• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.89-93
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• 2014

Clear cell chondrosarcoma is a very rare malignant bone tumor that shows a strong predilection for the epiphysis or metaphysis of long bones. Many studies have reported that the proximal end of the femur is the most commonly affected site, followed by the proximal end of the humerus. Histopathologically, tumor cells of this type have centrally located round nucleoli with clear cytoplasm and a distinct cytoplasmic membrane. Generally, clear cell chondrosarcomas is not confused with conventional chondrosarcomas. However, when it involves the diaphysis in long bones, diagnosis can be hindered, as only three reports of this exist in the literature. We report herein an unusual case of clear cell chondrosarcoma of the tibial diaphysis in a 42-year-old male.

• BMB Reports
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• v.48 no.5
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• pp.266-270
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• 2015

Over the last decade, comprehensive genome-wide sequencing studies have enabled us to find out unexpected genetic alterations of metabolism in cancer. An example is the identification of arginine missense mutations of isocitrate dehydrogenases-1 and -2 (IDH1/2) in glioma, acute myeloid leukemia (AML), chondrosarcomas, and cholangiocarcinoma. These alterations are closely associated with the production of a new stereospecific metabolite, (R)-2-hydroxyglutarate (R-2HG). A large number of follow-up studies have been performed to address the molecular mechanisms of IDH1/2 mutations underlying how these events contribute to malignant transformation. In the meanwhile, the development of selective mutant IDH1/2 chemical inhibitors is being actively pursued in the scientific community and pharmaceutical industry. The present review article briefly discusses the important findings that highlight the molecular mechanisms of IDH1/2 mutations in cancer and provides a current status for development of selective mutant IDH1/2 chemical inhibitors. [BMB Reports 2015; 48(5): 266-270]

• Korean Journal of Head & Neck Oncology
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• v.19 no.1
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• pp.80-83
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• 2003

Chondrosarcomas are uncommon tumors. Approximately 5-10% are located in the head and neck. Nasal septal chondrosarcoma is exceedingly rare, with an extensive review of the world literature revealing only 50 reported cases. Most occur in middle-aged man. Surgical resection is the definitive treatment of choice. Radiation and chemotherapy are reserved for residual or recurrent diseases and palliation. We present a case of nasal septal chondrosarcoma treated with craniofacial resection and postoperative radiation therapy.

• The Journal of the Korean bone and joint tumor society
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• v.11 no.2
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• pp.213-218
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• 2005

Dedifferentiated chondrosarcoma is a most highly malignant variant of chondrosarcoma and approximately 10% of chondrosarcomas dedifferentiated into more anaplastic lesions. In addition to the surgical treatment, adjuvant chemotherapy or radiation therapy has been used but with little success. Thus, the primary treatment remains wide excision and chemotherapy or radiation therapy can be used for palliation. This article shows the case of a dediffderentiated chondrosarcoma occuring in proximal femur after conservative surgical treatment of a low grade chondrosarcoma.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.34 no.6
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• pp.653-656
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• 2008

Mesenchymal chondrosarcoma is a rare malignant tumor of bone and soft tissue. This aggressive form of chondrosarcoma represents only 3% to 9% of all chondrosarcomas. This neoplasm is characterized by sheets or clusters of undifferentiated spindle or round cells surrounding discrete nodules of well-differentiated cartilage. We experienced a case of mesenchymal chondrosarcoma on mandibular body. Two years ago, the patient had been treated the intrabony cystic lesion on mandiblular left body. At that time, cartilage portion was not detected in the cystic specimen. Two years after cyst enucleation, the recurred large neoplasm in the mandibular left body was noted, and it was diagnosed as 4.5 cm sized mesenchymal chondrosarcoma. The mandibular tumor was widely resected and rigid-plate and cervical musculocutaneous flap were used for reconstruction of resected bone and soft tissues. No complications and recurrence were noted for 6 months postoperatively.

• Korean Journal of Head & Neck Oncology
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• v.10 no.1
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• pp.63-73
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• 1994

Chondrosarcomas are uncommon disease that are noted almostly in the pelvis, sternum, long bone. Chondrosarcoma in the head & neck was very rare. It can occur in nearly every bone in the head and neck. Lesion of the maxilla is most frequent site in head and neck region, but temporomandibular joint is rare site. In chondrosarcoma of temporomandibular joint, its danger lies in its local invasiveness and potential to seed into the meninges. Histologically, the tumor exhibits myxoid feature, which must be differentiated from chordoma and chondroid chordoma. The cranial nerve palsies frequently observed with the tumors are related to the anatomical location. Chondroma is a benign tumor that most frequently found in the hand, foot bone, but can be originated in any cartilagenous area of body. When it occurs in one site, it is named as solitary enchondroma, and when it occurs in multiple site, it is named as multiple enchondromatosis. In the head & neck, it may occur in the nasal cavity, nasopharynx, nasal septum, eustachian tube, palate. But chondroma that occur as neck mass is extremely rare. Recently, the authors experienced a case of chondrosarcoma confirmed pathology affecting 35-years-old female presenting mass in left temporomadibular joint and a case of chondroma confirmed pathology affecting 26-years-old female presenting neck mass, left.

• The Journal of the Korean bone and joint tumor society
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• v.15 no.2
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• pp.155-159
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• 2009

Clear cell chondrosarcoma is a rare, low-grade variant of chondrosarcoma that comprises approximately 2% of all chondrosarcomas. This tumor usually involves the epiphysis and epimetaphysis of long bones, especially the proximal part of the femur or humerus, whereas involvement of the scapula is rare. It occurs at any age, but the peak is third to fifth decade, and is rarely seen in the first and second decades of life. Histologically, tumor cells with abundant clear cytoplasm and benign giant cells are usually found. We report on a case of clear cell chondrosarcoma of the scapula in an 8-year-old girl.

• Journal of Korean Neurosurgical Society
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• v.29 no.1
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• pp.44-50
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• 2000

Objective : A few anecdotal cases of mesenchyme-derived tumors which grow into a cranial cavity have been reported that disclosed a dismal prognosis, due to their critical location, aggressive biological behavior, and high rate of surgical morbidity. The aim of this study is to search clinical factors related to these tumors. Methods : Eight patients who underwent surgical removal of intracranial mesenchymal tumors between January 1993 and December 1997 were studied retrospectively. The tumors included are three chordomas, two chondrosarcomas, two rhabdomyosarcomas, and one hemangiopericytoma. Authors compared clinical features, treatment, and results of our cases with reported cases. The mean follow-up period was 20.5 months. Results : All cases showed nonspecific, location-related clinical findings and arose from sphenopetroclival region. Single stage operation was performed in 4 cases, and skull base approaches in 3 cases. Adjuvant therapies were done in 2 cases. Recurrence was seen in 3 cases(37.5%), and 3 patients died. Interdisciplinary approach with otologic surgeon was done in 2 cases. Conclusion : Recent advancement of refined tactics has made these tumors amenable and provides prolongation of progression-free survival. These are modified skull base approaches, multi-modality treatment options, and inter-disciplinary team approaches. Good results may be expected for these mesenchymal tumors by aggressive resection and adjuvant therapies according to their biological nature.

• Korean Journal of Head & Neck Oncology
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• v.3 no.1
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• pp.97-105
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• 1987

Chondrosarcoma of the head and neck is very rare tumor and this incidence was reported to be about 10% of all chondrosarcomas. Maxilla is the most common site of involvement and mandible, paransal sinus, nasal cavity and base of skull in that order. In general, chondrosarcoma has been known to be radioresistant, however since the Princess Margaret Hospital reported that it was radioresponsive tumor in 1980, the role of radiotherapy has been emphasized in terms of local control, especially in head and neck regions where complete excion is often difficult to achieve. The authors experienced 4 cases of chondrosarcoma of head and neck among all 29 chondrosarcoma patients from 1971 to 1985. The clinical and pathologic feature of this disease, it's treatment and prognosis were reviewed along with the literatures.

• The Journal of the Korean bone and joint tumor society
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• v.1 no.2
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• pp.154-163
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• 1995

With the development of anticancer chemotherapy and improved radiographic imaging studies, limb salvage operation became possible in the treatment of malignant and aggressive benign tumors. High grade sarcomas of the shoulder or the upper extremity can be surgically treated with a forequarter amputation, shoulder disarticulation or limb salvage surgery such as Tikhoff-Linberg procedure, segmental resection and replacement with endoprosthesis, segmental resection and replantation, or segmental resection and free vascularized bone graft. Among them the limb salvage surgery showed not only preservation of the remained upper extremity but also the excellent functional results. When comparing amputation and limb salvage operation while performing anticancer chemotherapy in both cases, 5 year survival rate, local recurrence, and distant metastasis did not show much difference. We studied 13 cases of limb salvage for the malignant and aggressive benign bone tumor of the upper extremity from March 1986 to December 1993 at Severance hospital. The summarized results were as follows. 1. There were 21 cases of malignant bone tumors and 5 cases of benign aggressive ones. 2. Of the 26 cases of malignant and benign aggressive bone tumors, limb salvage procedures such as Tikhoff-Linberg operation(8 cases), endoprosthetic replacement(2 cases), segmental resection and replantation(2 cases), and segmental resection and free vascularized fibular graft(l case) were done in 13 cases. 3. In 13 patient on whom the limb salvage procedure was performed, there were 3 osteosarcomas, 4 chondrosarcomas, 3 giant cell tumors, 1 Ewing's sarcoma, 1 leiomyosarcoma and 1 chondroblastoma. 4. In 13 patients, there was no local recurrence nor distant metastasis except one who had a segmental resection of the entire humerus part including glenoid and then postoperative anticancer chemotherapy for the treatment of the Ewing's sarcoma of the entire shaft of the humerus with pathological fracture. Local recurrence occurred 2 years and 6 months postoperatively in this Ewing's sarcoma patient, so forequarter amputation was performed and the irradiation and the anticancer chemontherapy were performed, but multiple bony metastasis developed and died of the disease 22 months after local recurrence. 5. The patients were followed-up for I year to 7 years and 5 months(average 4 years 5 months). 6. In 8 cases in which Tikhoff-Linberg procedure was performed, the function of the hand was almost normal. 7. Segmental resection and endoprosthetic replacement was performed in 2 cases, and the function of the remained upper extremity was good with no evidence of aseptic loosening or nerve palsy. 8. In 1 case of segmental resection and free vascularized fibular graft for the patient of the chon drosarcoma in the humerus, the function of the shoulder, elbow and hand was nearly normal. 9. In I case of leiomyosarcoma which involved both forearm muscles and bone near wrist joint, segmental resection and replantation was performed, and the patient has useful hand function.