악성 근골격계 종양세서 CD117[c-kit]과 Her-2/neu 유전자 발현

정진규(Jin Gyu Jeong),최익수(Ik Su Choi),노수인(Su In Roh),곽철호(Cheol Ho Kwak),김상은(Sang Eun Kim),장희경(Hee Kyung Chang),김재도(Jae Do Kim) 대한정형외과학회 2004 대한정형외과학회지 Vol.39 No.6

목적: 본 연구는 악성 근골격계 종양에서 c-kit (CD117) 유전자 단백질 발현과 Her-2/neu 발현의 양상을 조사하고, 임상 예후와 치료의 측면에서 발현의 의의를 연구하였다. 대상 및 방법: 파라핀 포매 조직이 보관된 32예의 악성 골종양(골 육종 23예, 연골 육종 9예)과 8예의 활막 육종을 대상으로 후향적 연구를 시행하였다. 유전자의 발현은 면역조직화학 염색방법을 이용하여 조사하였다. 결과: c-kit 발현은 골 육종에서 56.5% (13/23), 연골 육종에서 44.4% (4/9), 활막 육종에서 50.0% (4/8)의 발현율을 보였다. 23예의 골 육종에서 c-kit의 발현은 여자에서(p=0.044), 폐로의 전이가 있는 증례에서(p=0.044), 종양 세포의 악성도가 높을수록(p=0.023) 통계적으로 의미있는 높은 발현율을 보였다. Her-2/neu는 악성 골종양 32예와 활막 육종 8예 모두에서 발현되지 않았다. 결론: 악성 골종양과 활막 육종의 발생에 c-kit 유전자의 발현이 중요한 역할을 할 것으로 추정할 수 있으며, 특히 폐 전이된 골 육종에서 높은 발현율을 보였다. 이로써 이 종양들의 치료에 c-kit 유전자를 겨냥한 tyrosine kinase억제제의 사용을 고려할 수 있겠다. 그러나 Her-2/neu 유전지는 악성 골종양과 활막 육종 발생과 치료에 중요한 역할을 하는 것 같지 않다. Purpose: The aim of this study was to determine the prevalence of c-kit (CD117) and Her-2/neu expressions in malignant bone and soft tissue tumors and to identify a possible predictive role in patients with these malignancies. Materials and Methods: A retrospective study was conducted on 40 archival paraffin-embedded tissue specimens of patients with 32 malignant bone tumors (23 osteosarcomas, 9 chondrosarcomas) and 8 synovial sarcomas. The gene expressions levels were evaluated by immunohistochemistry. Results: C-kit expression was observed in 13 out of 23 osteosarcoma patients (56.5%), 4 out of 9 chon-drosarcoma patients (44.4%) and 4 of 8 synovial sarcoma patients (50.0%). In particular, the relationship between c-kit expression and gender (p=0.044), a pulmonary metastasis (p=0.044), and the tumor cell grade (p=0.023) in osteosarcoma were found to be statistically significant. On the other hand, Her-2/neu expression was not found in any of the 40 cases under study. Conclusion: These results suggest that the c-kit expression appears to play an important role in the development of a malignant bone tumor and synovial sarcoma. In particular, it should be noted that c-kit expression is found frequently in an osteosarcoma with a pulmonary metastasis. In this respect, the tyrosine kinase inhibitors can be considered to be significant in the treatment of a malignant bone tumor and a synovial sarcoma. However, Her-2/neu does not appear to play any significant role in these malignancies.

사지활막육종

문장용(Jang Yong Moon),정소학(So Hak Chung),오선주(Sun Ju Oh) 대한정형외과학회 2016 대한정형외과학회지 Vol.51 No.6

목적: 활막육종은 주로 젊은 연령층에 호발하는 드문 악성 연부조직 종양이다. 이전 연구들은 사지가 아니거나 재발 종양들이 포함되어 있는 복합적인 코호트 군을 대상으로 하였기에 예후 인자들을 분석하는 데 제한이 있었다. 이 연구의 목적은 사지에 국한된 원발성 활막육종의 재발 및 전이 방지와 생존율 증가에 유용한 치료 방법에 대한 독립적인 인자를 결정하는 데 있다. 대상 및 방법: 1999년 4월부터 2014년 6월까지 활막육종으로 광범위 절제술을 시행한 73예를 대상으로 하였다. 분석된 예후인자는 수술과 병행한 보조적인 치료방법이었고 분석에 log-rank test 및 Cox regression model을 이용하였다. 결과: 수술만 시행한 경우(9), 항암약물만 시행한 경우(20), 방사선만 시행한 경우(20), 항암약물과 방사선 치료를 동시에 시행한 경우(24)에 재발은 각각 9, 8, 13, 4예였고, 전이는 각각 9, 5, 12, 2예에서 발생하였다. 항암약물 치료를 시행한 경우 생존율은 높았고, 국소 재발 및 원격 전이가 유의하게 낮았다(p<0.0001). 결론: 활막육종은 수술과 병행하여 항암약물 치료를 시행하는 것이 생존율을 높이고 국소 재발 및 전이를 낮추는 데 도움이 될 수 있다. Purpose: Synovial sarcoma is an uncommon malignant soft tissue tumor mostly prevalent in young adults. Previous studies analyzing the prognostic factors have been limited due to the inclusion of heterogenous cohorts of patients with nonextremity and recurrent tumors. The purpose of this study was to determine the independent prognostic factors for the treatment methods associated with local recurrence and metastasis of primary synovial sarcoma localized to the extremities. Materials and Methods: Between April 1999 and June 2014, a total of 79 patients were diagnosed with synovial sarcoma, of which 73 underwent wide excision at Kosin University Gospel Hospital and were followed-up for 60 months (24–72 months). The analyzed prognostic factors were treatment methods for local recurrence and metastasis during the postoperative follow-up period. The Cox regression model was used for multivariate analysis. Results: For local recurrence according to the treatment methods, 9 recurrences occurred in 9 surgical treatments, 8 in 20 chemotherapy added surgical treatments, 13 in 20 radiotherapy added surgical treatments, and 4 in 24 chemotherapy with radiotherapy added surgical treatments. For metastasis according to the treatment methods, 9, 5, 12, and 2 metastases occurred respectively. Chemotherapy had statistical significance in multivariate analysis (p<0.0001). Conclusion: Adjuvant chemotherapy can be useful for the treatment of synovial sarcoma.

불완전 절제된 무지구근 활막육종에서 광범위 절제술후 비골 이식술과 전외측 대퇴부 유리 피판 이식술 - 증례 보고 -

최병완,김정렬,Choi, Byung-Wan,Kim, Jung-Ryul 대한근골격종양학회 2007 대한골관절종양학회지 Vol.13 No.2

활막 육종은 수부에서는 드물게 발생하며 광범위 절제술을 요한다. 수부의 광범위 절제술을 시행할 때는 충분한 절제연을 얻어야 하지만 기능적인 면을 고려한 재건술이 요구된다. 저자들은 타병원에서 불완전 병소내 절제술후 전원된, 46세 남자의 우측 수지 무지구근 부위 활막육종에 대해 대능형골 및 제1 중수지골을 포함한 광범위 절제술 후, 비골 이식술과 전외측 대퇴 피판 이식술을 시행하여 재건술을 시행하여 우수한 결과를 얻은 증례를 보고하고자 한다. Synovial sarcomas of the hand are rare. It should be treated with wide resection. In the cases of soft tissue sarcomas of the hand, functional reconstruction must be considered. We report 46-year-old male patient with synovial sarcoma of the right thenar muscle which was treated with unplanned intralesional resection at outside hospital, that has been treated with wide resection including trapezium and first metacarapl bone then, reconstructed with nonvascularized fibular graft and anterolateral thigh free flap.

활막육종의 장기적 생존율 및 치료방침

송원석(Won Seok Song),전대근(Dae-Geun Jeon),박종훈(Jong-Hoon Park),조완형(Wan-Hyeong Cho),이수용(Soo-Yong Lee),오정문(Jung-Moon Oh),김진욱(Jin-Wook Kim) 대한정형외과학회 2004 대한정형외과학회지 Vol.39 No.3

목적: 활막육종 환자의 장기적 생존율을 구하고 예후에 영향을 미치는 치료방침을 확립하는데 있다. 대상 및 방법: 1985년 8월부터 2000년 6월까지 본원에서 치료받은 환자 중 초진 시 전이가 없었던 59예를 대상으로 하였으며 평균 추시 기간은 50.9 (8-161)개월이었다. Kaplan-Meier 법으로 장기 생존율을 구하였으며, 분석된 예후인자는 성별, 연령, 병기, 병소의 위치, 절제연, 화학요법, 방사선 치료, 국소 재발, 원격 전이였고 단변수 분석은 log-rank 법을, 다변수 분석은 Cox regression model을 이용하였다. 결과: 259예 전체의 14년 실질/지속적 무병 생존율은 56.4/49.7%였다. 병기 Ⅰ-Ⅱa/Ⅱb-Ⅲ의 생존율은 53.6/43.2%였다(p=0.55). 절제연은 광범위 이상이 44예, 변연부 5예, 병소내 절제가 2예였고, 이 중 국소 재발은 각각 4/1/1예(9/20/50%)였다. 국소 재발한 10예 중 4예에서 원격 전이가 있었다. 무병 생존율에 유의한 예후 인자로 국소 재발과 원격 전이(각각 p<0.0001, p<0.011) 및 절제연(p=0.011)이 단변수 분석 상 유의하였으며, 다변수 분석에서도 같은 결과를 보였다. 결론: 활막육종의 14년 무병 생존율은 49.7%였으며 지속적 무병 생존율을 높이기 위해 원격 전이와 국소 재발을 막는 것이 필수적이며, 조절 가능한 인자는 수술적 절제연 뿐으로 계획된 수술적 접근이 필수적으로 생각된다. Purpose: The object of the current study was to evaluate the long term survival of synovial sarcoma and to find an appropriate treatment strategy with a favorable prognosis. Materials and Methods: Between August 1985 and June 2000, fifty nine synovial sarcoma patients with￢out metastasis at presentation were analyzed and followed for 50.9 (8-161) months. Kaplan-Meier plots were used for survival analysis. Analyzed prognostic factors were sex, age, stage, location, surgical margin, chemotherapy, radiation therapy, local recurrence and metastasis. The log-rank test was used for univariate analysis and the Cox regression model for multivariate analysis. Results: Fourteen-year actual and continuous disease free survivals (ADF/CDF) were 56.4 and 49.7%, respectively. CDF of stage Ⅰ-Ⅱa and Ⅱb-Ⅲ were 53.6 and 43.2%, respectively (p=0.55). For local recurrence according to surgical margin, 4 recurrences occurred in 44 wide margins, 1 recurrence in 6 marginal margins, and 1 recurrence in 2 intralesional margins. Among 10 cases of local recurrence, 4 cases had metastasis. Local recurrence, metastasis, and surgical margin had statistical significance in the univariate (p<0.0001, p<0.0001, p=0.011) and multivariate analysis. Conclusion: Fourteen year CDF survival was 49.7%. The alleviation of local recurrence and metastasis is essential for long term survival. In the current study, the only factor under our control was surgical margin. A well designed surgical approach will reduce the recurrence rate.

초음파에서 양성 연부조직 종양으로 오인된 활막육종

민경대,황석하,김욱중,박상모,이병일,Min, Kyoung-Dae,Hwang, Seok-Ha,Kim, Wook-Joong,Park, Sang-Mo,Lee, Byung-Ill 대한정형외과초음파학회 2012 대한정형외과 초음파학회지 Vol.5 No.1

초음파 검사는 연부조직 종물 평가를 위해서 일차적으로 시행할 수 있는 효과적인 검사로 알려져 있다. 저자들은 좌측원위 대퇴부에 통증을 동반한 표재성 연부조직 종물로 내원한 환자의 초음파 검사에서 양성 종양으로 판단되었으나 수술 후 조직검사에서 활막육종으로 진단된 증례를 경험하여 문헌고찰과 함께 이를 보고하고자 한다. Ultrasonography is an effective first-line examination for soft-tissue masses. We reported a case who presented with superficial soft-tissue mass on the left distal thigh accompanying by pain. On ultrasonography, the lesion was determined as a benign mass. The patient, however, was diagnosed with synovial sarcoma based on the histopathologic result. Herein, we reported the case and reviewed of relevant literature.

하인두에 발생한 활막육종(Synovial Sarcoma) 1예

신호철(Ho Chul Shin),손수길(Su Gil Sohn),김태종(Tae Jong Kim),송달원(Dal Won Song) 대한두경부종양학회 2001 대한두경부 종양학회지 Vol.17 No.2

Synovial sarcoma is commonly found in the extremities of mesenchymal origin, but rare in the head and neck area. Histopathology is diagnostic and it shows a biphasic pattern with two neoplastic elements; spindle cell, sarcoma like stroma and gland like clefts lined by epitheloid cells. Synovial sarcoma is high-grade neoplasm that expresses epithelial as well as supporting features. This paper presents a case of synovial sarcoma of hypopharynx on 25 years old male. He was treated by surgical excision and postoperative chemoradiotherapy. There were no evidences of local recurrence & distant metastasis for 19 months postoperatively.

설기저부에 발생한 활막육종 1예

정필상,이상준,정용원,김세형 대한이비인후과학회 2004 대한이비인후과학회지 두경부외과학 Vol.47 No.12

Synovial sarcoma (SS) is defined as a malignant biphasic tumor of soft tisue of uncertain histogenesis characterized by epithe-lial glands situated within a spindled fibrosarcomatous stroma. SS comprising 5.6- 10% of primary adult soft tisue sarcomas are - 40 years. About 85- 95% of SS arise in the extremities, only 3- 10% arise in the head and neck region and rarely presents in the tongue. Males are affected twice as often as females. The hypopharynx and retropharynx are the most comon sites of involvement in the head and neck. Fewer than 100 cases of SS have been reported in the head and neck area. We experienced a 21-year-old male patient who first complained of oral bleding and dysphagia. Initial disection with tongue base tumor excision and postoperative radiotherapy were performed. We wil introduce this rare case report of SS with a literature review.

비인강암에서 AJCC와 Ho 병기 결정법에 따른 T병기의 비교

이상욱(Sang wook Lee),김귀언(Gwi Eon Kim),서창옥(Chang Ok Suh),이강규(Kang Kyoo Lee),박원(Won Park) 대한두경부종양학회 2002 대한두경부 종양학회지 Vol.18 No.2

Objective: A comparison of American Joint Committee on Cancer (AJCC) 1988 and 1997 nasopharyngeal carcinoma (NPC) classifications was made in terms of patient distribution and efficacy in predicting prognosis. Materials and Methods: Between Jan. 1981 and Dec. 1998, 60 cases of node negative nasopharyngeal carcinoma were retrospectively reviewed. The extent of disease each patients restaged according to the 4th and 5th AJCC system and Ho system, respectively. Results: The overall and disease free 5-year survival rates were 61.1% and 62.6%, respectively. Among T classifications of 4th AJCC, 5th AJCC and Ho staging system were not observed significantly different in disease-free survival rates, respectively. Conclusion: We observed a better patient distribution with AJCC 1997 comparing to AJCC 1988. The new classification also attained better statistical significances among stages in the overall survival and disease free survival rates was needed.

하비갑개의 악성흑색종 치험 1례

맹창환(Chang Hwan Maeng),윤동구(Dong Gu Yun),김춘동(Choon Dong Kim),노영수(Young Soo Rho) 대한두경부종양학회 2002 대한두경부 종양학회지 Vol.18 No.1

Malignant melanoma of the nasal cavity occurs rarely. Malignant melanoma, originated from the upper respiratory tract including nasal cavity, has clinical feature of local recurrence and easily metastasizes to regional or distant lymph nodes, lung, and liver. Malignant melanoma originated from nasal cavity frequently shows tumor cell invasion, ulceration, or infection. Owing to these characteristics, complete surgical excision of the malignant melanoma in nasal cavity is not easy. And also the prognosis of this tumor is not so good because of a high recurrence rate. Recently the authors have recently experienced a case of malignant melanoma originated from the inferior turbinate, which was treated with lateral rhinectomy, total maxillectomy. The defect developed after surgical extirpation was reconstructed with rotational forehead flap.

전경부에서 발생한 활막 육종 2예

이국행,이병철,권영조,정연우 대한이비인후과학회 2006 대한이비인후과학회지 두경부외과학 Vol.49 No.9

Synovial sarcomas is a mesenchymal tumor that usually occurs in the extremities. Fewer than 100 cases of synovial sarcoma havebeen reported in the head and neck lesion. We experienced 2 cases of synovial sarcoma of neck, and so reported with a review ofliterature. (Korean J Otolaryngol 2006;49:952-5)