• 대한골관절종양학회지
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• 제5권3호
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• pp.183-189
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• 1999

Ewing's sarcoma is an uncommon malignant neoplasm of the long bone and it has a poor prognosis due to its early metastasis and aggressive local spread. It is mostly found before the age of 30 and it is rare in extraskeletal sites. Extraskeletal Ewing's sarcoma has been reported to occur in various sites including the larynx, scalp, nasal fossa, neck, chest wall, lung, pelvis, perineum, arm, finger, leg and toe, but it is extremely rare as a primary epidural tumor of the spine. We experienced a case of extraosseous epidural Ewing's sarcoma arising in the lumbar spinal canal at L3-L5 level in a 9-year-old boy. Following total laminectomy from L3 to L5 with a lumbar vertebrae and mass excision, he received chemotherapy with complete remission.

• Asian Pacific Journal of Cancer Prevention
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• 제15권12호
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• pp.4861-4864
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• 2014

Background: The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors. Materials and Methods: All patients with documented Ewing's sarcoma/primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated. Results: Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated, only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival. Conclusions: Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.

• 대한골관절종양학회지
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• 제2권1호
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• pp.38-46
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• 1996

The traditional methodology in Ewing's sarcoma was chemotherapy and radiotherapy. Recently surgery is reemerging as an important therapeutic tool and some paper report increased survival with it. The purpose of this nonrandomized study is to evaluate our result of Ewing's sarcoma with surgery, retrospectively. We experienced 30 cases for seven years and among them 6 were extraskeletal. In location, axial was 10 cases and peripheral was 20. By Enneking's classification, state IIB was 26 cases and IIIB was 4. Eighteen patients took operation, chemotherapy and/or radiotherapy, and remaining twelve took chemotherapy and/or radiotherapy only. Type of operation was limb salvage in 16 cases and amputation 2. Average dosage of radiation was 45.1Gy. Six kinds of chemotherapeutic regimen were used, but among them main protocols were Ifosfamide-Adriamycin(17 cases) and IESS(Cytoxan, Adriamycin, Methotrexate, Vincristine:8 cases). Complications were as follows. In operation group, there were 3 local recurrence and one case of nonunion. In nonoperated group, one local recurrence and one pancytopenia resulting in death. Average follow up was 29.7 months. Kaplan-Meier's ten year actuarial survival rate for the whole 30 cases was 26.8%. Significant difference in survival exists between central and peripheral lesions(p=0.05, by log rank test). Types of chemotherapy and surgery itself showed no significance. But surgery is important in function and local control. More intensive chemotherapeutic regimen to prevent distant metastasis and combined surgery and radiotherapy may be needed in Ewing's sarcoma.

• 대한골관절종양학회지
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• 제6권4호
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• pp.143-151
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• 2000

목적 : p53 종양억제 유전자는 사람의 암에서 변형이 가장 많이 발견되는 유전자로 유잉 육종에서 p53 유전자의 변형을 관찰하고자 하였다. 재료 및 방법 : 유잉육종 환자 35례의 파라핀 블록을 사용하였으며, 유전자의 결핍과 p53 유전자의 염기서열의 변형을 관찰하였다. 결과 : 정성적인 중합효소 연쇄반응을 이용한 p53의 4-9번까지의 유전자검사중 2례에서 동일성의 유전자 결핍이 관찰되었으며, exon 5-8의 유전자 중합효소 연쇄반응에서는 3례에서 missense 점돌연변이가 관찰되었다. 결론 : 이상의 결과로 p53은 유전적으로 적은 부분에서 유잉육종에 관여하는 것으로 관찰 되었다.

• Journal of Yeungnam Medical Science
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• 제21권2호
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• pp.256-261
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• 2004

유잉육종은 소아와 청소년기에 발생하는 악성 골종양으로 주로 긴 뼈의 골간에 발생한다. 손과 발에 발생하는 유잉육종은 매우 드물다. 저자들은 왼쪽 발꿈치뼈에 발생한 유잉육종 1예를 경험하였기에 문헌 고찰과 함께 보고한다. 15세 여자가 왼쪽 발꿈치의 동통과 부종을 을 주소로 내원하였다. 방사선 소견에서 왼쪽 발꿈치뼈에 골용해성 및 골경화성 병변이 관찰되었다 수술 전 시행한 생검에서 종양세포들은 균일한 둥근 모양의 종양세포로 구성되어있었다. CD99에 대한 면역조직화학염색에서 대부분의 종양세포의 세포질이 강한 양성반응을 보였다. 유잉육종으로 진단을 하고 화학요법을 한 후 무릎밑절단을 시행하였다.

• 대한골관절종양학회지
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• 제17권2호
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• pp.95-99
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• 2011

악성 연부조직 종양 수술 후 이시성(metachronous)으로 발생하는 악성 골졸양은 매우 드물다. 이시성 종양은 두 경부 또는 복부에 주로 발생하며, 사지에 발생하는 경우는 더욱더 드물다. 저자들은 48세 남자환자에서 근위 대퇴부 악성 지방 육종의 광범위 변연 절제술 후 발생한 근위 경골 유잉육종/원시 신경 외배엽 세포종 1예를 경험하여 문헌 고찰과 함께 보고하는 바이다. 육종 환자들에게서는 이와 같이 이차적으로 악성 종양이 발생할 확률이 높아지기 때문에 추시 기간 동안 잠복 종양(occult tumor)에 대한 검사가 필요하겠다.

• 대한골관절종양학회지
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• 제5권1호
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• pp.63-69
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• 1999

Recently we experienced four cases of soft tissue Ewing sarcoma, developed in the calf muscles in two cases, and one case each in the foot and suprascapular region. We also found that the clinical course in all cases was rather rapid, because of local recurrence and metastatic lesions developing within a few months, followed by the patients' death in about 2 years after the primary tumor was excised. These cases were improperly treated initially as some kind of benign soft tissue tumor. The delay led to missed early accurate diagnosis and was thought to be one of the main causes for the rapid local recurrence and metastasis after tumor excision. The pulmonary metastasis was the most common cause of death.

• Journal of Chest Surgery
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• 제46권4호
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• pp.293-294
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• 2013

Gastropleural fistula (GPF) is a rare condition that can occur as a consequence of prior pulmonary surgery, trauma, or malignancy. Conservative management usually fails, and gastrectomy and even thoracotomy is often required, especially in debilitated patients. We present a patient with GPF who had a history of Ewing's sarcoma. Diagnosis of GPF was confirmed by upper gastrointestinal system endoscopy and radiographic contrast examination, and the patient underwent a laparoscopic wedge resection of the fistula. To our knowledge, this is the first report of a GPF, in the formation of which recurrence of Ewing's sarcoma had played a role and in the treatment of which wedge resection of the fistula was performed. Laparoscopic treatment of GPF may be associated with less morbidity and should be considered as the initial procedure of choice.

• Journal of Chest Surgery
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• 제42권3호
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• pp.404-407
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• 2009

43세 여환이 2달 전부터 간간히 생긴 좌측 흉부의 통증을 주소로 내원하였다. 시행한 흉부 전산화 단층 촬영상 좌측 흉부에 계란 모양의 종괴가 발견되었다. 신경원성 종양과의 감별이 어려워 국소 절제술을 시행하였고 조직 검사상 늑골 막에 생긴 골 외 유잉 육종으로 판명되었다. 양전자방출 단층 촬영 시행후 전이가 없어 재수술로 광범위 국소 절제술 및 흉부 재건술을 시행하였으며 항암 화학 요법으로 치료하였다. 환자는 1년간의 항암 화학 요법 치료를 받고 있으며 국소 재발 없이 건강한 상태이다. 환자의 나이와 종양의 기원이 일반적인 골 외 유잉 육종과 다른 특징이 있었다. 이에 국내에서 드물게 보고된 늑골 막에서 생긴 골 외 유잉 육종 1예를 치험하였기에 고찰과 함께 보고하는 바이다.

• Investigative Magnetic Resonance Imaging
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• 제21권1호
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• pp.51-55
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• 2017

Purpose: Extraosseous Ewing's sarcoma (EOE) of the rectum is extremely rare: only three cases have been reported in the literature and none of these reports described their imaging findings in detail. Herein, we describe the tumor imaging and pathological features in detail. Materials and Methods: We report a case of rectal EOE in a 72-year-old female who received local excision and was provisionally diagnosed with a rectal submucosal spindle cell tumor. We used immunohistochemistry, histopathology, and fluorescence in situ hybridization to characterize the tumor and provide a definitive diagnosis of EOE. Results: MRI revealed a well-demarcated submucosal tumor with heterogeneous enhancement and hemorrhagic foci in rectum. EOE was diagnosed by positive staining of tumor cells for CD99 and Fli-1 by immunohistochemistry and the presence of the EWSR1 gene translocation by fluorescence in situ hybridization. Although the patient underwent radiation treatment and surgery, the tumor recurred after 4 months as revealed by computed tomography and magnetic resonance imaging. Conclusion: Rectal EOE may present as a rectal submucosal tumor. The understanding of imaging and histological characteristics of this tumor are critical for accurate diagnosis and appropriate aggressive treatment.