Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clear cell chondrosarcoma is a rare bone tumor of low malignancy. It constitutes only 2% of all chondrosarcomas and shows a particular predilection for the epiphyses of long bones. So far, there have only been three cases of clear cell chondrosarcoma of the larynx published in the literature. We report the case of a 46 year old man who suffered from slowly worsening dyspnoea. Diagnostic evaluation revealed a directly subglottic stenosis of the larynx, narrowing the lumen to 1/3. When we tried to resect the stenosis via splitting the ring cartilage from outside, tumorous infiltration of the whole ring cartilage was found. Extended biopsies were taken and histology revealed a clear cell chondrosarcoma. The therapy of choice for this tumor is wide surgical resection. Additional radio- and/or chemotherapy are not recommended. Therefore, the patient underwent laryngectomy. Histologically, the clear cell chondrosarcoma can be easily confused with the highly malignant osteosarcoma or the conventional chondrosarcoma, both requiring more aggressive treatment. Thus, clear cell chondrosarcoma should be kept in mind and, when necessary, a reference histology by a bone tumor register should be requested.
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PMID:[Clear cell chondrosarcoma of the larynx. A rare tumor in an uncommon location]. 1531 26

Clear cell chondrosarcoma is a rare variant of chondrosarcoma that mostly involves the end of long bones. However, nine cases have been reported in the head and neck: four in larynx, two in nasal septum, two in maxilla and one in the skull. These cases form the basis of this review. Head and neck cases accounts for less than 5% of Clear cell chondrosarcomas in the whole body and the larynx is the most common place. The histological findings of head and neck cases are consistent with general features of this entity in the whole body and nearly all tumors in this case series had a component of conventional chondrosarcoma. Clear cell chondrosarcoma is an intracompartmental tumor and retains "Grenz zone" just beneath the epithelium. Therefore, the overlying mucosa remained intact in all laryngeal cases. Nasal tumor caused ballooning of the septum and the maxillary lesion did not involve the oral mucosa. This tumor presents various radiographic features in the head and neck area. Chondroblastoma, chondroma, osteoblastoma, osteosarcoma and metastatic renal cell carcinoma are included in the histologic differential diagnoses. Differentiation from chondroblastic osteosarcoma is important in the maxilla. A wide resection is adequate in most cases. However, some laryngeal cases show tendency to recur. Clear cell chondrosarcoma is a slow growing tumor and this necessitates a long time follow-up of patients. Due to the extreme rarity in the head and neck, diagnosis of Clear cell chondrosarcoma in this area, must be confirmed by histochemical and immunohistochemical studies.
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PMID:Clear cell chondrosarcoma of the head and neck. 2449 30

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.
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PMID:Epithelial and cartilaginous differentiation in clear cell chondrosarcoma. 2294 96

Chondrosarcoma is the third most common primary malignant bone tumor after osteosarcoma and Ewing's sarcoma. Clear cell chondrosarcoma is a rare subtype variant of chondrosarcoma, most commonly encountered in the proximal part of the femur or humerus. Vertebral involvement is exceedingly rare and shows a predilection for the thoracic spine. We report the case of a woman with clear cell chondrosarcoma of the thoracic spine, which has been surgically excised, and review the pertinent literature (PubMed). Although it has a reasonably benign biological behavior, clear cell chondrosarcoma needs to be treated as a malignancy. The best treatment for spinal chondrosarcoma is surgery. It should be promptly and adequately resected. Gross-total resection should be the ultimate surgical goal. Radiation therapy should also be considered, especially in the case of subtotal resection or inoperable lesions. In conclusion, it is important to keep in mind this entity in the differential diagnosis of spinal tumors, in order to optimize treatment planning. With adequate treatment, local recurrence rates as low as 20% can be achieved.
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PMID:Primary clear cell chondrosarcoma of the spine: a case report of a rare entity and a review of the literature. 2309 59