The specimen obtained from fine needle aspiration was suggestive of a malignant fibrous histiocytoma. 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. Dc, a rare entity of chondrosarcoma, has an estimated incidence of 814% of all. 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. Integrated molecular characterization of chondrosarcoma reveals. Dedifferentiated cs arising from the pelvis confers an even worse prognosis. In the context of highgrade cartilaginous tumors, the exceptionally aggressive dedifferentiated chondrosarcoma subtype may also be. 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. It makes up around 25% of all malignant bone cancer cases. 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.
Chondrosarcoma is the second most common primary cancer of the bone. Conventional chondrosarcoma with focal clear cell change. Dec 10, 2012 dedifferentiated chondrosarcomas consist of two distinguishable components. Dcs of the spine arising from osteochondromas is extremely rare. 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. It arises most frequently in the 5th and 6th decade of life. Chondrosarcoma, a malignant cartilaginous tumor, is the second most common primary malignancy of bone after osteosarcoma, often arising from the pelvis, femur, or humerus. Enchondromatosis or olliers disease is another risk factor for the disorder. The defining characteristic of a chondrosarcoma is that its cells produce cartilage. Prognostic factors in dedifferentiated chondrosarcoma. Treating specific bone cancers american cancer society. Dedifferentiated chondrosarcoma mimicking a giant cell tumor. Complete, wide surgical excision of the chondrosarcoma is the preferred method when it is feasible.
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. The rare hereditary diseases such as maffuccis syndrome and multiple exostoses hme may also increase the risk of developing chondrosarcoma. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t.
Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a highgrade nonchondroid spindle sarcoma. Chondrosarcoma accounts for 25% to 40% of these bone tumors. Pdf dedifferentiated chondrosarcoma laura campanacci. 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 ddcs is a rare and aggressive bone tumor with poor prognosis. 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. Integrated molecular characterization of chondrosarcoma. Tumors are classified as conventional chondrosarcoma when the histology resembles nonneoplastic cartilage, as opposed to dedifferentiated chondrosarcoma containing areas of high.
Education exhibit 97 radiographic, ct, and mr imaging features of dedifferentiated chondrosarcomas. The fourth and most serious grade is called dedifferentiated chondrosarcoma. Because of its rarity, there are few large studies of outcome which might identify potential prognostic factors. The esmopaedcaneuracan guidelines are in agreement with nccn regarding chemotherapy regimens for mesenchymal chondrosarcoma, and they also note the following 22.
Chondrosarcoma is a collective term that encompasses a group of heterogeneous lesions with diverse morphologic features and clinical behaviors. Among 356 mayo clinic bone tumors of the hands and feet, 143 40. In particular there remains uncertainty about the value of chemotherapy for this condition. Chondrosarcoma is mainly seen in adults in the third till sixth decade of life, with equal distribution among genders. For example, dedifferentiated chondrosarcoma is often treated like osteosarcoma, with chemo followed by surgery and then more chemo. Dedifferentiated chondrosarcoma is a highly malignant variant of chondrosarcoma. Also, in dedifferentiated chondrosarcoma the dedifferentiated. Genetic and epigenetic alterations in tumor progression in a dedifferentiated chondrosarcoma. Know the grades, types, causes, symptoms, treatment and prognosis of chondrosarcoma. Dedifferentiated chondrosarcoma of temporomandibular joint ncbi. Dedifferentiated chondrosacromas start out as typical chondrosarcomas but then some parts of the tumor change into cells like those of an osteosarcoma or fibrosarcoma. Dec 04, 2018 chondrosarcoma is a tumor of mesenchymal origin that predominantly is made of cartilage. Dedifferentiated chondrosarcoma dcs is a biphasic tumor, comprising a lowgrade chondrosarcoma juxtaposed to an anaplastic sarcoma with a high degree of malignancy. Therefore, our aim was to identify genes involved in.
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 mesenchymal and extraskeletal myxoid symptoms, treatment, prognosis, survival rate. The noncartilaginous component may constitute a very small or a very large proportion of. For dedifferentiated chondrosarcoma, if wide margins cannot be achieved with limb salvage, consideration of amputation is recommended. We developed and characterized three cell lines, derived. Do you know the risk factors, diagnostic strategies, and treatment approaches for these cancers. Chondrosarcoma is the most common primary bone cancer in adulthood, and the second most common primary bone cancer overall. Chondrosarcoma is a locally aggressive to malignant cartilaginous matrix producing neoplasm. Chondrosarcoma is a type of sarcoma that affects the bones and joints. In dedifferentiated chondrosarcoma, the cartilage is well juxtaposed to it rather than merging into it. Feb 02, 20 dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis. Fine needle aspiration cytology of a metastatic skin nodule diagnosed as a dedifferentiated chondrosarcoma. Dedifferentiated chondrosarcoma is defined as a high grade, nonchondroid sarcoma associated with a low grade cartilaginous tumor. 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.
Mesenchymal chondrosarcoma an overview sciencedirect. Chondrosarcoma is a form of cancer that results from the abnormal cellular. Unlike other primary bone cancers that mainly affect children and adolescents, chondrosarcoma can present at any age. We report a very unusual case of a 28yearold female with dcs originating from a cervical spine osteochondroma.
The high grade sarcoma is most commonly an mfh, osteosarcoma or fibrosarcoma although others may occur. Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and shows an increased growth rate and rapid metastatic spread in comparison with ordinary chondrosarcomas. A gradual merge between both components was observed. The symptoms of this disorder can vary from one patient to another. Osteosarcoma is the most common type of associated highgrade malignancy.
Figure 7 mesenchymal chondrosarcoma with small round blue cell. In chondroblastic osteosarcoma, the cartilage cells look malignant and merge into a spindle cell sarcoma. Dedifferentiated chondrosarcoma was first proposed in 1971 by dahlin and beabout. Chondrosarcomas are most frequently found in men between the ages of 3060 years although there is a slight female predominance for phalangeal chondrosarcomas. The prognosis of patients with dedifferentiated chondrosarcoma can be improved by an accurate preoperative diagnosis. 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. Pdf chondrosarcomas are malignant cartilageforming bone tumors, which are intrinsically.
Diagnostic utility of idh12 mutations to distinguish. 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. The architectural composition should be discriminating in these cases. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas. Dedifferentiated chondrosarcomas cs are a highgrade variant of cs that confers a 5year survival of around 1024%. Dedifferentiated chondrosarcoma may have a wide range of radiological. Dedifferentiated chondrosarcoma is a rare bone sarcoma, whose genetic background remains incompletely understood. The term dedifferentiated chondrosarcoma was first termed by dahlin and beabout in 1971 9,18,22,23. Dec 04, 2018 surgery is the primary treatment for any chondrosarcoma. People who have chondrosarcoma have a single tumor growth which can vary in size and location when first discovered. Dedifferentiated chondrosarcoma of the cervical spine in a. Dedifferentiated chondrosarcoma of the larynx american. It develops from normal cartilage which goes through malignant change or can form within a preexisting benign tumor.
Approximately 11 per cent of chondrosarcomas can be expected to dedifferentiate into more anaplastic lesions. Dedifferentiated chondrosarcoma ddcs is a type of cartilaginous tumor that is comprised of two distinct components. Pdf dedifferentiated chondrosarcoma of the cervical spine. Up to 20% of the tumour consisted of classical features of clear cell chondrosarcoma. 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. Success depends on the stage of the disease, with lowgrade intracompartmental lesions offering the best prognosis after complete surgical resection with surgically clear margins. Figure 4 clear cell chondrosarcoma lining wall of hemorrhagic abc cystlike space 400. Radiographs showed an expansion of the cortical contour of the c2 lamina and a soft tissue mass with. Clinical outcome for patients with dedifferentiated. Primary ddcs of the mobile spine is extremely rare, particularly in the cervical spine. People who have chondrosarcoma have a single tumor growth which can vary.
Dedifferentiated chondrosarcoma has to be differentiated from. Conventional chondrosarcoma with focal clear cell change rug. Experience in the treatment of dedifferentiated chondrosarcoma. Dedifferentiation can be suspected on the basis of the osteolysis with cortical interruption and the pres ence of a soft tissue mass. Chondrosarcomas are malignant cartilageforming bone tumors, which are intrinsically resistant to chemo and radiotherapy, leaving surgical removal as the only curative treatment option. However, in some cases the lesion can occur as an abnormal bony type of bump, which can vary in size and location. Chondrosarcoma is a rare malignancy characterized by the production of cartilage matrix, displaying heterogeneous histopathology and clinical behavior. Dedifferentiated chondrosarcoma portnotes orthopaedicsone. They make up approximately 11% of all chondrosarcomas, and are among the most aggressive cancers described. H3k27me3 deficiency defines a subset of dedifferentiated. Figure 5 mesenchymal chondrosarcoma of left pelvis, surrounding acetabulum with extensive soft tissue component. 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.
Discrete accounts of radiologic features for peripheral dedifferentiated chondrosarcoma include few case reportsandlimitedcaseseries. Figure 6 metastatic mesenchymal chondrosarcoma to adrenal gland. Nine cases 4 males, 5 females of dedifferentiated chondrosarcoma were treated in our institute. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Due to lack of effective treatment for advanced disease, the clinical management of metastatic chondrosarcoma is exceptionally challenging. Although the pathologic appearance varies with speci. 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. Diagnostic utility of idh12 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone. Dedifferentiated chondrosarcoma was first described in 1971 by dahlin and beabout. Chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas.
The most common locations for chondrosarcoma tumors are in the pelvis, hip and shoulder. Aims clear cell chondrosarcomas are known to occasionally contain areas. They usually arise in the thoracic bones, pelvic and long bones. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Approximately one third are extraskeletal and affect the soft tissues of the orbit, cranial and spinal meninges, and lower limbs. Mutations in suz12 or eed, which encode polycomb repressive complex 2 prc2. Highgrade conventional chondrosarcoma and dedifferentiated chondrosarcoma have a poor outcome. Dedifferentiated chondrosarcoma is a rare malignancy with reported 5. Dedifferentiated chondrosarcoma has a very poor prognosis. Chondrosarcoma is the second most common primary sarcoma of bone. Dedifferentiated chondrosarcomas of the larynx are extremely rare. These tumors generally occur in older patients, typically over the age of 50. Dedifferentiated chondrosarcoma occurs in approximately 11% of patients diagnosed with chondrosarcoma and is considered to be a histological subtype of this chondroid lesion.
He is alive without evidence of disease at last contact 91 mo. A total laryngectomy was performed for the dedifferentiated chondrosarcoma. Frontiers clinical benefit of pazopanib in a patient. A rare occurrence in the distal phalanx of the fourth digit. We suggest that this tumor might be categorized in the group of lowgrade dedifferentiated chondrosarcomas. Aug 28, 2012 chondrosarcoma is the second most common primary sarcoma of bone. 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. 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. Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas. Bone tumors in general are uncommon, with 5,000 to 6,000 cases diagnosed each year, which is about 0. Chondrosarcoma chs is a malignant cartilageforming tumor and usually occurs within the medullary canal of long bones and pelvic bones. From a pathologists perspective, multiple sections of a lowgrade chondrosarcoma should be seen to avoid overlooking an anaplastic zone.
Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. Several case series of dedifferentiated chondrosarcoma have included mixed reporting of central and peripheral tumors. Final diagnosis dedifferentiated chondrosarcomas upmc. After further resection, the final diagnosis was dedifferentiated chondrosarcoma. Case reports 373 clinical radiology 1996 51, 373376 case report. A case of osteosarcoma arising in a solitary osteochondroma. The two components are juxtaposed with abrupt clear demarcation line. Patients with dedifferentiated chondrosarcoma show a 5year overall survival between 7 and 24%. 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. The tumor location was the long bone in 7 cases femur, n5. Chondrosarcoma hxbenefit recent health articles and news.
Patients with the disorder appear to be at increased risk for developing secondary chondrosarcomas. Utility of idh12 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone. Approximately 720% of lowgrade chondrosarcomas can be expected to dedifferentiate 15. B ra diograph of a dedifferentiated cen tral chondrosarcoma. A the appearance of the lesion is almost the same as that of central chondrosarcoma. Chondrosarcoma is a rare type of cancer that usually begins in the bones, but can sometimes occur in the soft tissue near bones. Targeting survivin as a potential new treatment for chondrosarcoma. All these things have to be taken into consideration. The diagnosis is made histologically when a lowgrade cartilage lesion is found in conjunction with a highgrade sarcoma. Conventional chondrosarcomas can be categorized according to their location in bone. It is characterized by two distinct histopathological components. Dedifferentiated chondrosarcoma of the maxilla munshi a, atri.
Dedifferentiated chondrosarcoma of temporomandibular joint. Furthermore, dedifferentiated chondrosarcoma is a relatively rare high grade sarcoma next to a lowgrade conventional malignant cartilageforming tumor, comprising 610% of all chondrosarcomas. Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a highgrade sarcoma coexists with a lowgrade chondroid tumor. The survival rate for dedifferentiated chondrosarcoma is the poorest. Chondrosarcoma is a rare cancer that most often forms in the bone, but can also very rarely appear in the soft tissue. Radiographs showed an expansion of the cortical contour of the.
Jude is the only national cancer institutedesignated comprehensive cancer center devoted solely to children. 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. Conventional chondrosarcomas can be categorized according to. About 30% of skeletal system cancers are chondrosarcomas. In dedifferentiated chondrosarcoma the percentage of dedifferentiated component. Chondrosarcomas harbor molecular abnormalities, such as overexpression of. Corticaldestructionwasthe firstdescribedharbingerofdedifferentiation17.
Fewer than 10 percent of patients survive longer than one year. A new softtissue component or rapid growth of the mass can be indicative of a diagnosis of dedifferentiated chondrosarcoma. Extraskeletal chondrosarcoma radiology reference article. B ra diograph of a dedifferentiated cen tral chondrosarcoma in a woman aged 64. White, md rita kandel, md murali sundaram, md up to 11% of chondrosarcomas may undergo regional.
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