The symptoms of this disorder can vary from one patient to another. Dcs of the spine arising from osteochondromas is extremely rare. However, in some cases the lesion can occur as an abnormal bony type of bump, which can vary in size and location. A total laryngectomy was performed for the dedifferentiated chondrosarcoma. There are very few published data on the survival of patients with dedifferentiated chondrosarcoma, or, more specifically, on the efficacy and role of chemotherapy, especially in the era of modern diagnostic and treatment modalities. Experience in the treatment of dedifferentiated chondrosarcoma. 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. Conventional tumors are divided by location into central, peripheral and juxtacortical periosteal forms tumors often recur at a higher histologic grade poorly differentiated tumors are uncommon, recur locally due to satellite nodules. Dedifferentiated chondrosarcomas cs are a highgrade variant of cs that confers a 5year survival of around 1024%. Dedifferentiated chondrosarcoma was first proposed in 1971 by dahlin and beabout. All these things have to be taken into consideration.
Chondrosarcomas are most frequently found in men between the ages of 3060 years although there is a slight female predominance for phalangeal chondrosarcomas. Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and shows an increased growth rate and rapid metastatic spread in comparison with ordinary chondrosarcomas. Chondrosarcoma, a malignant cartilaginous tumor, is the second most common primary malignancy of bone after osteosarcoma, often arising from the pelvis, femur, or humerus. Up to 20% of the tumour consisted of classical features of clear cell chondrosarcoma. The diagnosis is made histologically when a lowgrade cartilage lesion is found in conjunction with a highgrade sarcoma. The architectural composition should be discriminating in these cases. Therefore, our aim was to identify genes involved in. Integrated molecular characterization of chondrosarcoma reveals. This means a rare coincidence of factors allowing escape from senescence and apoptosis together with induction of angiogenesis and migration is needed to generate a chondrosarcoma. They usually arise in the thoracic bones, pelvic and long bones.
For dedifferentiated chondrosarcoma, if wide margins cannot be achieved with limb salvage, consideration of amputation is recommended. Patients with the disorder appear to be at increased risk for developing secondary chondrosarcomas. Success depends on the stage of the disease, with lowgrade intracompartmental lesions offering the best prognosis after complete surgical resection with surgically clear margins. Dedifferentiated chondrosarcoma of temporomandibular joint. Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas.
Aims clear cell chondrosarcomas are known to occasionally contain areas. The rare hereditary diseases such as maffuccis syndrome and multiple exostoses hme may also increase the risk of developing chondrosarcoma. Dedifferentiated chondrosarcoma may have a wide range of radiological. Dedifferentiation can be suspected on the basis of the osteolysis with cortical interruption and the pres ence of a soft tissue mass. About 30% of skeletal system cancers are chondrosarcomas. Dedifferentiated cs arising from the pelvis confers an even worse prognosis. Genetic and epigenetic alterations in tumor progression in a dedifferentiated chondrosarcoma.
Chondrosarcoma is mainly seen in adults in the third till sixth decade of life, with equal distribution among genders. Chondrosarcoma accounts for 25% to 40% of these bone tumors. Mutations in suz12 or eed, which encode polycomb repressive complex 2 prc2. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Dedifferentiated chondrosarcoma has a very poor prognosis. It arises most frequently in the 5th and 6th decade of life. Patients with dedifferentiated chondrosarcoma show a 5year overall survival between 7 and 24%. We herein describe a first case of cervical ddcs in an 81yearold male presenting with a slowly growing mass. Chondrosarcomas are malignant cartilageforming bone tumors, which are intrinsically resistant to chemo and radiotherapy, leaving surgical removal as the only curative treatment option. Rarer subtypes of chondrosarcoma include mesenchymal chondrosarcoma and clear cell chondrosarcoma conventional chondrosarcomas may rarely dedifferentiate into a very highgrade tumor with a dismal prognosis so called dedifferentiated chondrosarcoma biopsy biopsy diagnosis is mandatory. Enchondromatosis or olliers disease is another risk factor for the disorder. Chondrosarcoma is the second most common primary sarcoma of bone. Nine cases 4 males, 5 females of dedifferentiated chondrosarcoma were treated in our institute.
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. Sciot departments of radiology and pathology, university hospitals ku leuven, belgium we present a case of a dedifferentiated chondrosarcoma with vertebral and pelvic osseous metastases with dis cussion of the contribution of plain films, ct and mr for. Aug 28, 2012 chondrosarcoma is the second most common primary sarcoma of bone. Figure 6 metastatic mesenchymal chondrosarcoma to adrenal gland. Chondrosarcoma is a rare and malignant cancer which affects the joints and bones. Chondrosarcomas harbor molecular abnormalities, such as overexpression of.
Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. In chondroblastic osteosarcoma, the cartilage cells look malignant and merge into a spindle cell sarcoma. Dedifferentiated chondrosarcoma of the cervical spine. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. Fewer than 10 percent of patients survive longer than one year. 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. Characteristically on plain radiographs, a combined pattern composed of the aggressive parts of dedifferentiated chondrosarcoma components and the less aggressive parts of welldifferentiated chondrosarcoma components suggests dedifferentiated chondrosarcoma. Dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis. Due to lack of effective treatment for advanced disease, the clinical management of metastatic chondrosarcoma is exceptionally challenging. Chondrosarcoma is a rare type of cancer that usually begins in the bones, but can sometimes occur in the soft tissue near bones. Tumors are classified as conventional chondrosarcoma when the histology resembles nonneoplastic cartilage, as opposed to dedifferentiated chondrosarcoma containing areas of high. Chondrosarcoma is a type of sarcoma that affects the bones and joints. The noncartilaginous component may constitute a very small or a very large proportion of.
Conventional chondrosarcoma with focal clear cell change rug. Dedifferentiated chondrosarcoma occurs in approximately 11% of patients diagnosed with chondrosarcoma and is considered to be a histological subtype of this chondroid lesion. Pdf chondrosarcomas are malignant cartilageforming bone tumors, which are intrinsically. We report a very unusual case of a 28yearold female with dcs originating from a cervical spine osteochondroma. It is characterized by two distinct histopathological components.
Dedifferentiated chondrosarcoma ddcs is a type of cartilaginous tumor that is comprised of two distinct components. He is alive without evidence of disease at last contact 91 mo. 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 in forearm. Chondrosarcoma symptoms, prognosis, treatment, survival rate. In dedifferentiated chondrosarcoma the percentage of dedifferentiated component. Radiographs showed an expansion of the cortical contour of the. Dedifferentiated chondrosarcoma portnotes orthopaedicsone. Extraskeletal chondrosarcoma radiology reference article. Approximately one third are extraskeletal and affect the soft tissues of the orbit, cranial and spinal meninges, and lower limbs. Do you know the risk factors, diagnostic strategies, and treatment approaches for these cancers.
Chondrosarcoma mesenchymal and extraskeletal myxoid symptoms, treatment, prognosis, survival rate. Complete, wide surgical excision of the chondrosarcoma is the preferred method when it is feasible. Figure 4 clear cell chondrosarcoma lining wall of hemorrhagic abc cystlike space 400. The esmopaedcaneuracan guidelines are in agreement with nccn regarding chemotherapy regimens for mesenchymal chondrosarcoma, and they also note the following 22. Chondrosarcoma chs is a malignant cartilageforming tumor and usually occurs within the medullary canal of long bones and pelvic bones.
Among 356 mayo clinic bone tumors of the hands and feet, 143 40. Dedifferentiated chondrosarcoma dcs is a biphasic tumor, comprising a lowgrade chondrosarcoma juxtaposed to an anaplastic sarcoma with a high degree of malignancy. Dedifferentiated chondrosarcoma is a rare bone sarcoma, whose genetic background remains incompletely understood. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas. Dedifferentiated chondrosarcoma was first described in 1971 by dahlin and beabout. A case of osteosarcoma arising in a solitary osteochondroma. Dedifferentiated chondrosarcoma is a rare malignancy with reported 5. For example, dedifferentiated chondrosarcoma is often treated like osteosarcoma, with chemo followed by surgery and then more chemo. We suggest that this tumor might be categorized in the group of lowgrade dedifferentiated chondrosarcomas. Although the pathologic appearance varies with speci. Radiographs showed an expansion of the cortical contour of the c2 lamina and a soft tissue mass with. Bone tumors in general are uncommon, with 5,000 to 6,000 cases diagnosed each year, which is about 0.
The fourth and most serious grade is called dedifferentiated chondrosarcoma. Mesenchymal chondrosarcoma mc is an uncommon cartilaginous tumor that primarily occurs in adolescents and young adults, accounting for about 1% to 2% of all chondrosarcomas. Characteristically on plain radiographs, a combined pattern composed of the aggressive parts of dedifferentiated chondrosarcoma components and the less aggressive parts of welldifferentiated chondrosarcoma components suggests dedifferentiated chondrosarcoma 8, 9. The conventional intramedullary chondrosarcoma is the most frequent type, and it most commonly involves the long bones or pelvis in up to 65% of cases. Treating specific bone cancers american cancer society. In the context of highgrade cartilaginous tumors, the exceptionally aggressive dedifferentiated chondrosarcoma subtype may also be. A new softtissue component or rapid growth of the mass can be indicative of a diagnosis of dedifferentiated chondrosarcoma. The survival rate for dedifferentiated chondrosarcoma is the poorest. Osteosarcoma is the most common type of associated highgrade malignancy.
Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. Mesenchymal chondrosarcoma an overview sciencedirect. 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. Conventional chondrosarcoma with focal clear cell change. Dedifferentiated chondrosacromas start out as typical chondrosarcomas but then some parts of the tumor change into cells like those of an osteosarcoma or fibrosarcoma. Integrated molecular characterization of chondrosarcoma. Approximately 720% of lowgrade chondrosarcomas can be expected to dedifferentiate 15. Chondrosarcoma of small bones of the hands and feet. Case reports 373 clinical radiology 1996 51, 373376 case report. Also, in dedifferentiated chondrosarcoma the dedifferentiated. Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a highgrade sarcoma coexists with a lowgrade chondroid tumor. The tumor location was the long bone in 7 cases femur, n5. Dedifferentiated chondrosarcoma is a highly malignant variant of chondrosarcoma. Dedifferentiated chondrosarcomas of the larynx are extremely rare.
Dedifferentiated chondrosarcoma is defined as a high grade, nonchondroid sarcoma associated with a low grade cartilaginous tumor. Education exhibit 97 radiographic, ct, and mr imaging features of dedifferentiated chondrosarcomas. Prognostic factors in dedifferentiated chondrosarcoma. Chondrosarcoma is a form of cancer that results from the abnormal cellular. They make up approximately 11% of all chondrosarcomas, and are among the most aggressive cancers described. Chondrosarcoma is the most common primary bone cancer in adulthood, and the second most common primary bone cancer overall. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. B ra diograph of a dedifferentiated cen tral chondrosarcoma. A the appearance of the lesion is almost the same as that of central chondrosarcoma. The two components are juxtaposed with abrupt clear demarcation line. Dc, a rare entity of chondrosarcoma, has an estimated incidence of 814% of all. Know the grades, types, causes, symptoms, treatment and prognosis of chondrosarcoma. Pdf dedifferentiated chondrosarcoma of the cervical spine.
It develops from normal cartilage which goes through malignant change or can form within a preexisting benign tumor. Chondrosarcoma is a collective term that encompasses a group of heterogeneous lesions with diverse morphologic features and clinical behaviors. Dedifferentiated chondrosarcoma of the maxilla munshi a, atri. Chondrosarcoma is a collective term for a group of tumors that consist predominantly of cartilage and that range from lowgrade tumors with low metastatic potential to highgrade, aggressive tumors characterized by early metastasis. Feb 02, 20 dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis.
The specimen obtained from fine needle aspiration was suggestive of a malignant fibrous histiocytoma. Dedifferentiated chondrosarcoma has to be differentiated from. From a pathologists perspective, multiple sections of a lowgrade chondrosarcoma should be seen to avoid overlooking an anaplastic zone. Several case series of dedifferentiated chondrosarcoma have included mixed reporting of central and peripheral tumors. Dedifferentiated chondrosarcoma of the cervical spine in a. Dedifferentiated chondrosarcoma mimicking a giant cell tumor. Dec 10, 2012 dedifferentiated chondrosarcomas consist of two distinguishable components. Furthermore, dedifferentiated chondrosarcoma is a relatively rare high grade sarcoma next to a lowgrade conventional malignant cartilageforming tumor, comprising 610% of all chondrosarcomas. Chondrosarcoma is a rare cancer that most often forms in the bone, but can also very rarely appear in the soft tissue. Dec 04, 2018 chondrosarcoma is a tumor of mesenchymal origin that predominantly is made of cartilage. Final diagnosis dedifferentiated chondrosarcomas upmc.
Dedifferentiated chondrosarcoma in patients with multiple. Chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. Chondrosarcoma is the second most common primary cancer of the bone. Figure 5 mesenchymal chondrosarcoma of left pelvis, surrounding acetabulum with extensive soft tissue component. Discrete accounts of radiologic features for peripheral dedifferentiated chondrosarcoma include few case reportsandlimitedcaseseries. Because of its rarity, there are few large studies of outcome which might identify potential prognostic factors. These tumors generally occur in older patients, typically over the age of 50. Chondrosarcoma is a rare malignancy characterized by the production of cartilage matrix, displaying heterogeneous histopathology and clinical behavior. The high grade sarcoma is most commonly an mfh, osteosarcoma or fibrosarcoma although others may occur. The prognosis of patients with dedifferentiated chondrosarcoma can be improved by an accurate preoperative diagnosis.
Chondrosarcoma is a locally aggressive to malignant cartilaginous matrix producing neoplasm. Primary ddcs of the mobile spine is extremely rare, particularly in the cervical spine. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Chondrosarcoma hxbenefit recent health articles and news. A rare occurrence in the distal phalanx of the fourth digit. In particular there remains uncertainty about the value of chemotherapy for this condition. White, md rita kandel, md murali sundaram, md up to 11% of chondrosarcomas may undergo regional.
Multiple osteochondromatosis mos is a familial disorder of autosomal dominant transmission characterized by the development of multiple exostoses and often derangements of epiphyseal cartilage, sometimes resulting in long bone growth retardation. Conventional chondrosarcomas can be categorized according to their location in bone. Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a highgrade nonchondroid spindle sarcoma. People who have chondrosarcoma have a single tumor growth which can vary. Utility of idh12 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone. A gradual merge between both components was observed. Based on the morphologic feature alone, a correct diangosis of chs may be difficult, therefore, correlation of radiological and clinicopathological features is mandatory in the diagnosis of chs. Fine needle aspiration cytology of a metastatic skin nodule diagnosed as a dedifferentiated chondrosarcoma. Approximately 11 per cent of chondrosarcomas can be expected to dedifferentiate into more anaplastic lesions. Prognostic factors and outcome from a european group. Pdf dedifferentiated chondrosarcoma laura campanacci. Figure 7 mesenchymal chondrosarcoma with small round blue cell. Corticaldestructionwasthe firstdescribedharbingerofdedifferentiation17.
Frontiers clinical benefit of pazopanib in a patient. Dec 04, 2018 surgery is the primary treatment for any chondrosarcoma. Diagnostic utility of idh12 mutations to distinguish. Jude is the only national cancer institutedesignated comprehensive cancer center devoted solely to children. After further resection, the final diagnosis was dedifferentiated chondrosarcoma. Highgrade conventional chondrosarcoma and dedifferentiated chondrosarcoma have a poor outcome. People who have chondrosarcoma have a single tumor growth which can vary in size and location when first discovered. Dedifferentiated chondrosarcoma of temporomandibular joint ncbi. Conventional chondrosarcomas can be categorized according to. Patients with mesenchymal chondrosarcomas also get chemo before surgery. In summary, this report highlights a very rare case of a dedifferentiated chondrosarcoma in which the dedifferentiated component of the tumor bears histologic resemblance to a conventional giant cell tumor of bone. The most common locations for chondrosarcoma tumors are in the pelvis, hip and shoulder.
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