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1 515 pissn : , eissn : Original Article J Korean Orthop Assoc 2016; 51: 연부조직에발생한단소성섬유종양의임상결과 공창배 최성우 조상현 송원석 조완형 고재수 * 전대근 원자력병원정형외과, * 병리과 The Clinical Outcome of Soft Tissue Solitary Fibrous Tumor Chang-Bae Kong, M.D., Sung Woo Choi, M.D., Sang-Hyun Cho, M.D., Won-Seok Song, M.D., Wan-Hyeong Cho, M.D., Jae-Soo Koh, M.D.*, and Dae-Geun Jeon, M.D. Departments of Orthopedic Surgery and *Pathology, Korea Cancer Center Hospital, Seoul, Korea Purpose: Solitary fibrous tumors (SFT) represent a rare entity of soft tissue tumors which are exclusively located in the thoracic cavity as pleural fibrous tumors. Extrathoracic SFT in the soft tissues are very rare. We analyzed the oncologic outcome of the soft tissue SFT. Materials and Methods: Between January 2009 and December 2014, we treated 6 soft tissue SFT cases. Patients clinical data, magnetic resonance imagings and pathologic findings were reviewed. Results: The patients included two women and four men. The average age of the patients was 45 years (range, years). The mean tumor size is 7.2 cm (range, cm). One patient received wide excision of the tumor and remaining 5 patients marginal excision. After the excision, pathologic report confirmed that 4 patients showed margin positive. However, no patient received further surgery or adjuvant treatments. After the immunohistochemistry, 2 out of 6 patients identified as malignant soft tissue SFT. The mean follow-up period was 25.8 months (range, 6 66 months). Although 4 patients revealed margin positivity, no subsequent local recurrence or distant metastasis was observed. Conclusion: Since the SFT are categorized as intermediated biological potential with a low risk of metastasis and recurrence, marginal excision can be a one of the surgical options for these tumors which are located adjacent to vital structures. However, close observation is needed because local recurrence can be significantly higher the patients with positive resection margins. Key words: solitary fibrous tumors, soft tissue neoplasms, prognosis, margin 서론 단소성섬유종양은 1931 년 Klemperer 와 Rabin 1) 이흉막에생긴 종양에서처음기술하였으며, 섬유조직을형성하는간엽종양으로 2013년세계보건기구 (World Health Organization, WHO) 연부종양분류에서는드물게전이를일으키는중등도종양으로분류되어있다. 2) 발생률에있어서성별의차이는보이지않으며, 50-70대에많이발생한다고한다. 3) 흉막에가장흔하게발생하는것으로알려져왔으나후복막, 종격동, 음낭등의체강내와안와, Received February 5, 2016 Revised April 28, 2016 Accepted May 29, 2016 Correspondence to: Wan-Hyeong Cho, M.D. Department of Orthopedic Surgery, Korea Cancer Center Hospital, 75 Nowon-ro, Nowon-gu, Seoul 01812, Korea TEL: FAX: chowanda@naver.com 비강및부비동등장막으로피복되지않는부위, 폐, 간, 신장, 부신, 갑상선, 척수등의실질장기내부및이들장기를덮고있는피막에서도드물게발생하며, 연부조직에서의발생도보고된바있다. 4) 흉막외에발생하는단소성섬유종양은전체연부조직종양중 0.6% 로극히드물며, 중등도종양으로재발이나전이를일으킬수있다. 5) 단소성섬유종양은특징적인조직학적소견이없이다양한소견을보이며, 방추성세포를보이는다른연부조직종양과의감별이어려워임상및병리학적소견을숙지하는것이필요하나아직임상병리적자료, 환자처치와예후에대한문헌이적고통일된견해를보이지않는실정이다. 단소성섬유종양환자의예후예측이어렵다는것은많은임상의들과병리의들의고민이다. 이질환은병리학적으로악성도를판정하여악성단소성섬유종양이란진단을별도로구분하고 The Journal of the Korean Orthopaedic Association Volume 51 Number Copyright 2016 by The Korean Orthopaedic Association This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

2 516 Chang-Bae Kong, et al. 있다. 하지만단소성섬유종양의 45% 에이르는환자들이악성으로진단받고있음에도전이는많이발생하지않는특이한경과를나타내어임상의들의예후예측을더어렵게한다. 6) 최근들어국외에서는수십명의단소성섬유종양에대한국소재발및전이를일으키는위험인자에대한임상결과가보고된바있다. 7,8) 하지만국내에서는대부분증례보고로만발표되었고, 환자의치료결과및예후가아직보고된바없다. 이에저자들은원자력병원에서경험한흉막외단소성섬유종양환자들의종양학적결과에대해분석하여보고하고자한다. 대상및방법 본연구는 2009년 1월부터 2014년 12월까지본원에서사지의단소성섬유종양으로진단된이후치료받은 6명의환자를대상으로하였다. 본원에내원하여조직검사를통해단소성섬유종양으로진단된환자를선정기준으로하였다. 희귀한종양의특성상별도의배제기준은설정하지않았고, 추시기간이짧은환자도포함하였다. 이들의임상기록, 자기공명영상, 병리소견을후향적으로분석하였다. 환자의특성과종양의해부학적위치, 크기, 수술적방법, 병리학적절제연과국소재발, 원격전이여부를확인하였다. 조직학적진단은 WHO 기준을따랐으며, 면역조직화학염색소견상 CD34와 bcl-2에양성, CD31, 상피막항원, 케라틴, 데스민등에음성인특징을기준으로진단하였다. 4) 고배율에서높은유사분열을보이는세포가네개이상일때, 비정상적인세포질모양과괴사나핵의다형성이관찰될때악성단소성섬유종양으로진단하였다. 9) 결과 남자가 4예, 여자가 2예였으며, 평균연령은 45세 (32-56세) 였다. 종양의크기는평균 7.2 cm ( cm) 였으며, 발생부위를살펴보면손과배부에발생한 2예를제외한 4예에서전부근막을포함하는깊은위치의연부조직종양이었으며배부, 어깨, 상완부, 손에각 1예, 대퇴부에 2예발생하였다 (Table 1). 환자들은모두만져지는무통성종괴를주소로내원하였고, 종괴인지후평균 27 개월 (1-120개월) 에수술을받았다. 모든환자들은특이처치없이내원하였으며, 자기공명영상에서악성종양을배제할수없어본원에서모두수술전절개생검술을시행받았고, 이중 2예는방추세포종양이라는소견만을얻을수있었다. 한명의환자에서광범위절제술, 다섯명의환자에서변연부절제술을시행하였다 (Fig. 1, 2). 수술후 4예에서현미경적으로절제연에종양세포가있음이확인되었으나추가적인수술및방사선치료, 항암치료는시행하지않았다. 절제술후면역조직화학염색을통한최종적인조직학적진단은단소성섬유종양이 4예, 악성단소성섬유종양이 2예였다. 추시기간은평균 25.8개월 (6-66개월) 이었다. 네명의환자에서수술적절제연에해부학적으로종양세포가관찰되었지만, 국소재발이나원격전이가발생한환자는없었다. 고찰 저자들은본연구를통해절개생검을시행하더라도단소성섬유종양을진단하는것이쉽지않음을확인할수있었다. 이종양의조직학적기원은섬유모세포로생각되며, 대부분의증례들은이전에는혈관주변세포종 (hemangiopericytoma) 으로인식되었을가능성이있다고한다. 5) 이종양은특징적인조직학적소견없이다양한소견을보여혈관주변세포종뿐아니라악성섬유성조직구종등양성및악성의다른연부조직종양과의감별이어렵다고알려져있다. 4) 본연구에서도최종절제술을통해서만 2예의악성단소성섬유종양의진단이확인된것을볼때, 이종양의정확한진단을위해서는절개생검시의충분한조직채취와병리의사의많은경험이필요하다고생각된다. 최근까지국내에서는증례보고로만단소성섬유종양이소개가되었었다. Seo 등 10) 은대퇴부에발생한악성단소성섬유종 1예보고를통해사지에발생한단소성섬유종의경우악성가능성이있으므로광범위절제술이필요하다고하였다. 크기가 10 cm 이상이고조직학적악성소견을보이며절제변연부에종양이존 Table 1. Patients Demographics and Treatment Results Patient No. Gender/ Age (yr) Location Onset (mo) Tumor size (cm) Incisional biopsy Surgery Microscopic margin Final pathology 1 Female/32 Upper arm Spindle cell tumor Excision Negative SFT CDF 17 2 Male/39 Hand Spindle cell tumor Excision Positive SFT CDF 6 3 Male/44 Shoulder SFT Excision Positive Malignant SFT CDF 66 4 Male/52 Thigh SFT Excision Positive Malignant SFT CDF 23 5 Male/56 Back SFT Excision Positive SFT CDF 31 6 Female/51 Thigh SFT Wide excision Negative SFT CDF 12 F/U, follow-up; SFT, solitary fibrous tumor; CDF, continuous disease free. Final status F/U (mo)

517 The Clinical Outcome of Soft Tissue Solitary Fibrous Tumor A C B D Figure 1. A 52-year-old man with left thigh mass (case 4).

(B) Axial T1 weighted enhancement image demonstrates a well-enhancing mass.

(D) Diagnosis was confirmed based on the malignant portion of this tumor.

A 56-year-old man with back mass (case 5).

(B) Sagittal T1 weighted enhancement image demonstrates a well-enhancing lesion. 재할때국소재발과원격전이의가능성이높아진다고하였다.

3 517 The Clinical Outcome of Soft Tissue Solitary Fibrous Tumor A C B D Figure 1. A 52-year-old man with left thigh mass (case 4). (A) Axial T1 weighted image demonstrates a low to intermediate SI tumor with cystic component adjacent to the femoral vessel. (B) Axial T1 weighted enhancement image demonstrates a well-enhancing mass. (C) Pathologic examination of benignlooking area shows patternless growth and hemangiopericytoma-like vessel (H&E, 100). (D) Diagnosis was confirmed based on the malignant portion of this tumor. Photomicrograph reveals increased cellularity, nuclear pleomorphism and increased mitotic count (H&E, 400). A B Figure 2. A 56-year-old man with back mass (case 5). (A) Sagittal T1 weighted image shows a poorly marginated mass in subcutaneous fat layer with skin invasion. (B) Sagittal T1 weighted enhancement image demonstrates a well-enhancing lesion. 재할때국소재발과원격전이의가능성이높아진다고하였다. Kim 등 11) 도증례보고를통해 10 cm 이상의크기가단소성섬유종의악성도를예측하는데가장중요한인자라고하였으며악성가능성과절제이후장기적인추시관찰의중요성에대해강조하였다. 이외에도 Kim 등 12) 은사지에발생한혈관외피세포종 3예보고를통해광범위절제와빠른치료가예후에긍정적인요소가될것이라하였다. 완전한수술적절제가흉막외에발생한단소성섬유종양에대한가장적절한치료로알려져있다. 5) 국소재발이나원격전이 의대부분은 2년이내에발생한다. 하지만 14년이후에재발한예도보고된바있어충분한추시가요구된다. 5) 종양의크기도나쁜예후와관련된인자로알려져있으며, 본연구에서도 10 cm 이상크기종양을보인 2예모두악성으로진단되었다. 13) 수술이외의보조적치료의역할은제한적이다. Bishop 등 14) 은 31 명의환자군에수술적절제와방사선치료를병행한결과국소재발이발생하지않아서, 방사선치료가단소성섬유종양의예후향상에도움이된다고보고하였으며, 일부문헌에서는 Anthracycline 기반의항암치료나 dacarbazine, temozolomide+bevacizumab,

4 518 Chang-Bae Kong, et al. tyrosine kinase inhibitors, sorafenib, pazopanib, imatinib, trabectedin 등의항암치료가효과가있다고보고하였다. 15) 하지만아직방사선이나항암치료효과의증거는아직불충분하며수술적절제가검증된유일한치료법이라고생각된다. 4,5,16) 단소성섬유종양에서절제연양성인경우와병리학적악성도가높은경우국소재발의위험이높다고한다. 8) 국소재발의빈도는보고에따른편차가많아서 van Houdt 등 8) 은 29% 의국소재발률을보고하였으나, Wilky 등 7) 은 16%, Vallat-Decouvelaere 등 9) 과 Gold 등 13) 은 4.3% 와 6.7% 의국소재발률을보고하였다. 증례가희귀하여보고자간의차이가큰것으로판단된다. 본연구에서는국소재발과원격전이가없었으나, 이는평균 25.8개월의짧은추시기간과적은증례수때문인것으로생각된다. 단소성섬유종양의성공적인수술적절제후절제연에서현미경적으로종양세포가관찰되었을경우, 국소재발방지를위해바로재수술을시행하여야할것인지가임상의에게큰고민일것이다. 절제연양성과국소재발의상관관계에대해서, van Houdt 등 8) 은절제연양성인경우국소재발의위험율이 4.8배높아진다고보고하였지만, 비슷한시기에 Wilky 등 7) 은단변량분석을통해통계적유의성이없다고하였다. 이렇게국소재발과절제연양성의상관관계에서서로다른결과를보이는이유로저자들은악성연부종양에서조차절제연양성인환자라고하여전부다국소재발을하지는않는것때문에발생하는통계적오류의가능성을생각하였다. O Donnell 등 17) 은연부조직육종환자중절제연양성인 169명에대한국소재발양상을비교분석한바있으며, 이들중 35명 (20.7%) 의환자에서만국소재발이발생했음을보고한바있다. 특히절제연에종양이관찰되더라도 5년국소재발률은 critical structure-positive margin의경우 14.6%, unexpected positive margin의경우 36.4% 라하여절제연의양상에따라국소재발률에차이가있을수있음을지적하였다. 17) 본연구의경우네명의절제연종양양성환자들중세명은신경, 혈관이나관절, 주요건에종양이가깝거나붙어있었던경우로 critical structure-positive margin으로분류할수있었고, 종양이배부에위치한 1예만이 unexpected positive margin에해당하였다. 비록추시기간이짧기는하지만, 이들모두에서국소재발이관찰되지않았다. 따라서단소성섬유종양환자에서수술시적절한경계를얻었다고판단되는경우현미경적절제연양성인경우라도바로수술을시행하는것보다는재발의위험성이높을수있다는것을환자에게설명해주고, 3개월간격으로추시하면서면밀하게관찰하는것도하나의방법이될수있다고생각된다. 결론 단소성섬유종양은전신에서발생가능한중등도의종양으로적절한진단을위해서는충분한조직채취와면역화학적조직병리 검사가필요하다. 조직학적소견이예후인자로중요하며완전한수술적절제가제일중요한치료법이다. 단소성섬유종양은재발과전이가잘되지않는중등도종양으로신경, 혈관, 뼈와같은주요해부학적구조물에종양이근접한경우이를보존하는수술법도하나의방법이될수있다고생각된다. 하지만절제연양성의결과가나왔을경우환자에게국소재발의위험성이높음을주지시키고면밀히관찰해야한다. CONFLICTS OF INTEREST The authors have nothing to disclose. REFERENCES 1. Klemperer P, Rabin CB. Primary neoplasm of the pleura. A report of five cases. Arch Pathol. 1931;11: Fletcher CD. The evolving classification of soft tissue tumours: an update based on the new 2013 WHO classification. Histopathology. 2014;64: Hyodo R, Komada T, Takada A, et al. Solitary fibrous tumors in the extremities: imaging findings for six patients. Nagoya J Med Sci. 2015;77: Kim HJ, Park MJ, Park HJ, Chung WY, Kim KR, Park KK. Chemopreventive and Anticancer Activities of Allium victorialis var. platyphyllum Extracts. J Cancer Prev. 2014;19: Daigeler A, Lehnhardt M, Langer S, et al. Clinicopathological findings in a case series of extrathoracic solitary fibrous tumors of soft tissues. BMC Surg. 2006;6: Doyle LA, Fletcher CD. Predicting behavior of solitary fibrous tumor: are we getting closer to more accurate risk assessment? Ann Surg Oncol. 2013;20: Wilky BA, Montgomery EA, Guzzetta AA, Ahuja N, Meyer CF. Extrathoracic location and "borderline" histology are associated with recurrence of solitary fibrous tumors after surgical resection. Ann Surg Oncol. 2013;20: van Houdt WJ, Westerveld CM, Vrijenhoek JE, et al. Prognosis of solitary fibrous tumors: a multicenter study. Ann Surg Oncol. 2013;20: Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol. 1998;22: Seo JY, Lee ES, Lee HS, et al. Solitary fibrous tumor that de-

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6 520 pissn : , eissn : Original Article J Korean Orthop Assoc 2016; 51: 연부조직에발생한단소성섬유종양의임상결과 공창배 최성우 조상현 송원석 조완형 고재수 * 전대근 원자력병원정형외과, * 병리과 목적 : 연부조직에발생한단소성섬유종은드문종양으로, 저자들은원자력병원에서경험한연부조직단소성섬유종환자들의종양 학적결과에대해알아보고자하였다. 대상및방법 : 2009 년 1 월부터 2014 년 12 월까지단소성섬유종으로진단된이후치료받은 6 명의환자를대상으로하였다. 이들의 임상기록, 자기공명영상, 병리소견을후향적으로분석하였다. 결과 : 남자가 4 예, 여자가 2 예였으며, 평균연령은 45 세 (32-56 세 ) 였다. 한명의환자에서광범위절제술, 다섯명의환자에서변연 부절제술을시행하였다. 수술후 4예에서현미경적으로절제연에종양세포가있음이확인되었으나추가적인수술및방사선치료, 항암치료는시행하지않았다. 절제술후조직학적진단은단소성섬유종이 4예, 악성단소성섬유종이 2예였다. 추시기간은평균 25.8개월 (6-66개월) 이었으며국소재발이나원격전이가발생한환자는없었다. 결론 : 단소성섬유종은재발과전이가잘되지않는중등도종양으로신경, 혈관, 뼈와같은주요해부학적구조물에종양이근접한경우이를보존하는수술법도하나의방법이될수있다고생각된다. 하지만절제연양성의결과가나왔을경우환자에게국소재발의위험성이높음을주지시키고면밀히관찰해야한다. 색인단어 : 단소성섬유종양, 연부조직종양, 예후, 절제연 접수일 2016 년 2 월 5 일수정일 2016 년 4 월 28 일게재확정일 2016 년 5 월 29 일책임저자조완형 01812, 서울시노원구노원로 75, 원자력병원정형외과 TEL , FAX , chowanda@naver.com 대한정형외과학회지 : 제 51권제 6호 2016 Copyright 2016 by The Korean Orthopaedic Association This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

김범수

김범수 Analysis of Outcomes after Resection of Sarcomatous Hepatocellular Carcinoma Purpose: Sarcomatous hepatocellular carcinoma (HCC) is rare. Therefore, the clinicopathologic characteristics and prognosis