominant negative way. We therefore studied loss of heterozygosity and DNA ploidy in eight sporadic and six hereditary osteochondromas. EXT1- and EXT2-mutation analysis was performed in a total of 34 sporadic and hereditary osteochondromas and secondary peripheral chondrosarcomas. We demonstrated osteochondroma to be a true neoplasm, since aneuploidy was found in 4 of 10 osteochondromas. Furthermore, LOH was almost exclusively found at the EXT1 locus in 5 of 14 osteochondromas. Four novel constitutional cDNA alterations were detected in exon 1 of EXT1. Two patients with multiple osteochondromas demonstrated a germline mutation combined with loss of the remaining wild-type allele in three osteochondromas, indicating that, in cartilaginous cells of the growth plate, inactivation of both copies of the EXT1 gene is required for osteochondroma formation in hereditary cases. In contrast, no somatic EXT1 cDNA alterations were found in sporadic osteochondromas. No mutations were found in the EXT2 gene.

Adolescent↗
PubMed1978

Extraskeletal myxoid chondrosarcoma with "chordoid" features (chordoid sarcoma).

The light and electron microscopic features of a so-called chordoid sarcoma arising in the area of the gastrocolic ligament are described and illustrated. Despite the tumor's histologic resemblance to chordoma, the ultrastructural markers are those of chondroid rather than chordoid neoplasms. These are well-developed, vesciular granular endoplasmic reticulum, intracytoplasmic glycogen, lipid bodies, and a collagen-poor extracellular matrix with electron-dense granules, presumably representing chondroitin sulfate. Similar submicroscopic alterations have been observed in extraskeletal myxoid chondrosarcomas. Because of the distinctive morphologic appearance of this peculiar lesion, the term "myxoid chondrosarcoma with chordoid features" is suggested to reconcile histologic and ultrastructural features and histogenesis.

Aged↗
PubMed1981

Light- and electron-microscopic study of clear cell chondrosarcoma.

Clear cell chondrosarcoma is a recently recognized tumor of the bone. It has a low-grade malignant potential and characteristic histologic appearance. A case studied by light and electron microscopy showed considerable resemblance to chondroblastoma, but in addition, there were clusters of cells with clear cytoplasm containing glycogen and representing areas of well-differentiated chondrosarcoma. Electron microscopy demonstrated chondroid cells in various stages of differentiation from immature chondroblasts to mature chondrocytes.

Adult↗
PubMed1992

Extraskeletal myxoid chondrosarcoma of the pleura. Report of a case clinically simulating mesothelioma.

A primary extraskeletal myxoid chondrosarcoma of the pleura that clinically mimicked a malignant mesothelioma in a 66-year-old man with a history of asbestos exposure is described. Although exceedingly rare in this location, the characteristic histologic features, immunohistochemical reactivities, and ultrastructural features support the diagnosis of extraskeletal myxoid chondrosarcoma. Many ferruginous (asbestos) bodies consistent with the exposure history were found in the lung tissue sections and confirmed by energy-dispersive spectrometry. This case demonstrates an unusual pleural primary neoplasm associated with asbestos.

Aged↗
PubMed1993

Chondrosarcoma of the head and neck. The UCLA experience, 1955-1988.

Chondrosarcoma of the head and neck is a rare tumor. In an attempt to clarify optimal treatment of these lesions, we reviewed the records and pathologic material of 18 consecutive cases of head and neck chondrosarcoma seen at our institution between 1955 and 1988. Follow-up ranged from 3 to 168 months with a median of 72. Absolute 5-year survival was 68% (11/16), with 9/16 (56%) patients surviving disease-free. Grade was the most important prognostic factor. Only one of 7 (14%) patients known to have high grade histology was rendered disease-free, as opposed to 9/10 (90%) with low-grade lesions. Tumor size and completeness of surgical resection were also important prognostic factors. Four of 10 patients managed initially with surgery alone achieved local control with greater than 5-year survival. All four had low-grade lesions. Five patients received surgery and radiation as primary treatment, and three are disease-free with greater than 5-year follow-up. Two of these were irradiated because of positive margins. One patient received radiation alone and has persistent disease. Two patients received combined chemotherapy and surgery because of high-grade lesions, and one is free of disease with greater than 5-year follow-up. Patients with incomplete resections should receive further surgery or postoperative radiation therapy. High-grade lesions should be treated aggressively.

Adolescent↗
PubMed1984

Clear cell chondrosarcoma of bone. Observations in 47 cases.

Clear cell chondrosarcoma of bone, a low-grade malignant tumor, frequently presents diagnostic difficulties. Its clinical, roentgenographic, and pathologic characteristics separate it from conventional chondrosarcoma and, more importantly, from several benign bone tumors with which it is often confused. This report analyzes 47 cases seen at the Mayo Clinic and in consultation. The lesion is more common in males than females (2.6:1) and has a predilection for the end of long bones, particularly the proximal femur. The age range is wide; most patients are in the third and fourth decades of life. Roentgenographically, the lesion most often is purely lytic and slightly expansile, with a sharp margin between the tumor and the adjacent normal bone. Pathologically, clear cells arranged in an indistinct lobular pattern characterize the tumor. Frequently, areas mimicking other primary bone tumors, benign and malignant, are present, often to such an extent as to obscure the true identity of the process. The overall mortality in the present series was 15%. En bloc resection, including a margin of normal bone and soft tissue, appears to be the treatment of choice.

Adolescent↗
PubMed1993

Cytogenetic findings in a metastasizing primary testicular chondrosarcoma.

The first case of a primary chondrosarcoma of the testis is reported. The testicular tumor, 2.5 cm in diameter, metastasized to the retroperitoneal lymph nodes in a 24-year-old male. Histologically, the testicular tumor and the lymph node metastases revealed a well-differentiated chondrosarcoma. Cytogenetic analysis of the retroperitoneal metastases showed a complex chromosomal aberration with two copies of the isochromosome of the short arm of chromosome 12. This is a specific chromosomal anomaly in testicular germ cell tumors. Thus, the cytogenetic analysis proved the germ cell origin of tumor cells as opposed to a possible paratesticular mesenchymal histogenesis. Moreover, the triploid modal chromosomal number of tumor cells confirmed the relationship between polyploidization and a high degree of differentiation in germ cell tumors. These findings stress the clinical relevance of the cytogenetic analysis in at least a subset of cytogenetically well-characterized solid tumors.

Adult↗