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