Unlike other primary bone cancers that mainly affect children. In total, files from 160 patients with a diagnosis of dedifferentiated central chondrosarcoma at the rizzoli. Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. The molecular pathogenesis of dedifferentiated chondrosarcoma. Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. These tumors make less than 2% of all chondrosarcoma cases 11, having the highest incidence in the 2030 yr age group and the 5year survival of about 50% 12. The extraskeletal localization of undifferentiated chondrosarcoma is very rare. Referring to a cell or tissue which has acquired a less mature appearance and metabolic profile, an event classically associated with aggressive malignancies. Conventional chondrosarcoma juxtaposed to high grade pleomorphic or spindle cell sarcoma may have features of angiosarcoma, clear cell chondrosarcoma, fibrosarcoma, osteosarcoma, rhabdomyosarcoma, squamous cell carcinoma. Fine needle aspiration cytology of a metastatic skin nodule diagnosed as a dedifferentiated chondrosarcoma. It arises most frequently in the 5th and 6th decade of life. They make up approximately 11% of all chondrosarcomas, and are among the most aggressive cancers described.
H3k27me3 deficiency defines a subset of dedifferentiated. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. The fourth and most serious grade is called dedifferentiated chondrosarcoma. Chondrosarcoma symptoms, prognosis, treatment, survival. Dedifferentiated chondrosarcoma in the scapula hong kong. In preclinical research aiming at the identification of novel treatment targets, the need for representative cell lines and model systems is high, but availability is scarce. Peripheral dedifferentiated chondrosarcoma dcs is an exceedingly rare aggressive surface bone neoplasm in which a highgrade sarcoma arises within an osteochondroma. Who classification of tumours of soft tissue and bone, 4th, fletcher cdm, bridge ja, hogendoorn pcw, mertens f eds, iarc, lyon 20. Dedifferentiated chondrosarcoma of the cervical spine. Highgrade conventional chondrosarcoma and dedifferentiated chondrosarcoma have a poor outcome.
Pdf background dedifferentiated chondrosarcomas consist of two distinguishable components. Chondrosarcoma is usually slow growing at low histological grades and can be well managed by. Clinical outcome for patients with dedifferentiated. Dedifferentiated chondrosarcoma of bone springerlink. Bone tumors in general are uncommon, with 5,000 to 6,000 cases diagnosed each year, which is about 0. Dedifferentiated chondrosarcoma italian ministry of health.
People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. Dedifferentiated chondrosarcoma was first described in 1971 by dahlin and beabout. Conventional chondrosarcoma is the most common 90% subtype. The noncartilaginous component may constitute a very small or a very. Dedifferentiated chondrosarcomas consist of two distinguishable components. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Amplification of the cmyc protooncogene in human chondrosarcoma. Multiple rings and arcs with relatively intact cortex indicated a chondroid lesion. The diagnosis of dedifferentiated central chondrosarcoma requires a bimorphic histologic pattern with areas of a low. Peripheral dedifferentiated chondrosarcomas are among the rarest malignant. This helps to estimate the time likely to be taken by the tumor to progress into cancer. There are many confusing issues concerning the imaging feature that can facilitate early diagnosis and the factors that might be related to outcomes. Pathology outlines chondrosarcoma dedifferentiated.
Chondrosarcoma cs is the collective term for a group of heterogeneous, generally slowgrowing, primary malignant tumors of bone characterized by the formation of hyaline cartilaginous neoplastic tissue. Chondrosarcoma cs consists of a heterogeneous group of tumors that share the common feature of cartilage matrix production. The disease usually starts in the bones of the arms. Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and shows an increased growth rate and rapid metastatic spread in comparison with ordinary chondrosarcomas. Anatomically, the femur, pelvis and humerus are the most common sites of involvement. Grimer rj1, gosheger g, taminiau a, biau d, matejovsky z, kollender y, sanjulian m, gherlinzoni f, ferrari c. The cases of chondrosarcoma variants, such as clear cell chondrosarcoma, dedifferentiated chondrosarcoma, mesenchymal chondrosarcoma, and chondrosarcomas arising from soft tissue, were excluded from. We herein describe a first case of cervical ddcs in an 81yearold male presenting with a slowly growing mass. Dedifferentiated chondrosarcoma is a rare but highly malignant form of chondrosarcoma with grave prognosis. Chondrosarcoma grading and staging system grading of the tumor is done on the basis of the appearance of the cancerous cells under the microscope. Dedifferentiated chondrosarcoma is a relatively rare variant of chondrosarcoma, arising in between 10% and 15% of all chondrosarcomas. They primarily affect adults and are the second most common primary solid tumor of bone after osteogenic sarcoma1. Axial ct showed a large expansile lesion arising from the left thyroid cartilage.
Pdf clinical outcome for patients with dedifferentiated. Dedifferentiated chondrosarcoma portnotes orthopaedicsone. All authors read and approved the final manuscript. Background dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a poor survival.
About 30% of skeletal system cancers are chondrosarcomas. The manual selection tool was used to trace the periphery of the tumor, and the. Primary ddcs of the mobile spine is extremely rare, particularly in the cervical spine. Dedifferentiated central chondrosarcoma staals 2006. Clinical outcome for patients with dedifferentiated chondrosarcoma. Furthermore, dedifferentiated chondrosarcoma is a relatively rare high grade sarcoma next to a lowgrade conventional malignant cartilageforming tumor, comprising 610% of all chondrosarcomas. Immunohistochemical analysis of laryngeal chondrosarcoma. Nine cases 4 males, 5 females of dedifferentiated chondrosarcoma were treated in our institute.
Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a highgrade nonchondroid spindle sarcoma. Dedifferentiated central chondrosarcoma american cancer society. The diagnosis is made histologically when a lowgrade cartilage lesion is found in conjunction with a highgrade sarcoma. Dedifferentiated chondrosarcomas are cartilaginous tumors that consist of two distinguishable components, a lowgrade chondrosarcoma chondrogenic. Dedifferentiated chondrosarcoma arising in the orbit. A case report m mitroi1, 2, a capitanescu1, 2, 1,c 1foarfa1, 2, d albulescu 2, r ionascu abstract laryngeal chondrosarcoma is a rare entity, reportedly comprising less than 1% of all laryngeal tumors. Dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis. Dedifferentiated chondrosarcoma ddcs comprises approximately 10% of all chondrosarcomas and has the worst outcome with 5year survival of 10%. Types defination cf frequency radiology epidemiology histology etiology 4. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long. Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its. Dedifferentiated chondrosarcoma is defined as a high grade, nonchondroid sarcoma associated with a low grade cartilaginous tumor. Chondrosarcoma is the second most common primary sarcoma of bone. Dedifferentiated chondrosacromas start out as typical chondrosarcomas but then some parts of the tumor change into cells like those of an osteosarcoma or fibrosarcoma.
The preferred localizations are femur, humerus and pelvis. Chondrosarcoma of small bones of the hands and feet. Dedifferentiated chondrosarcoma is defined as a nonchondrogenic highgrade sarcoma associated with welldifferentiated chondrosarcoma with abrupt transition 1, 2. Final diagnosis dedifferentiated chondrosarcomas upmc. Chondrosarcoma symptoms, diagnosis, treatment and prognosis. All these things have to be taken into consideration. So, for example, if there is a healthy young male with a large grade 3 dedifferentiated chondrosarcoma in the pelvis, he would receive different treatment than a retired lady with a small grade 1. Digital images were saved as joint photographic experts group jpeg files. Full text get a printable copy pdf file of the complete article 1. Researched and authored by prof michael c herbst d litt et phil health studies. Chondrosarcoma is a form of cancer that results from the abnormal cellular growth of the cells responsible for the formation of cartilage in the bones. Various types of cancer can affect the skeletal system, and an. Histologically these tumours consist of an underlying cartilaginous component either benign or malignant juxtaposed to a highgrade noncartilaginous component, with a typically abrupt transition between the two tissue types.
Dedifferentiated chondrosarcoma of the larynx american. Four such examples were identified in our files, representing 11. Chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. We report a case of ddcs that arose in proximal phalanx of lefthand thumb of a 66yearold man. Dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a poor survival. Chondrosarcoma is a malignant bone tumor arising from cartilaginous tissue, most frequently occuring at the ends of the femur and tibia, the proximal end of the humerus and the pelvis. Conventional also known as central is a lowgrade conventional malignant cartilageforming tumor and makes up approximately 90% of all chondrosarcomas dedifferentiated high grade sarcoma next to a, comprising 610% of all. A 74yearold man with a history of a chondroid lesion of the larynx noted an enlarging neck mass. Chondrosarcoma n10 osteosarcoma n10 leiomyosarcoma n10 undifferentiated pleomorphic sarcoma n10 dedifferentiated liposarcoma n10 vascular sarcoma n7 treat until. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Dedifferentiated chondrosarcoma occurs in approximately 11% of patients diagnosed with chondrosarcoma and is considered to be a histological subtype of this chondroid lesion. Pdf dedifferentiated chondrosarcoma of the cervical.
It is a rare cancer that accounts for about 20% of bone tumors and is diagnosed in approximately 600 patients each year in the united states. A unique case of dedifferentiated chondrosarcoma arising solely within the orbit is presented. Osteosarcoma is the most common type of associated highgrade malignancy. Chondrosarcoma genetic and rare diseases information. Irregularity of the anterolateral margin abutted a prominent softtissue component. This particular type of cancer belongs to the classification of soft tissue malignancies, known specifically as sarcomas. Clear cell ccc is commonly considered to be a lowgrade subtype and less than 1% of chondrosarcoma. Radiographs showed an expansion of the cortical contour of the c2. Chondrosarcoma is a type of sarcoma that affects the bones and joints. Dedifferentiated 25 wd component 17 017 0% dd component 23 922 41% validation cohort 22 dedifferentiated 22 wd component 15 015 0% dd component 22 1121 52% pdl1 is only expressed in dedifferentiated chondrosarcoma but not in other chondrosarcoma subtypes or benign chondroid tumors kostine m et al. Dedifferentiated chondrosarcoma is a rare, highly malignant tumor with. Dedifferentiated chondrosarcoma is a rare malignancy with reported 5. The two components are juxtaposed with abrupt clear demarcation line.
Dedifferentiated definition of dedifferentiated by. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas. We used five conventional and three dedifferentiated chondrosarcoma cell lines and investigated the effect of pi3kaktgsk3. Obtaining the necessary tissue for a histological examination can be difficult. The high grade sarcoma is most commonly an mfh, osteosarcoma or fibrosarcoma although others may occur. Undifferentiated chondrosarcoma has a very low survival rate. Chondrosarcoma accounts for 25% to 40% of these bone tumors. Conventional chondrosarcomas can be categorized according to. The purpose of this investigation is to determine the overall survival of dedifferentiated chondrosarcoma in a modern patient series and how it is impacted by patient demographics, tumor characteristics, and surgical.