Figure 7 mesenchymal chondrosarcoma with small round blue cell. The tumor location was the long bone in 7 cases femur, n5. Two cases of dedifferentiated chondrosarcoma, one primary and one recurrent, demonstrated muscle differentiation when studied with monoclonal antibodies to muscle specific actin, desmin and myoglobin. Novel therapeutics in chondrosarcoma tom weiwu chen, md department of oncology, national taiwan university hospital 5th singapore sarcoma symposium oct 7th, 2017 photo by dr.
Dedifferentiated chondrosarcoma of the cervical spine. Tumors are classified as conventional chondrosarcoma when the histology resembles nonneoplastic cartilage, as opposed to dedifferentiated chondrosarcoma containing areas of high. Isocitrate dehydrogenase 1 idh1 and idh2 mutations have been recently described in low. Evaluation of different treatment and management options for. Primary chondrosarcomas arise from a small collection of cartilage cells.
Biomarkers of chondrosarcoma journal of clinical pathology. Thom h, baumal r 1983 role of antibody to s100 protein in diagnostic pathology. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. These tumors can be ditinguished by using tissue patterns derived from biologic nature. A, dedifferentiated chondrosarcoma with a sharp interface between conventional 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. Dedifferentiated liposarcoma displays the greatest histomorphologic diversity, including a subset with myofibroblastic differentiation that shares similarities with a spectrum of reactive, benign, and malignant soft tissue lesions. Chondrosarcoma is the second most common nonlymphoproliferative tumor of the spine. Subtypes of chss, such as dedifferentiated, mesenchymal, and clear cell. Histopathological grading is more related to tumour aggressiveness and disease prognosis.
Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. Dedifferentiated chondrosarcoma, although typically arising from conventional chondrosarcoma. White, md rita kandel, md murali sundaram, md up to 11% of chondrosarcomas may undergo regional anaplastic. Chondrosarcoma chs is a malignant cartilageforming tumor and usually occurs. Site axial skeleton more aggressive, medullary cavity of femur, humerus, ribs and rarely digits. Dedifferentiated central chondrosarcoma staals 2006. Figure 5 mesenchymal chondrosarcoma of left pelvis, surrounding acetabulum with extensive soft tissue component. Pdf dedifferentiated chondrosarcoma laura campanacci.
The highgrade areas are poorly differentiated and may have little or no resemblance to cartilage. We herein describe a first case of cervical ddcs in an 81yearold male presenting with a slowly growing mass. Dedifferentiated chondrosarcomas consist of two distinguishable components. Other variants of chondrosarcoma dedifferentiated, mesenchymal and clear cell chondrosarcoma are rare. Final diagnosis dedifferentiated chondrosarcomas upmc. Clinical outcome for patients with dedifferentiated.
Patil medical college, hospital and research centre. A rare site prakash m patil1, surekha u arakeri 1, balasaheb r yelika, anirudh v kushtagi1 1department of pathology, bldeus shri b. They make up approximately 11% of all chondrosarcomas, and are among the most aggressive cancers described. Histopathologic differentiation between enchondroma and. In this particular case, suboptimal surgical treatment of the lowgrade chondrosarcoma, albeit at the patients request, led to recurrence of the lesion. Dedifferentiated chondrosarcoma is a rare lesion where a benign cartilage tumor.
One of the tumours was also positive for cytokeratin, identified by ae1ae3 and cam 5. Chondrosarcomas make up 712% of all spine tumors, and the spine is. Mesenchymal chondrosarcoma an overview sciencedirect. It is characterized by two distinct histopathological components. The conventional intramedullary chondrosarcoma is the most frequent type, and it most commonly involves the long bones or pel vis in up to.
Pleomorphism is not a feature of chondrosarcoma except the dedifferentiated tumors. 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. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. An essential histological feature of dedifferentiated chondrosarcoma is an abrupt interface between the lowgrade cartilaginous and highgrade mesenchymal components. Prognostic factors in dedifferentiated chondrosarcoma. The highgrade areas may have a spindle cell appearance, and may resemble malignant. Chondrosarcoma is a malignant tumor where the tumor cells produce cartilage matrix. A 19 year old female patient presented with chondrosarcoma in left mandibular region near angle of mouth. Dedifferentiated chondrosarcoma is the most malignant form of chondrosarcoma and rare in its occurrence. 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.
Chondrosarcoma, rare malignant tumour of bone formed from cartilage. Approximately one third are extraskeletal and affect the soft tissues of. They are frequently seen in the 4th and 5th decades with a slight male predominance 1. Osteosarcoma is the most common type of associated highgrade malignancy. Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and shows an increased growth rate and rapid metastatic spread in comparison with ordinary chondrosarcomas. It is rare to observe the natural history of a dedifferentiated chondrosarcoma as documented in this report.
Read dedifferentiated chondrosarcoma with muscle and cytokeratin differentiation in the anaplastic component, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Dedifferentiated chondrosarcoma ddchs is an aggressive type of chondrosarcoma that results from high. Lobulation pattern and fibrous tissue formation around the tumor can be an effective and helpful indicator for histopathologic differentiation between enchondroma and welldifferentiated chondrosarcoma. Astorino rn, tesluk h 1985 dedifferentiated chondrosarcoma with a. Mutations in suz12 or eed, which encode polycomb repressive complex 2 prc2. The high grade sarcoma is most commonly an mfh, osteosarcoma or fibrosarcoma although others may occur.
Dedifferentiated chondrosarcoma with telangiectatic. Mesenchymal chondrosarcoma is a rare, highgrade malignancy of bone or soft tissue first described by lichtenstein and bernstein1 in 1959. Histology of dedifferentiated chondrosarcoma with a sharp in. The diagnosis is made histologically when a lowgrade cartilage lesion is found in conjunction with a highgrade sarcoma. Figure 6 metastatic mesenchymal chondrosarcoma to adrenal gland. Subtypes vary substantially chondrosarcoma nos and myxoid chondrosarcoma have a five year survival of 70%, but mesenchymal chondrosarcoma only 50%, and dedifferentiated chondrosarcoma an abysmal 0%. Radiographs showed an expansion of the cortical contour of the c2 lamina and a soft tissue. Case report open access dedifferentiated chondrosarcoma of the cervical spine. Furthermore, dedifferentiated chondrosarcoma is a relatively rare high grade sarcoma next to a lowgrade conventional malignant cartilageforming tumor, comprising 610% of all chondrosarcomas.
Imaging they are located in metaphysis and extend into diaphysis producing r adiolucent defect with scalloped inner cortex. Liposarcoma is divided into myxoid, pleomorphic, welldifferentiated, and dedifferentiated subtypes. The two components are juxtaposed with abrupt clear demarcation line. Chondrosarcoma, a malignant cartilaginous tumor, is the second most common primary malignancy of bone after osteosarcoma, often arising from the pelvis, femur, or humerus. Pathology outlines chondrosarcoma dedifferentiated. The histologic subtypes of chondrosarcoma are uncommon. Figure 4 clear cell chondrosarcoma lining wall of hemorrhagic abc cystlike space 400. H3k27me3 deficiency defines a subset of dedifferentiated. Osteosarcoma is very sensitive to chemotherapy but not dedifferentiated chondrosarcoma.
Dedifferentiated chondrosarcoma occurs in approximately 11% of patients diagnosed with chondrosarcoma and is considered to be a histological subtype of this chondroid lesion. Approximately 720% of lowgrade chondrosarcomas can be expected to dedifferentiate 15. Dedifferentiated chondrosarcoma is a cartilage forming neoplasm with an area of low grade juxtaposed with an area of highgrade sarcoma. Chondrosarcoma is the second most common primary malignant bone tumor, occurring more commonly in. Histologic variants of chondrosarcoma diagnostic histopathology. Genetic and epigenetic alterations in tumor progression in a dedifferentiated chondrosarcoma. Dedifferentiated chondrosarcoma ddcs is a rare and aggressive bone tumor with poor prognosis. Dedifferentiated chondrosarcoma consists of a low grade malignant hyaline cartilage tumor associated with a highgrade nonchondroid spindle sarcoma.
Usually a good prognosis 75% five year survival in one large data set. Conventional chondrosarcomas can be categorized according to their location in bone. It arises most frequently in the 5th and 6th decade of life. Webpathology is a free educational resource with 10308 high quality pathology images of benign and malignant neoplasms and related entities. Despite their rarity, they present with characteristic clinical and histopathologic features distinguishing them from conventional type of chondrosarcoma. Dedifferentiated chondrosarcoma was first described in 1971 by dahlin and beabout. Dedifferentiated chondrosarcoma of bone springerlink. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Diagnostic utility of idh12 mutations to distinguish.
It represents only 2% to 10% of all chondrosarcomas27 and has a frequency of 0. Dedifferentiated chondrosarcoma is a rare bone sarcoma, whose genetic background remains incompletely understood. Primary ddcs of the mobile spine is extremely rare, particularly in the cervical spine. This report presents clinicopathologic, radiographic, and immunohistochemical features of an unusual dedifferen tiated chondrosarcoma with a gastrointestinal stromal tu. 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.
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