• 대한골관절종양학회지
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• 제20권2호
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• pp.89-93
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• 2014

투명세포연골육종은 매우 드문 골종양으로 장골의 골단이나 골간단에 발생한다. 많은 연구에서 대퇴골의 근위부를 가장 흔하게 발생하는 부위로 보고하였고, 상완골의 근위부가 그 다음으로많이 보고되었다. 조직학적으로 이 종류의 종양 세포들은 투명한 세포질과 뚜렷한 세포질막과 함께 중심성으로 위치하는 둥근 핵소체를 가지고 있다. 일반적으로 투명세포연골육종은 고식적인 연골육종과 혼동되지 않는다. 하지만, 방사선학적으로 장골의 간부에 발생한 경우 진단이 어려우며, 이전 문헌을 통하여 단 3례만이 보고된 바 있다. 저자들은 이전에 보고된 바 없는 42세 남자 환자의 경골 간부에서 발생된 투명세포연골육종을 보고하고자 한다.

• BMB Reports
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• 제48권5호
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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]

• 대한두경부종양학회지
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• 제19권1호
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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.

• 대한골관절종양학회지
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• 제11권2호
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• pp.213-218
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• 2005

미분화 연골육종은 연골육종 중 가장 악성도가 높은 종양으로서, 연골육종의 약 10%에서 발생되는 것으로 알려져 있다. 미분화 연골 육종에 의해 발생된 악성종양은 방사선 및 화학요법에 반응을 하지 않아 광범위 종양 절제술만이 근치적 치료법이다. 본 연구는 근위 대퇴골에 발생한 저악성도 연골육종을 보존적으로 수술하고 추시 관찰 중, 골육종으로 미분화한 연골육종 1례를 보고하고자 한다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제34권6호
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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.

• 대한두경부종양학회지
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• 제10권1호
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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.

• 대한골관절종양학회지
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• 제15권2호
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• pp.155-159
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• 2009

투명세포연골육종은 모든 연골육종의 2%를 차지하는 매우 드문 저등급성 종양이다. 주로 긴뼈의 뼈끝에서 발생하며 대퇴골과 상완골의 근위부에서 가장 흔하고, 견갑골을 포함한 납작뼈에는 드물다. 25-50세 사이의 연령에서 호발하며, 20세 이전의 발생은 흔치 않다. 조직학적으로 종양 세포는 소엽상 무리를 지어 관찰되며, 투명하고 풍부한 세포질을 특징적으로 가진다. 저자들은 8세 여아의 견갑골에서 발생된 투명세포연골육종을 보고하고자 한다.

• Journal of Korean Neurosurgical Society
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• 제29권1호
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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.

• 대한두경부종양학회지
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• 제3권1호
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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.

• 대한골관절종양학회지
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• 제1권2호
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• pp.154-163
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• 1995

1986년 3월부터 1993년 12월까지 연세대학교 의과대학 정형외과학 교실에서 상지에 발생한 악성 및 침윤성 양성 골종양에 대하여 사지구제술을 시행하여 다음과 같은 결과를 얻었다. 1. 총 13명의 상지에 발생한 악성 및 침윤성 양성골종양 환자에 대해 사지 구제술을 시술 하였다. 2. 사지 구제 술의 내용은 8 례가 Tikhoff -Linberg 수술, 2 례가 분절절제 및 재접합술, 2 례가 종양삽입물 치환술 그리고 1 례가 분절절제술 및 유리혈관부착 생비골이식술이었다. 3. 13 례중 3 례가 골육종, 4 례가 연골육종, 3 례가 거대세포종, 1 례가 병적 골절을 동반한 유잉육종, 1 례가 연골아세포종, 1 례가 전완부 건 및 근육과 원위요골 및 척골을 동시에 침범한 평활근 육종이었다. 4. 추시기간은 술수 1년에서부터 7년 5개월로 평균 4년 5개월이었다. 5. 총 13명의 환자 중 1 례의 상완골에 발생한 병적 골절을 동반한 유잉 육종의 환자에서 국소재발 및 다발성 골전이가 나타나 수술 후 4년 4개월만에 사망하였고 나머지 12 례의 환자는 국소재발이나 원격전이의 소견은 없었다. 6. 상지에 발생한 악성 및 침윤성 양성 골종양의 치료로 여러방법의 사지구제술은 병의 치료 면이나 기능적인 면에서 만족스런 결과를 가져왔다.