Cases Of Clear Cell Chondrosarcoma Research Articles

An Unusual Case of Clear Cell Chondrosarcoma With Early Metastatic Recurrence.

We present a case of a 58-year-old male who presented following 4 months of progressively worsening right upper extremity pain. Initial pathology demonstrated pleomorphic chondroblasts with increased mitotic activity indicating an intermediate grade (Grade 2) clear cell chondrosarcoma of the proximal humerus. Following surgical resection, the primary lesion demonstrated aggressive behavior and early metastasis to the cervical and thoracic spine. The patient unfortunately expired 30 months after initial presentation. Although clear cell chondrosarcoma has been historically regarded as low grade, we present a unique example of an aggressive clinical course of clear cell chondrosarcoma.

Oncological and functional outcome in a rare case of clear cell chondrosarcoma of the proximal femur

Chondrosarcomas are the second most common bone sarcomas, accounting for less than 0.5% of all cancers. The treatment plan can be improved by analyzing clinical characteristics and treatment patterns. The rarity and slow-growing nature of this tumour often lead to prolonged symptoms and also initial misdiagnosis with avascular necrosis of the femur, benign bone lesions like chondroblastoma. The initial reports of this patient suggested avascular necrosis of the femur head. His pain became severe and further investigations were done, MRI confirmed the diagnosis of Chondrosarcoma. An image-guided core needle biopsy suggested Clear Cell Chondrosarcoma (CCC). Wide local resection with negative margins forms the mainstay of treatment since intralesional procedures predispose to a high local recurrence rate. A prolonged follow-up is required since late local recurrences of tumour and distant metastases are common.

Clear cell chondrosarcoma calcaneum – A case report and review of literature

Chondrosarcoma is the second most common primary bone malignancy accounting for 20–25% of all bone sarcomas. However chondrosarcoma of the foot is rare with just a handful of cases being described. Among the subtypes clear cell variant is the rarest and has never been documented in the foot. We present a rare case of clear cell chondrosarcoma of the calcaneum with multiple metastases that was treated at our institute. The patient was a 62-year old male who presented to us with pain and mass in the left hindfoot with difficulty in walking for 2 years and a discharging ulcer over the lateral aspect for 4 months. Radiography showed aggressive, destructive, lytic lesion in the calcaneum with cortical breach and soft tissue invasion. Bone scan and PET-CT revealed multiple bony metastases and lung metastasis. After initial biopsy, patient underwent below knee amputation and has been in remission since the last 18 months. Given the rarity of this tumor in the calcaneum, this report highlights the importance to consider the possibility of this tumor in the calcaneum as an early diagnosis; complete metastatic workup and expeditious management can thus significantly improve prognosis.

An unusual case of clear cell chondrosarcoma with very late recurrence and lung metastases, 29 years after primary surgery.

Clear cell chondrosarcoma is a rare bone neoplasm with low-grade clinical course and the potential to metastasize to the skeleton and lungs. The aim of this report is to present a case that is extremely rare, but in accordance with the literature where the clear cell chondrosarcoma reportedly has a tendency for late metastases. In our patient the primary surgery was intralesional, since it was mistakenly interpreted as a benign tumour in the early 80s. The local recurrence and lung metastases occurred, however, 29 years after the initial treatment. The local recurrence was resected with wide margins, no additional surgery or oncological treatments were given, and two and half years postoperatively patient is doing well and there is no progression in the disease. In conclusion, it is important to have a long follow-up to the clear cell chondrosarcoma patients even for decades or lifelong, because the malignancy tends to metastasize or recur after an extended period. The course of metastasized disease may be unusually slow, so relatively aggressive treatment in metastasized and recurring cases is justified.

Clear cell chondrosarcoma: Cytologic findings in six cases

Clear cell chondrosarcoma (CCCS) is a rare variant of chondrosarcoma characterized, in most instances, by indolent behavior and a long interval to progression of disease. CCCS commonly occurs in adult individuals and has a proclivity for the epiphysis of long bones, although it has been reported in other sites. This lesion is difficult to diagnose preoperatively. Factors contributing to difficulty in recognizing this lesion include its relative scarcity as well as its tendency to be confused with other lesions on imaging studies. In the following, we report six cases of CCCS initially diagnosed by fine needle aspiration and/or touch preparations of needle biopsy samples. The cytologic features identified include large, plasmacytoid cells with foamy cytoplasm as well as extracellular chondroid type matrix material. Definitive diagnosis was made in each case by recognizing the "clear cell" nature of the tumor on cell block material.

Epithelial and cartilaginous differentiation in clear cell chondrosarcoma

Clear cell chondrosarcoma is a rare cartilaginous bone tumor, and little is known about its pathology. We investigated the immunohistochemical expression profiles of cytokeratins (CAM5.2, AE1/AE3, CK7, CK8, CK18, and CK20), epithelial membrane antigen, SRY (sex-determining region Y)-box 9, type II collagen, runt-related transcription factor 2, and osteocalcin in clear cell chondrosarcoma and compared them with those in chondroblastoma, conventional chondrosarcoma, and osteosarcoma. Of 5 cases of clear cell chondrosarcoma, 3 demonstrated positive staining for AE1/AE3 and some form of cytokeratin in the clear cell component. Of the 5 cases, 4 strongly expressed SRY (sex-determining region Y)-box 9 in the clear cell component but weakly expressed it in the cartilaginous component. Of the 5 cases of clear cell chondrosarcoma, 3 expressed runt-related transcription factor 2 in both the clear cell and cartilaginous components, but no expression of osteocalcin was detected. In chondroblastoma, 8 of 13 cases expressed AE1/AE3, and other cytokeratins, such as CK7 (4/13), CK8 (6/13), CK18 (8/13), and CK20 (3/13), demonstrated a similar staining extensity pattern between the cellular and cartilaginous components. Clear cell chondrosarcoma and chondroblastoma have similar immunohistochemical features in that they both express epithelial and chondrogenetic markers. On the other hand, tumor cells of clear cell chondrosarcoma have no osteoblastic immunohistochemical expression in comparison with chondroblastoma.

Clear Cell Chondrosarcoma Arising From the Sternum: A Rare Tumor in an Uncommon Location

A 52-year-old woman presented with bulging of the anterior chest wall. The computed tomographic scan revealed an expansive localized mass based on the sternal manubrium. The patient was successfully treated with en bloc radical resection and reconstruction with clear resection margin. Histopathologic examination of the surgical specimen confirmed the diagnosis of clear cell chondrosarcoma. After the surgery, the patient has been free of disease for 43 months after surgery without other treatment. Our search and review of the literature did not reveal any published cases of clear cell chondrosarcoma arising from the sternum; therefore, we have presented a summary of this novel case with a review of the relevant literature.

Rare Occurrence of Primary Chest Wall Bone Tumours

Primary tumors involving the bony skeleton of the chest wall are uncommon. After a retrospective review of the histopathology archive at our institution from Oct 08 to Mar 09 we found three malignant neoplasms affecting the rib, clavicle and the costal cartilages, one mesenchymal chondrosarcoma, one case of clear cell chondrosarcoma and an aggressive osteoblastoma each having distinctive histological diagnosis. Our limited experience in the occurrence of primary chest wall tumors showed that there are occurrences of rare bony lesions. A strong clinical suspicion, clinico-radiological correlation and histopathological confirmation are required for proper evaluation.

Clear cell chondrosarcoma: an ultrastructural study

Clear cell chondrosarcoma (CCC) is a rare neoplasm. We report here a case of CCC. A 67-year-old Japanese man presented with right arthralgia for 1 year, and histological examination of the subsequent surgical resection of the right femoral bone showed the finding of CCC. Ultrastructurally, most organelles were observed in the perinuclear area. Clear neoplastic cells contained many glycogen particles in the area of the cytoplasm lacking organelles, although glycogen particles overall seemed to be evenly distributed in the cytoplasm. Some mitochondria, Golgi complex, actin-like filaments, and rough endoplasmic reticulum were also demonstrated in the cytoplasm of clear cells. Well-developed microvilli were also seen on the surface of neoplastic cells. These structures in neoplastic cells corresponded notably to structures of normal chondrocytes. Finally, our ultrastructural findings support further evidence that clear cells in CCC may show chondrocyte differentiation and a lack of an organelles area as well as abundant glycogen particles, may contribute to the clear cell morphology in CCC.

Clear cell chondrosarcoma of bone: long time follow-up of 18 cases

Clear cell chondrosarcoma is a variant of conventional chondrosarcoma behaving as low grade malignant bone tumor. We reviewed our cases of clear cell chondrosarcoma with the aim to assess the best diagnostic and curative approach to this rare tumor. We report 18 cases of clear cell chondrosarcoma treated at the authors' institution. Pertinent clinical information, pre and postoperative serum alkaline phosphatase (SAP) level were obtained from the patients charts. Available imaging studies including plain roentgenograms, computed tomography (CT) images (in 6 patients) and magnetic resonance imaging (MRI) images (in 2 patients). Eight cases were firstly treated in other institutions, seven with one or more curettage, while one, presented with pathologic fracture, was treated with repeated osteosynthesis. Of the seven patients treated with curettage, five were referred with local recurrence, the mean time between surgical treatment and local recurrence was 52.2 months (from 12 to 132). The other two patients were curetted out 4 and 1 month before, respectively, and were then referred to us to decide about further treatment after incidental diagnosis. Of these cases, three (37.5%) recurred again after our treatment and two of them presented with late bone metastases. Eight patients were originally treated with wide (six) or marginal (two) surgical margins by us. Among them only one proximal humerus (wide margins) had local recurrence 26 months from the first operation. Two patients underwent curettage in our institution, and one of them had a local recurrence. Pre-operative SAP level were obtained in 17 patients. Among them, 11 patients had high pre-operative SAP level (64.7%) and 6 cases were normal. Post-operative SAP levels were obtained in nine patients. After removal of the tumors; SAP levels decreased to normality in seven of the nine patients with high pre-operative SAP. In the other two patients, although the SAP levels were normal, post-operative SAP levels were decreased. Serum alkaline phosphatase can be used as a good tumor marker in diagnosis and follow-up. Due to the occurrence of local recurrences and distant metastasis after curettage, we suggest surgical treatment with "en bloc" resection from the first presentation.

Aggressive Clear Cell Chondrosarcomas: Do Distinctive Characteristics Exist?: A Report of 4 Cases

Clear cell chondrosarcoma (CCC) is commonly considered to be a low-grade subtype of chondrosarcoma. However, a few cases of CCC behave as high-grade lesions (with early metastases or multiple/synchronous locations). To investigate morphologic features that can help predict the aggressiveness of these CCCs. To investigate possible hallmarks of this aggressiveness, we are presenting the clinicopathologic features of 6 cases of CCC, 4 of which presented aggressive features and 2 low-grade behavior. The patients were 5 men and 1 woman; their ages ranged from 22 to 47 years. Histologic appearance, ultrastructure, and immunohistochemical expression of metalloproteinase 1 and 2 and their inhibitors were evaluated in all 6 cases. Pain was the most common symptom; the lesions were located in the femur (4), humerus (2), and vertebral body (1), with 1 patient presenting a double/synchronous lesion. Although no major differences were detected using conventional light microscopy, an ultrastructural analysis--at variance with usual cases--showed a lack of superficial microvilli in more than 50% of neoplastic cells in the aggressive cases, therefore suggesting a less differentiated phenotype. In addition, metalloproteinase 2 was more diffusely expressed in the aggressive tumors than in the conventional CCCs, whereas p53 labeling was always negative. The aggressive behavior of some CCCs may be, at least in part, correlated to a lesser degree of cell differentiation and to the expression of tumor cell proteins, such as metalloproteinase 2, which are able to favor neoplastic spreading.

Aggressive clear cell chondrosarcomas: do distinctive characteristics exist?: a report of 4 cases.

Abstract Context.—Clear cell chondrosarcoma (CCC) is commonly considered to be a low-grade subtype of chondrosarcoma. However, a few cases of CCC behave as high-grade lesions (with early metastases or multiple/synchronous locations). Objective.—To investigate morphologic features that can help predict the aggressiveness of these CCCs. Design.—To investigate possible hallmarks of this aggressiveness, we are presenting the clinicopathologic features of 6 cases of CCC, 4 of which presented aggressive features and 2 low-grade behavior. The patients were 5 men and 1 woman; their ages ranged from 22 to 47 years. Histologic appearance, ultrastructure, and immunohistochemical expression of metalloproteinase 1 and 2 and their inhibitors were evaluated in all 6 cases. Results.—Pain was the most common symptom; the lesions were located in the femur (4), humerus (2), and vertebral body (1), with 1 patient presenting a double/synchronous lesion. Although no major differences were detected using conventional light micr...

Type II collagen, but not aggrecan expression, distinguishes clear cell chondrosarcoma and chondroblastoma

Chondroblastoma and clear cell chondrosarcoma are uncommon skeletal neoplasms that have a strong tendency to involve the epiphysis of long bones. They also share some overlapping histological features. Thus, it can be difficult both radiographically and histologically to distinguish these neoplasms. So far there are no immunohistochemical markers available that have been shown to be helpful in differentiating these neoplasms. In our study of a series of clear cell chondrosarcomas (n = 15) and chondroblastomas (n = 35), S100, vimentin, aggrecan and collagen type II were detected by immunohistochemistry. We detected immunohistochemical evidence of type II collagen within both the extracellular matrix-rich (chondroid) and matrix-poor areas in all 15 cases of clear cell chondrosarcoma. In contrast, immunohistochemical analysis failed to show staining of collagen type II in any of the 35 chondroblastomas. Other markers, including S100 protein, vimentin and aggrecan proteoglycan were tested in parallel and found to be focally positive in both neoplasms. Therefore, our data show that in cases when clear cell chondrosarcoma and chondroblastoma pose a diagnostic challenge, the presence of type II collagen in the extracellular tumour matrix significantly supports the diagnosis of clear cell chondrosarcoma and aids in distinguishing it from chondroblastoma.

Cytogenetic findings in clear cell chondrosarcoma

Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy that most often arises in the epiphysis of long bones in the third and fourth decades of life. Cytogenetic studies of clear cell chondrosarcoma are few. In this study, the cytogenetic findings of 4 cases of clear cell chondrosarcoma are presented. Clonal chromosomal abnormalities were detected in 3 cases. A tumor specific anomaly was not identified, however, extra copies of chromosome 20 and loss or rearrangements of 9p appear to be recurrent.

Chondroblastoma and clear cell chondrosarcoma: radiological and MRI characteristics with histopathological correlation.

To analyze and compare the radiological and magnetic resonance imaging (MRI) appearances of chondroblastoma and clear cell chondrosarcoma with histopathological correlation. Twelve patients with histologically proven chondroblastoma and of another four patients with clear cell chondrosarcoma were investigated by radiographs and MRI (T1-, T2-weighted sequences, intravenous gadolinium application). Additionally, the clinical and radiologic data of seven cases of clear cell chondrosarcoma without available MRI were considered. The localization, calcification of tumor matrix, periosteal reaction, cortical bone and patterns of bone destruction were analyzed according to the Lodwick radiological grading system (LRGS). The signal intensity on T1- and T2-weighted sequences, characteristics of contrast enhancement, associated bone marrow edema, soft tissue reaction and joint involvement were evaluated. Histopathological specimens were available in all cases. The age of patients with chondroblastoma (range 15-59 years, mean 22.3 years) was lower than that of those with clear cell chondrosarcoma (range 19-61 years, mean 36.6 years), and the lesions were smaller in the chondroblastoma group (range 1-4 cm, mean 2.3 cm) than in patients with clear cell chondrosarcoma (range 3-7.5 cm, mean 5.2 cm). The chondroblastomas were more confined to the epiphysis (10/12) than the clear cell chondrosarcomas. All chondroblastomas and clear cell chondrosarcomas except one were classified as grade 1A or 1B according to the LRGS; one clear cell chondrosarcoma was judged as grade 2. Signal intensity of the tumors on MRI was very heterogeneous in both groups. High signal intensity on T2-weighted MR images in chondroblastoma mostly corresponded to areas with aneurysmal bone cyst components and in clear cell chondrosarcoma to islands of hyaline cartilage. Contrast enhancement occurred in all tumors and tended to be more intense with clear cell chondrosarcoma. Chondroblastoma was more frequently associated with bone marrow edema (11/12), periosteal reaction (10/12), soft tissue reaction (7/12) and synovitis (3/12). Chondroblastoma occurs in younger patients, is smaller than clear cell chondrosarcoma and is more confined to the epiphysis. The overlap of signal intensity and contrast enhancement patterns does not allow a reliable differentiation of the two tumors by MRI. Chondroblastomas are typically associated with bone marrow edema, periosteal reaction and soft tissue reaction.

Clear cell chondrosarcoma of the pelvis in a skeletally immature patient

We report on a case of clear cell chondrosarcoma (CCCS) of the left iliac bone in a 12-year-old skeletally immature boy. Radiographic examination revealed an aggressive osteolytic lesion with areas of mineralization. Fluid-fluid levels were seen on T2-weighted MR images. Laboratory data showed slight elevation of serum alkaline phosphatase. The biopsy specimen showed histological features of CCCS with some resemblance to osteosarcoma, such as prominent irregular osteoid formation among clear tumor cells. Surgical treatment was accomplished without pre- or post-operative chemotherapy. Because of the patient's age, elevated serum alkaline phosphatase, and histopathology with prominent osteoid production, this case could be confused with osteosarcoma. Although CCCS is an extremely rare bone tumor in children, it is important to be aware that it may arise in a skeletally immature patient. CCCS, unlike osteosarcoma, is not treated with neo-adjuvant chemotherapy.

Clear cell chondrosarcoma of bone

We report 8 cases of clear cell chondrosarcoma from 350 cases of chondrosarcoma (2.2%) seen at Istituto Rizzoli. The lesion is always low grade in nature and occurs in an epiphyseal location, usually in the proximal femur or humerus. This relatively inaccessible location may hinder the exact diagnosis. Wide resection is the treatment of choice, although 3 of our patients underwent intralesional or marginal procedures. At present all patients are free of disease. However, a longer followup is essential because of the slow-growing nature of this sarcoma.

Histogenesis of clear cell chondrosarcoma

The histogenesis of clear cell chondrosarcoma is still unclear: Apart from typical clear cell tumor areas, extensive production of woven bone formation suggests within the clear cell cartilagenous stroma is an intriguing phenomenon. Three cases of clear cell chondrosarcoma documented in the Bone Tumor Registry of Westphalia were examined for their patterns of osteonectin expression, and compared with other bone tumors of either osseous or cartilaginous origin, and with normal cartilage tissue. Found predominantly in osseous structures, the protein osteonectin takes part in the formation of new bone. The three clear cell chondrosarcomas showed a strong immunoexpression of osteonectin in clear cell, chondroid and in osseous tumor areas. Similarly, evidence of osteonectin was also found in osteoblastic and in chondroblastic osteosarcomas as well as in osteoblastomas. In contrast, osteonectin could not be demonstrated in the chondrosarcomas and mesenchymal chondrosarcomas from our registry that were analysed for comparison, and was found only minimally in the fibroblastic areas of dedifferentiated chondrosarcomas. The chondroblastic tumor components were always negative. There was no immunoexpression of osteonectin either in fetal or adult intervertebral disc tissue. The present immunohistochemical study of osteonectin has distinctly separated clear cell chondrosarcoma from the other variants of chondrosarcoma, and aptly verified the specificity of this entity. Moreover, the study would call for further histogenetic evaluation of clear cell chondrosarcoma, since the pattern of osteonectin expression in that tumor seems to indicate an osteogenic rather than a chondrogenic origin.

Clear cell chondrosarcoma: case report and ultrastructural study

A case of clear cell chondrosarcoma in a Chinese patient is described. The clear cells showed strongly positive S-100 protein immunoreactivity. Ultrastructurally 2 types of chondroid cells were demonstrated. One type appeared more primitive with abundant electron-lucent cytoplasm and sparse organelles. The other type of cell was more differentiated with presence of microvilli and numerous dilated cisternae of endoplasmic reticulum. Previous ultrastructural studies on these lesions were reviewed and compared with the present findings.