The most common locations for chondrosarcoma tumors are in the pelvis, hip and shoulder. Clinical outcome for patients with dedifferentiated. To further clarify the clinical, radiographic, and pathologic features of this unusual variant, we report three additional cases of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation. Dedifferentiated chondrosarcoma is a highgrade, aggressive anaplastic sarcoma that progresses from a previous low. Dedifferentiated chondrosarcoma occurs in approximately 11% of patients diagnosed with chondrosarcoma and is considered to be a histological subtype of this chondroid lesion. One of the subtypes of chondrosarcoma dedifferentiated chondrosarcoma is a highgrade pleomorphic noncartilaginous sarcoma, arising within a lowgrade chondrosarcoma. The role of chemotherapy with updated outcomes, journal of bone and joint surgery on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
Dedifferentiated chondrosarcoma is a high grade, aggressive anaplastic sarcoma that progresses from a previous low. What about radiation and chemotherapy for chondrosarcoma. They make up approximately 11% of all chondrosarcomas, and are among the most aggressive cancers described. Loewya departments of pathology and medical imaginga, college of medicine, royal university hospital university of saskatchewan, saskatoon, canada summary we describe a 44yearold female with a known history of a solitary osteochondroma of the scapula followed on xray for five years. Chondrosarcoma is the second most common primary cancer of the bone. An essential histological feature of dedifferentiated chondrosarcoma is an abrupt interface between the lowgrade cartilaginous and highgrade mesenchymal components. Pdf background dedifferentiated chondrosarcomas consist of two. Chemotherapy may be taken by mouth in the form of a pill, or it may be put into the body by a needle in a vein or muscle. Low grade chondrosarcoma 10 year survival rate 90% high grade chondrosarcoma 10 year survival rate 2040% recurrence generally have higher grade than primary malignancy bcoz slow growing tumour and recurrence or mets cant be detected years after primary procedure long term follow up with regular imaging of operative site and chest. The diagnosis of dedifferentiated central chondrosarcoma requires a bimorphic histologic pattern with areas of a low. Using recist, the objective response rate was significantly different according to histological subtype, being 31% for mesenchymal chondrosarcoma, 20.
The disease usually starts in the bones of the arms, legs or pelvis. These results suggest a possible benefit of chemotherapy for some patients with dedifferentiated chondrosarcoma. In the surgical group, there were higher rates of diseasefree survival in patients who underwent hindquarter amputation and those who. Because of its rarity, there are few large studies of outcome which might identify potential prognostic factors. Several histological subtypes have been described, including conventional, dedifferentiated, mesenchymal and clearcell chondrosarcoma. Dedifferentiated chondrosarcoma with rhabdomyosarcomatous di. Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. Dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Conclusion the prognosis of dedifferentiated chondrosarcoma is dismal.
Rationale chondrosarcoma is a malignant mesenchymal tumor originating from cartilage. Rare subtypes of chondrosarcoma, including dedifferentiated, mesenchymal, and clear cell chondrosarcoma, are discussed as well. Although the oncologic outcome for patients with pelvic and chest wall chondrosarcoma has been reported, 57 site. 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. Chondrosarcoma accounts for 25% to 40% of these bone tumors. Dec 10, 2012 however, in a previous report, 2 out of patients with dedifferentiated chondrosarcoma experienced a good response to chemotherapy, demonstrating more than 90% necrosis. Final diagnosis dedifferentiated chondrosarcomas upmc. The fourth and most serious grade is called dedifferentiated chondrosarcoma. The high grade sarcoma is most commonly an mfh, osteosarcoma or fibrosarcoma although others may occur. We can even find you a free ride to treatment or a free place to stay when treatment is far from home. Chondrosarcoma is the second most frequent malignant bone tumor that forms cartilage. Dedifferentiated central chondrosarcoma american cancer society. Dedifferentiated central chondrosarcoma staals 2006.
Idh1 and idh2 overexpression sox9 identifies chondrogenic differentiation, particularly useful in high grade areas seen on biopsy without sampled cartilaginous areas p53 overexpression, although not useful in practice. Nine cases 4 males, 5 females of dedifferentiated chondrosarcoma were treated in our institute. Conventional chondrosarcoma is the most common 90% subtype. Pdf chondrosarcoma is a malignant cartilaginous tumor composed of a hyaline cartilage stroma and. Chondrosarcoma can occur at any age but is more common in people older than 40 years. Genomic profiling identifies association of idh1idh2 mutation with. Dedifferentiated chondrosarcoma was first described in 1971 by dahlin and beabout. Most types of chondrosarcoma are resistant to chemo. Extraskeletal chondrosarcoma radiology reference article. As the microscopic findings focally showed a lowgrade chondrosarcoma, the diagnosis of dedifferentiated chondrosarcoma with telangiectatic osteosarcomalike features was made. A dedifferentiated chondrosarcoma is a combined tumor made up to two components which are often sharply demarcated from one another thus raising the possibility of it representing a collision tumor.
Dedifferentiated chondrosarcoma radiology reference. Tcf1 participates in the occurrence of dedifferentiated chondrosarcoma xiaolong xu 0 1 xiaodong tang 0 1 wei guo 0 1 kang yang 0 1 tingting ren 0 1 0 musculoskeletal tumor center, peking university peoples hospital, no. Chondrosarcoma has an indolent natural history, the clinical appearance of it is usually nonspecific. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas. Dedifferentiated definition of dedifferentiated by. Dedifferentiated chondrosarcoma is a highgrade noncartilaginous sarcoma next to a. Still, chemo can be used to treat some special types of chondrosarcoma. Chondrosarcoma treatment, pictures, symptoms, causes. Dec 04, 2018 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. Dickey id, rose ps, fuchs b, wold le, okuno sh, sim fh, scully sp.
Chemotherapy may be taken by mouth in the form of a pill, or it may. Chondrosarcoma is the second most common primary malignant solid tumor of bone, and accounts for approximately 25% of all bone sarcomas bertoni et al. Dedifferentiated chondrosarcoma has a very poor prognosis. In particular there remains uncertainty about the value of chemotherapy for this condition. 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.
Very rarely, if the chondrosarcoma is dedifferentiated, then radiation therapy, or chemotherapy to reduce the tumor size may be followed by surgery to remove the cancer. Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and shows an increased growth rate and rapid metastatic spread in comparison with ordinary chondrosarcomas. Our previous studies have shown that cartilage tumors arising at the surface of bone. Radiograph of the left hip in a patient with histologically proved dedifferentiated chondrosarcoma demonstrates a nonspecific, malignant, purely osteolytic destructive lesion in the periacetabular region with extensive cortical destruction medially arrows and a softtissue mass along the pelvic side wall arrowheads. Genetic and epigenetic alterations in tumor progression in a dedifferentiated chondrosarcoma. Chondrosarcoma is the most common bone sarcoma in adults. Extraskeletal chondrosarcoma is a rare type of chondrosarcomadoes, which forms in the soft tissues, rather than bone cartilage.
Dedifferentiated chondrosarcoma of the cervical spine. For progressionfree survival pfs, pathological fracture and metastasis at. Bone tumors in general are uncommon, with 5,000 to 6,000 cases diagnosed each year, which is about 0. For example, dedifferentiated chondrosarcoma is often treated like osteosarcoma, with chemo followed by surgery and then more chemo. Definition and pathogenesis a conventional chondrosarcoma juxtaposed next to a highgrade noncartilaginous sarcoma two groups of precursor lesions have been identified lowgrade to highgrade malignant chondrosarcoma moderate to highgrade malignant. Dedifferentiated chondrosarcoma with rhabdomyosarcomatous. Pain is a common sign, which worsens as the disease progresses. Referring to a cell or tissue which has acquired a less mature appearance and metabolic profile, an event classically associated with aggressive malignancies. Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas.
The diagnosis is made histologically when a lowgrade cartilage lesion is found in conjunction with a highgrade sarcoma. Dedifferentiated chondrosarcomas consist of two distinguishable components. Chondrosarcoma is the second most common malignant skeletal tumor. Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a highgrade nonchondroid spindle sarcoma. Primary ddcs of the mobile spine is extremely rare, particularly in the cervical spine. Dedifferentiated chondrosarcoma revisited request pdf. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. Dec 14, 2018 dedifferentiated chondrosarcomas cs are a highgrade variant of cs that confers a 5year survival of around 1024%. Metastases developed in 1 of 10 patients with grade 1 chondrosarcoma, in 2 of 10 patients with grade 2 chondrosarcoma, and 2 of 7 patients with grade 3 chondrosarcoma. Conventional chondrosarcoma is the most common form, and the low. Chondrosarcoma is a rare type of cancer that usually begins in the bones, but can sometimes occur in the soft tissue near bones. Chondrosarcoma accounts for 2025% of all bone sarcomas, most commonly involving not only pelvis, but may also the diaphysis regions of long bones. The aim of the present study was to report a case of chondrosarcoma in the scapula patient concerns a 42yearold woman presented with a 3month history of a painful mass in the right. We herein describe a first case of cervical ddcs in an 81yearold male presenting with a slowly growing mass.
Jun 17, 2005 the median time to metastasis was 28 months range, 756 months. Chondrosarcoma is a type of sarcoma that affects the bones and joints. Pathology outlines chondrosarcoma dedifferentiated. Know the grades, types, causes, symptoms, treatment and prognosis of chondrosarcoma. We further aimed at identifying potential improvements to. Chemotherapy chemo is not often used to treat chondrosarcoma, because it doesnt work. Pdf clinical outcome for patients with dedifferentiated.
Radiographs showed an expansion of the cortical contour of the c2 lamina and a soft tissue mass with. High grade malignant neoplasm composed of a well differentiated cartilaginous tumor, usually low grade conventional chondrosarcoma, with either high grade pleomorphic, undifferentiated pleomorphic ups or spindle cell sarcoma like areas. 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. Treating specific bone cancers american cancer society. Dedifferentiated central chondrosarcoma request pdf. Chondrosarcoma is a rare and malignant cancer which affects the joints and bones. As such, surgical resection has been the cornerstone of treatment for over 50 years. Radiographic, ct, and mr imaging features of dedifferentiated. Chondrosarcoma frequently involves the flat bones, including the pelvis, ribs, sternum, and scapula. Osteosarcoma is the most common type of associated highgrade malignancy. Radiograph of the left hip in a patient with histologically proved dedifferentiated chondrosarcoma demonstrates a nonspecific. The current study examines the influence of advancement. There are many confusing issues concerning the imaging feature that can facilitate early diagnosis and the factors that might be related to outcomes.
Chondrosarcoma of the scapula pant 2005 cancer wiley. Data from 41 patients with dedifferentiated chondrosarcoma diagnosed and treated at the university of texas md anderson cancer center from 1986 to 2010 were analyzed for demographics, treatments. The pelvis, ribs, femur, and humerus are the most frequently affected sites, and scapula involvement is relatively rare. Pdf dedifferentiated chondrosarcoma laura campanacci. Dedifferentiated chondrosarcomas cs are a highgrade variant of cs that confers a 5year survival of around 1024%. Dedifferentiated chondrosarcomas ddcss are highly malignant tumors with a. Radiographs showed an expansion of the cortical contour of the c2 lamina and a soft tissue. Patients with mesenchymal chondrosarcomas also get. Dedifferentiated chondrosarcoma with telangiectatic. The tumor location was the long bone in 7 cases femur, n5. Tcf1 participates in the occurrence of dedifferentiated. Dedifferentiated chondrosacromas start out as typical chondrosarcomas but then some parts of the tumor change into cells like those of an osteosarcoma or fibrosarcoma. Dedifferentiated cs arising from the pelvis confers an even worse prognosis. Nov 25, 2011 the purpose of this longterm retrospective study was to examine a group of patients with primary central chondrosarcoma of bone who presented with localized disease and were treated with a uniform surgical protocol at our institution, in order to determine the factors that influence overall and event free survival.
The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. The symptoms associated with chondrosarcoma can vary from person to person depending on where their tumor is located and the size of the tumor. Dedifferentiated definition of dedifferentiated by medical. It arises most frequently in the 5th and 6th decade of life. Dedifferentiated chondrosarcoma is defined as a nonchondrogenic highgrade sarcoma associated with welldifferentiated chondrosarcoma with abrupt transition 1. The clinical approach towards chondrosarcoma the oncologist. From previous studies, we know that the overall survival depends on the histological subtype of chondrosarcoma 10, 12.
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. Dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis. Currently, surgical resection with diseasefree margins and limb salvage remain a preferred treatment for dedifferentiated chondrosarcoma18. Prognostic factors in dedifferentiated chondrosarcoma. Chondrosarcoma cs consists of a heterogeneous group of tumors that share the common feature of cartilage matrix production. Mesenchymal chondrosarcoma is aggressive variant of chondrosarcoma, which tend to occur in younger people. Dedifferentiated chondrosarcoma is defined as a high grade, nonchondroid sarcoma associated with a low grade cartilaginous tumor. Background dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a poor survival. It is largely considered to be resistant to conventional chemotherapy and radiotherapy healey and lane 1986, campanacci 1999, gelderbloom et al. H3k27me3 deficiency defines a subset of dedifferentiated.
The median survival time for the dedifferentiated chondrosarcoma patients is 7. A report of the clinicopathological features and treatment of seventyeight cases. Dedifferentiated chondrosarcoma portnotes orthopaedicsone. The two components are juxtaposed with abrupt clear demarcation line. However, in a previous report, 2 out of patients with dedifferentiated chondrosarcoma experienced a good response to chemotherapy, demonstrating more than 90% necrosis.
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