• 제목/요약/키워드: Ewing's Sarcoma

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Clinical study of benign and malignant fibrous-osseous lesions of the jaws

  • Lee, Ju-Min;Song, Won-Wook;Lee, Jae-Yeoul;Hwang, Dae-Seok;Kim, Yong-Deok;Shin, Sang-Hun;Chung, In-Kyo;Kim, Uk-Kyu
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제38권1호
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    • pp.29-37
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    • 2012
  • Introduction: Fibrous-osseous lesions of the jaws are difficult to diagnose precisely until excised biopsy results are found, so they might be confused with malignant lesions. This clinical study focused on the diagnostic aids of lesions that demonstrate different clinical, radiologic, and histological findings. Materials and Methods: A total of 16 patients with benign fibrous-osseous lesions on the jaws (6 fibrous dysplasias, 6 ossifying fibromas, 3 cemental dysplasias, and one osteoblastoma) were reviewed. Nine patients with malignant fibrous-osseous lesions (8 osteosarcomas and one Ewing's sarcoma) were also retrospectively reviewed. Results: Osteosarcoma patients complained of facial swelling and tooth mobility. The radiographic findings showed the irregular resorption of cortical bone and periosteal reactions. Histological features included cellular pleomorphism and atypical mitosis. An Ewing's sarcoma patient complained of tooth mobility and facial swelling. Onion-skin appearance and irregular expansile marginal bony radiolucency were seen in the radiography. Fibrous dysplasia patients complained of facial swelling and asymmetry. The radiographic features were mostly ground-glass radiopacity. Histological findings showed a bony trabeculae pattern surrounded by fibrous ground substances. Ossifying fibroma patients complained of buccal swelling and jaw pains, showing expanded cortical radiolucent lesions with a radiopaque margin. Histological findings were revealed as cellular fibrous stroma with immature woven bones. In cemental dysplasia, most of their lesions were found in a routine dental exam. Well-circumscribed radiopaque lesions were observed in the radiography, and cementum-like ossicles with fibrous stroma were seen in the microscopy. An osteoblastoma patient complained of jaw pain and facial swelling. Radiographic findings were mottled, dense radiopacity with osteolytic margin. Trabeculae of the osteoid with a vascular network and numerous osteoblasts with woven bone were predominantly found in the microscopy. Conclusion: Our study showed similar results as other studies. We suggest the clinical parameters of diagnosis and treatment for malignant and benign fibrous-osseous lesions of the jaws.

원발성 흉벽종양 (Primary Tumors of the Chest Wall)

  • 마중성;최병우;유회성
    • Journal of Chest Surgery
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    • 제7권1호
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    • pp.61-66
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    • 1974
  • Primary tumors of the chest wall are rare than those of other portions of the body. Soft tissue tumors of the chest wall, though these are benign or malignant, should not be paid special attentions about their management than other soft tissue tumors of the body. Thoracic skeletal tumors, however, have some problems in the treatment because of defect in chest wall leading to herniation of lung and paradoxical movement of thoracic cage. The authors experienced 10 case of primary chest wall tumors at the department of thoracic and cardiovascular surgery, the national medical center, during last 15 years. Five of 10 cases were soft tissue tumors, and they were 2 case of lipoma and each one case of myxosarcoma and leiomyosarcoma. Among 5 bone tumors there no cases of sternal tumor, and their histopathological diagnosis were each one of fibrous dysplasia, giant cell tumor, osteochondroma, Ewing`s sarcoma and osteogenic sarcoma. Wide excision, though it was palliative one in certain case, was performed in 9 cases and only diagnostic incisional biopsy in one case, There were no postoperative deaths during admission to the hospital and all cases were missed during short term follow up after discharge from the hospital.

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Association of Cytotoxic T-lymphocyte Antigen-4 Polymorphisms with Malignant Bone Tumors Risk: A Meta-analysis

  • Zhang, Chao;Hou, Wei-Hua;Ding, Xuan-Xi;Wang, Xiong;Zhao, Hui;Han, Xing-Wen;Wang, Wen-Ji
    • Asian Pacific Journal of Cancer Prevention
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    • 제17권8호
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    • pp.3785-3791
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    • 2016
  • Background: Previous studies have assessed the association between the Cytotoxic T-lymphocyte Antigen-4(CTLA-4) polymorphism with the risk of malignant bone tumor, but the conclusions were inconsistent. We aimed to clarify association of cytotoxic T-lymphocyte antigen-4 polymorphisms with malignant bone tumors risk by performing a meta-analysis. Materials and Methods: The databases including PubMed, EMBase databases and the Cochrane Library were searched to identify the eligible studies prior to January 30 2016. Odds ratio (OR) with 95% confidence interval (95%CI) were used to estimate the strengths of the association between the CTLA-4 polymorphism and the malignant bone tumor risks. The meta-analysis was performed by STATA 12.0. Results: Four individual studies with a total of 1003 cases with malignant bone tumor and 1162 controls were included in our meta-analysis. The results of meta-analysis on those data demonstrated that CTLA-4 +49G>A polymorphism was associated with the risk of Ewing's sarcoma and osteosarcoma strongly (A vs. G: OR=1.36, 95%CI:1.20-1.54, p=0.000; AA+AG vs. GG: OR=1.35, 95%CI:1.14-1.61, p=0.001; AA vs. GG: OR=2.24, 95%CI:1.67-2.99, p=0.000; AA vs. AG+GG: OR=2.00, 95%CI:1.53-2.62, p=0.000), but CTLA-4 -318C/T polymorphism was not associated with the risk of malignant bone tumor (C vs. T: OR=0.76, 95%CI:0.76-1.08, p= 0.262; CC+CT vs. TT: OR=0.70, 95%CI:0.41-1.20, p= 0.198; CC vs. TT: OR=0.69, 95%CI:0.40-1.19, p= 0.183; CC vs. CT+TT: OR=0.92, 95%CI:0.75-1.13, p= 0.419). Subgroup analysis showed that there are significantly positive correlations between CTLA-4 +49G>A polymorphism and increased risks of malignant bone tumors in large size of sample (A vs. G: OR=1.347, 95%CI: 1.172,1.548, p=0.000; AA vs. GG: OR=2.228, 95%CI: 1.608,3.085, p=0.000), Ewing's Sarcoma or Osteosarcoma (A vs. G: OR=1.361, 95%CI: 1.201,1.540, p=0.000; AA vs. GG: OR=2.236, 95%CI: 1.674,2.986, p=0.000), and PCR-RFLP or Sequencing(A vs. G: OR=1.361, 95%CI: 1.201,1.540, p=0.000; AA vs. GG: OR=2.236, 95%CI: 1.674,2.986, p=0.000), but CTLA-4 -318C/T polymorphism was not associated with the risk of malignant bone tumors in diagnosis, genotype method, and sample size (all p>0.05). Conclusions: CTLA-4 +49A/G variant was associated with an increased risk of developing the malignant bone tumors, such as Ewing's sarcoma and osteosarcoma. However, it failed to show the association between CTLA-4 -318C/T polymorphism and the risk of malignant bone tumors. Future large-scale studies remain to be done to confirm our conclusions.

소아악성고형종의 진단에 있어서 chimeric transcript의 유용성 (Usefulness of Chimeric Transcript in the Diagnosis of Pediatric Solid Tumors)

  • 최승훈
    • Advances in pediatric surgery
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    • 제5권1호
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    • pp.45-52
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    • 1999
  • Pediatric solid tumors have many histologic similarity. These tumors contained small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the differentiation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumors. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is a useful tool for rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

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원발성 악성 골종양의 $^{99m}Tc$-MDP 골스캔 소견 (The Findings of $^{99m}Tc$-MDP Bone Scan in Primary Malignant Bone Tumors)

  • 현인영;이경한;이동수;정준기;이명철;고창순;강흥식;이상훈;이한구
    • 대한핵의학회지
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    • 제29권1호
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    • pp.73-78
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    • 1995
  • 본 연구는 원발성 악성 골종양의 감별진단에 대한 $^{99m}Tc$-MDP 골스캔의 임상적인 유용성을 알아보기 위해 시행하였다. 골조직검사로 원발성 악성골종양으로 진단받았고 골조직검사 전에 $^{99m}Tc$-MDP 골스캔을 하였던 골육종 22예, 연골 육종 5예, 유잉 육종 4예의 총 31예의 환자들을 대상으로 각각의 악성골종양의 공통적인 소견을 관찰하여 이를 방사선학적 검사와 비교하였고, 골육종에서는 조직학적인 간질조직의 형성에 따라 골아세포성, 연골아세포성, 섬유아세포성, 모세혈관 확장성으로 분류하여 골스캔소견과 비교하였다. 골육종에서 공통적인 소견은 강한 방사능 섭취와 동반된 내부의 불규칙한 방사능감소와 골윤곽의 심한 변형으로 22예 중 16예에서 나타났고 조직학적 분류에 따른 차이점은 발견할 수 없었다. 연골 육종에서 공통적인 소견은 정상보다는 증가되었지만 천장관절보다 약한 방사능섭취와 내부의 불규칙한 방사능증가, 비교적 잘 유지된 골윤곽으로 5예중 3예에서 관찰되었다. 유잉 육종에서 공통적인 소견은 천장관절보다 높은 방사능 섭취와 내부의 균일한 방사능 분포, 중등도의 골윤곽 변형으로 4예중 3예에서 관찰되었다. 결론적으로 악성골종양 3예에서 각각 특징적 소견을 나타내는 경향이 있고 환자의 임상적 정보와 방사선학적 소견을 참고하면 감별진단에 도움이 되었다. 그러므로 이에 대한 관심을 갖고 판독하는 것이 중요하다고 생각된다.

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폐로 전이한 Wilms 종양의 세침흡인 세포학적 소견 - 2예 보고 - (Fine Needle Aspiration Cytology of Metastatic Wilms' Tumor in the Lung - Report of Two Cases -)

  • 김완섭;김남훈;고영혜;박문향;이중달
    • 대한세포병리학회지
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    • 제7권2호
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    • pp.218-224
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    • 1996
  • We describe two cases of metastatic Wilms' tumor in the lung with emphasis on the cytologic features of specimens obtained by needle aspiration. One of them was extrarenal Wilms' tumor. The findings were correlated with the histopathologic features of the primary lesion. Cellular components in the fine needle aspiration cytology (FNAC) slides included blastemal, epithelial, stromal and inflammatory cells with immature tubular differentiation and rosette formation. Recognition of these cellular components in FNAC smears help in establishing FNAC diagnosis of Wilms' tumor. The blastemal cells were represented by small to medium sized cells with scanty cytoplasm having ill-defined borders and round to slightly oval nuclei with evenly dispersed chromatin and small marginated nucleoli. They were seen in our two cases. The differential diagnosis includes neuroblastoma, malignant lymphoma, malignant rhabdoid tumor, clear cell sarcoma, Ewing's sarcoma and embryonal rhabdomyosarcoma. In conclusion, making a definite cytologic diagnosis of metastatic Wilms' tumor may be possible by light and electron microscopy and immunohistochemical staining. The above findings may contribute to the diagnosis of FNAC of metastatic Wilms' tumor.

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소아 흉벽에 생긴 Askin`s tumor 1례 (Askin Tumor[Peripheral Neuroectodermal Tumor of the Chest Wall] - A Case Report -)

  • 조광조;김영대;정황규
    • Journal of Chest Surgery
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    • 제25권12호
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    • pp.1422-1427
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    • 1992
  • Askin tumor is rare malignant small round cell tumor that orgins from interconstal nerve of chest in children It was not until 1979 that Askin first reported that tumor. Although few sporadic reports had been reported, its incidence were too low to analize its clinical featurs. That tumors prognosis is so grave that no therapy would success to cure, but early diagnosis and enbloc excision with following combind chemotherapy and radiotherapy will prolong their survival. Other small round cell tumors of chest wall that must differentiate are Ewing`s sarcoma, rhabdomyosarcoma, lymphoma, neuroblastoma and pulmonary bla-stoma. The most prominant histologic charactersistics of this tumor is neuron specific eno-lase which is detected with immunohistochemistry technique, and neurosecretary electron dense granules within cytoplasm. We expirienced a case of Askin tumor occuring 12-year-old female who has huge right lower chest mass with dull chest pain. She have been underwent excision and postoperative radiotherapy. We are following her up for months and there is no evidence of local recurrence.

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흉폐부에서 발생한 악성소세포 종양의 방사선치료 (Radiation Treatment for Malignant Small Cell Tumor of the Thoracopulmonary Region (Primitive Pluripotent Histogenesis and Differential Diagnosis - A Case Report and Review of Literatures -))

  • 오원용;양진영;황인순
    • Radiation Oncology Journal
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    • 제9권1호
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    • pp.117-122
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    • 1991
  • 흉폐부 또는 흉벽에서 발생하는 악성소세포 종양군인 Ewings sarcoma, 횡문근육종, Askin tumor, 신경아세포종, PNET, 임파종 등은 현미경학적 소견만으로는 감별하기 어렵다. 그러나 최근에는 조직세포화학적검사, 면역세포화학적검사, 세포배양, 세포유전학적 검사등의 도움으로 상기한 악성소세포 종양군들이 모두가 같은 계통의 primitive pluripotent cells로 부터 분화되어 발병되는 것으로 확인되었다. 치료는 외과적 절제술, 방사선치료, 항암요법 등이 시도되고 있으나 예후는 재발 과원격전이로 인하여 불량한 것으로 보고되고 있다. 본 저자들은 본원에서 치료한 예를 보고하면서 흉폐부에서 발생하는 악성소세포 종양군의 조직발생과 감별진단에 대하여 논하고자 한다.

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Supratentorial Intraparenchymal Haemorrhages during Spine Surgery

  • Leung, Gilberto Ka Kit;Chan, Johnny Ping Hon
    • Journal of Korean Neurosurgical Society
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    • 제55권2호
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    • pp.103-105
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    • 2014
  • Intracranial haemorrhages are rare but potentially life-threatening complications of spine surgery. Most reported cases involved subdural or cerebellar haemorrhages; supratentorial parenchymal bleeding is very uncommon. We report a 28-year-old woman who underwent resection of a thoracic Ewing's sarcoma, and developed fatal haemorrhages around her cerebral metastases during surgery. The clinical presentations, possible pathogenesis and potential preventive measures are discussed. Patients with disseminated metastases within the neural axis are at risks of intracranial complications during spine surgery. The presence of intracranial mass lesions should be considered as a relative contraindication to intradural spine surgery.

상지에 발생한 악성 및 침윤성 양성골종양에 대한 사지 구제술 (Limb Salvage in the Treatment of the Upper Extremity Bone Tumors)

  • 한수봉;신규호;김범수
    • 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.

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