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1 164 pissn : , eissn : Original Article J Korean Orthop Assoc 2019; 54: 역분화연골육종의임상결과 공창배 이승용 송원석 조완형 고재수 * 전대근 원자력병원정형외과, * 병리과 The Clinical Outcome of Dedifferentiated Chondrosarcoma Chang-Bae Kong, M.D., Seung Yong Lee, 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: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. Materials and Methods: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients final status were analyzed. Results: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2 23 months) and 7 months (range, 1 32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. Conclusion: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal. Key words: dedifferentiated chondrosarcoma, pathology, pathologic fracture, prognosis 서론 역분화연골육종은전체연골육종환자중약 10%-15% 가량을 차지하는매우드물고악성도가높은종양으로 1971 년 Dahlin 과 Received March 28, 2018 Revised June 4, 2018 Accepted June 14, 2018 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 ORCID: Beabout 1) 에의해처음기술되었다. 2) 병리학적으로는저악성도의연골육종소견과함께비연골성의고등급육종의양상이같이나타나는특징을보인다. 역분화과정에의해발생했다고생각되는부분은다양한육종의소견을나타내며, 골육종, 악성섬유조직구종, 혈관육종, 섬유육종, 그리고거대세포종등의다양한특징을보일수있다. 3,4) 역분화연골육종은 50대와 60대에서주로발생하며통상적인연골육종과유사하게대퇴골과골반골, 상완골이호발부위이다. 4) 악성도가매우높은것으로알려져있으며평균생존기간은 13개월, 5년생존율은 24% 로보고된바있다. 3,5) 발생빈도가적기때문에 Grimer 등 3) 은유럽에서다기관연구 The Journal of the Korean Orthopaedic Association Volume 54 Number Copyright 2019 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.

2 165 Dedifferentiated Chondrosarcoma 를통해예후인자를분석한논문을발표한바있다. 하지만현재까지국내에서는증례보고로만알려져있다. 6,7) 이에저자들은원자력병원에서경험한역분화연골육종환자의종양학적결과를분석하고자하였다. 대상및방법 2007년부터 1월부터 2016년 12월까지본원에서역분화연골육종으로진단된이후치료받은 11명의환자를대상으로하였다. 이들의나이, 성별, 증상발현기간, 종양의위치및크기, 병기, 자기공명영상, 수술적절제연, 병리소견을후향적으로분석하였다. 병기는 Enneking의분류 8) 에따라구분하였다. 병리학적분석을위해 15년이상의골관절종양병리경험을가진병리과전문의가슬라이드를모두재판독하였다. Frassica 등 2) 의보고에따라원발성, 속발성여부를구분하였으, 원발성역분화연골육종환자의경우수술전조직검사여부를확인하였다. 병리학적으로역분화연골육종의특징인이상성여부를확인하였으며저등급연골육종부위의병리학적등급및고악성도 육종부위의면역조직화학염색 (immunohistochemistry) 을통한병리학적인분류를시행하였다. 속발성역분화연골육종환자의치료시작시점은역분화연골육종으로진단된날을기준으로하였으며대상환자의국소재발및원격전이발생시간, 추시기간및종양학적결과를분석하였다. 결과 남자가 7예, 여자가 4예였으며평균연령은 54세 (33-80세) 였다. 종양의위치는대퇴골이 6예, 골반골이 4예, 중족골이 1예였으며종양의평균크기는 12.7 cm ( cm) 였다. 진단당시증상은 8예에서동통, 2예에서종괴였으며, 나머지 1예는방사선에서우연히발견된골병변이었다. 증상발현기간은평균 4개월 (1-12개월 ) 이었으며, 2예에서병적골절이동반되었다. 병기는 1예에서 IIA기, 9예에서 IIB기, 1예에서 III기였고진단시병적골절이동반된환자는 2명이었으며 8예가원발성, 3예가속발성역분화연골육종이었다 (Table 1). Table 1. Patient Demographics and Staging Case No. Sex/Age (yr) Location Symptom (mo) Initial tumor size (cm)* Stage Previous treatment (mo) Definitive treatment Margin of resection 1 M/59 Proximal femur Pain (1) 15.7 III Wide excision Wide None 2 F/50 Ilium & acetabulum Pain (1) 11.2 IIB Internal hemipelvectomy (type I+II) Wide PCNB Preoperative biopsy 3 F/70 Proximal femur Pain (3) 13.0 IIB Wide excision Wide Incisional biopsy 4 M/52 Pubis & acetabulum Pain (12) 8.2 IIB Internal hemipelvectomy (type II+III) 5 F/80 Proximal femur Pain (2) 10.6 IIA Curettage Wide excision Wide None (curettage) 6 M/46 Femur shaft Pain (2) 15.4 IIB Intercalary resection (12) 7 F/44 Pubis & acetabulum Pain (3) 6.2 IIB Internal hemipelvectomy (type II+III) 8 M/60 Femur shaft Mass (2) 26.1 IIB Intercalary resection 9 M/61 Ilium & acetabulum Incidental 11.9 IIB Internal hemipelvectomy (type I+II) Wide None Wide excision Wide Known chondrosarcoma Marginal Marginal 10 M/46 Proximal femur Pain (6) 15.5 IIB Curettage (8) Wide excision Wide Known chondrosarcoma 11 M/33 1st metatarsal Mass (12) 6.0 IIB Curettage (49) Wide excision Marginal Known chondrosarcoma *Maximum tumor diameter. According to the system of the Musculoskeletal Tumor Society. M, male; F, female; PCNB, percutaneous core needle biopsy. Wide PCNB None PCNB

166 Chang-Bae Kong, et al. 원발성역분화연골육종환자 8예중 1예는방사선적으로저등급의연골육종으로오인되어소파술을시행받은후진단되었다 ( 증례 5).

조직검사결과역분화연골육종으로확인되어바로광범위절제술을시행하였다. 나머지 7예중 4예는조직검사이후최종수술을진행하였다. 경피적침생검술을시행받은 3예중 1예와절개생검을시행받은 1예는수술전조직검사에서역분화연골육종으로진단되었다.

속발성역분화연골육종으로진단된 3예는초기진단이모두조직학적등급 2의연골육종이었다 ( 증례 6, 10, 11). 이환자들의경우연골육종의재발로판단하여수술전조직검사없이바로광범위절제술을시행한후역분화연골육종으로진단되었다 (Fig. 2).

역분화부위의조직학적소견은 5예에서골육종, 6예에서미분화육종 (undifferentiated sarcoma) 이었다 (Table 2). 평균추시기간은 17 개월 (5-56 개월 ) 이었고, 국소재발이 8예, 원격전이가 10예에서발생했다.

An 80-year-old female with left hip pain that developed 2 months ago (case 5).

$Note the oblique fracture line with minimal displacement. The image shows no endosteal scalloping.$ peripheral soft tissue edema. Subtle cortical disruption can be seen at the greater trochanter.

peripheral soft tissue edema. Subtle cortical disruption can be seen at the greater trochanter.

3 166 Chang-Bae Kong, et al. 원발성역분화연골육종환자 8예중 1예는방사선적으로저등급의연골육종으로오인되어소파술을시행받은후진단되었다 ( 증례 5). 이환자는내원당시병적골절을동반한 80세의여자로방사선소견상피질골파괴나골막반응을보이지않고내부에석회화병변을보이고있었다 (Fig. 1). 방사선적으로저등급연골육종일가능성이높고병적골절을동반한고령의환자임을감안하여조직검사를시행하지않았으며소파술및내고정술을시행하였다. 조직검사결과역분화연골육종으로확인되어바로광범위절제술을시행하였다. 나머지 7예중 4예는조직검사이후최종수술을진행하였다. 경피적침생검술을시행받은 3예중 1예와절개생검을시행받은 1예는수술전조직검사에서역분화연골육종으로진단되었다. 경피적침생검술을시행받은 3예중나머지 2예는중심성연골육종진단하에최종수술을받았다. 수술전조직검사를받지않은 3예는수술전영상검사에서피질골파괴및연부조직종괴를동반하고있어고등급육종으로판단하고최종수술을진행하였다. 속발성역분화연골육종으로진단된 3예는초기진단이모두조직학적등급 2의연골육종이었다 ( 증례 6, 10, 11). 이환자들의경우연골육종의재발로판단하여수술전조직검사없이바로광범위절제술을시행한후역분화연골육종으로진단되었다 (Fig. 2). 최초진단후연골육종의재발의심하에수술한뒤역분화연골육종로진단되기까지걸린시간은각각 12, 8, 49개월이었다. 본연구에포함된모든환자가조직학적으로역분화연골육종의특징인이상성 (biphasic) 을보이고있었으며, 연골육종부분의병리학적등급은 6예에서 1등급, 5예에서 2등급이었다. 역분화부위의조직학적소견은 5예에서골육종, 6예에서미분화육종 (undifferentiated sarcoma) 이었다 (Table 2). 평균추시기간은 17 개월 (5-56 개월 ) 이었고, 국소재발이 8예, 원격전이가 10예에서발생했다. 국소재발은평균 8개월 (2-23개월 ), 원격전이는평균 7개월 (1-32개월) 에서관찰되었다. 원격전이가발생한시점의중앙값 (median) 은 3개월이었으며, 32개월에 E A B F C D G H Figure 1. An 80-year-old female with left hip pain that developed 2 months ago (case 5). (A) The initial plain radiograph shows well marginated calcified bone lesion at the proximal femur. Note the oblique fracture line with minimal displacement. The image shows no endosteal scalloping. (B, C) The coronal T2 and axial short tau inversion recovery T2-weighted magnetic resonance imagings show a lobular marginated bone lesion with peripheral soft tissue edema. Subtle cortical disruption can be seen at the greater trochanter. The initial presumptive diagnosis based on the images was low grade chondrosarcoma. (D) Curettage and bone cementing was performed. (E) The specimen was given a diagnosis of dedifferentiated chondrosarcoma. Low-power histologic photomicrograph shows the cartilage component and dedifferentiated component (H&E, 100). (F) Cartilaginous areas show mild nuclear pleomorphism and hyperchromatism (H&E, 400). (G) The dedifferentiated part consists of osteosarcomatous tumor cells without any chondroblastic differentiation (H&E, 400). (H) Wide excision and reconstruction using a tumor prosthesis was performed.

167 Dedifferentiated Chondrosarcoma 15 cm 15 cm A B 14 cm C 18 cm D E 9 cm F G Figure 2.

(A) The initial coronal T2 weighted magnetic resonance imaging (MRI) shows an intraosseous well-marginated

(B) The initial axial T2 weighted MRIs shows a subtle cortical disruption with soft tissue involvement.

(D) The specimen revealed a histologic grade 2 chondrosarcoma (H&E, 200).

Note the soft tissue mass at the gluteal muscle and endosteal scalloping of the femur.

Wide excision and reconstruction was performed under the impression of recurrence.

4 167 Dedifferentiated Chondrosarcoma 15 cm 15 cm A B 14 cm C 18 cm D E 9 cm F G Figure 2. A 46-year-old male with left hip pain that developed 6 months ago (case 10). (A) The initial coronal T2 weighted magnetic resonance imaging (MRI) shows an intraosseous well-marginated bone lesion. (B) The initial axial T2 weighted MRIs shows a subtle cortical disruption with soft tissue involvement. (C) Curettage and bone grafting were performed at the referral hospital. The diagnosis was chondrosarcoma. (D) The specimen revealed a histologic grade 2 chondrosarcoma (H&E, 200). (E) Left hip pain re-developed 3 months after surgery. Note the soft tissue mass at the gluteal muscle and endosteal scalloping of the femur. (F) Axial T2 weighted MR image shows the cortical destruction with soft tissue mass formation. Wide excision and reconstruction was performed under the impression of recurrence. (G) This photomicrograph shows the bimorphic pattern of grade 2 chondrosarcoma and osteoblastic osteosarcoma (H&E, 200). 전이가 발생한 증례를 제외한 역분화 연골육종 환자의 원격 전 전체 연골육종 환자 중 약 10%-15% 가량을 차지하는 매우 드물 이 발생 시점은 평균 3.3개월이었다. 역분화 연골육종 환자의 Ka- 고 악성도가 높은 종양으로,2) 국내에서는 증례 보고들로만 발표 plan-meier 3년 생존율은 18%였으며, 10명의 환자가 질병으로 인 되었다.6,7) 역분화 연골육종의 예후는 불량하여 평균 생존 기간은 해 사망하였다(Table 2). 13개월, 5년 생존율은 24%로 보고된 바 있으며 본 연구에서도 평 균 생존 기간은 17개월, 3년 생존율은 18%로 확인되었다.3,5) 본 연 고 찰 1) 1971년에 Dahlin과 Beabout 가 처음 보고한 역분화 연골육종은 구에서 진단 당시 전이가 없었던 원발성 역분화 연골육종 7예 중 사망한 환자 6명의 평균 폐 전이 발생 시간은 진단 후 3개월이었 다. 기존의 보고들과 이러한 본 연구 결과를 종합하면 역분화 연

5 168 Chang-Bae Kong, et al. Table 2. Clinical Courses & Oncologic Outcomes Case No. Histological grade of cartilage Subtypes of dedifferentiation Time to LR (mo) LR treatment Metastasis Time to metastasis (mo) Follow-up duration (mo) 1 1 Osteosarcoma 2 Lung, heart Initially 7 DOD 2 1 Undifferentiated 5 Surgery+RT Lung, liver 6 15 DOD 3 1 Undifferentiated None None 56 CDF 4 2 Undifferentiated 3 Surgery Lung 3 5 DOD 5 1 Osteosarcoma None Lung, spleen 2 12 DOD 6 2 Undifferentiated 4 Surgery Lung 2 6 DOD 7 2 Osteosarcoma 2 Lung 2 9 DOD 8 1 Undifferentiated None Lung, bone 1 5 DOD 9 1 Undifferentiated 3 Lung 6 10 DOD 10 2 Osteosarcoma 19 Lung, bone 5 23 DOD 11 2 Osteosarcoma 23 Surgery Lung DOD LR, local recurrence; RT, radiation; DOD, died of disease; CDF, continuous disease free. Final status 골육종은예후가매우좋지않은공격적인육종이라고판단할수있겠다. 역분화연골육종의방사선적인특징은저등급과고등급의두가지구성요소가같이관찰되는것이다. 방사선적으로전형적인연골육종의특성과함께피질골파괴, 연부종괴형성, 그리고석회화병변의혼재등이같이관찰된다 ( 증례 8; Fig. 3). Mercuri 등 9) 은이를세가지종류로분류한바있으며, 제1형은중심성연골육종의방사선적특징에공격적인골파괴를보이는경우, 제2형은양성내연골종과유사하나심한골파괴나큰연부조직종괴를형성하는경우 (Fig. 3), 제3형은연골성병변을암시하는소견이없는고등급의골파괴병변이라하였다. 10) 하지만본연구대상환자중역분화연골육종을방사선적으로의심하지못하여소파술을시행한환자의경우수술전단순방사선영상에서석회화병변은관찰되었으나피질골파괴혹은내피질의물결모양침식 (endosteal scalloping) 은보이지않았으며자기공명영상검사에서이상성이관찰되지않아저등급의연골육종으로판단하였다 ( 증례 5; Fig. 1). 소파술이후역분화연골육종이라는병리학적진단을확인하였고, 치료지연없이바로광범위절제술을시행하였으나수술후 2개월만에폐전이가발생하여 12개월에환자는사망하였다. 병리학적으로역분화연골육종은분화가좋은연골육종부분과분화가나쁜육종부분이명확한경계를이루며섞여있으며분화가나쁜부분에는골육종, 섬유육종, 악성섬유조직구종등이나올수있다. 3-6) 분화가나쁜육종부분이적을수있기때문에종양전체를병리학적으로꼼꼼하게확인하는것이역분화연골육종진단에필수적이다. 5) Frassica 등 2) 은역분화성연골육종의병리학적인이상성특징이처음진단시에관찰되면원발성, 후에나 타나는경우를속발성이라분류한바있으며연구대상환자의 86% 가원발성, 14% 가속발성이라하였다. 이렇듯같은질환에속발성과원발성이있기에병리학적으로분화가나쁜부분이저분화연골육종에서기원했을것으로추측할수있으나이는아직확실하지않다. 분화가좋은연골부분과분화가나쁜육종부분이같은기원세포를가진다는가설이있는반면두부분은다른기원세포에서생긴다는가설이있다. 6) Bovée 등 11) 은역분화연골육종발생초기에하나의전구세포 (precursor cell) 에서두개의클론이분리된다고했으나, Têtu 등 12) 은두개의다른클론이동시에 (synchronous) 분화를일으키면서이러한연골육종이발생한다고보고하였다. 역분화연골육종의기원세포가같은지여부에대해서는더많은연구가필요하다고생각된다. 역분화연골육종은항암치료및방사선치료에잘반응하지않는다. 4,7) Dickey 등 13) 은수술만받은환자와항암치료와수술을받은환자간에생존율에유의한차이가없었으며특히수술전항암치료를받은 22명의환자에게서나타난항암반응은불량하다고하였다. 이러한점을근거로저자들은역분화연골육종환자에게항암치료를시행할지여부에대해서재고해볼필요가있다고하였다. Grimer 등 3) 은역분화연골육종이항암치료에불량한반응을보이기때문에항암치료를시도해본다고하더라도, 수술후에고려해보는것이좋겠다고제안한바있다. 결론 역분화연골육종은조기에폐전이가발생하여매우좋지않은예후를갖는치사율이높은악성골종양으로확인되었다.

169 Dedifferentiated Chondrosarcoma A B C E D F Figure 3. A 60-year-old man with right thigh mass that developed 2 months ago (case 8).

(B, C) The sagittal T2 and axial enhanced T1 weighted magnetic resonance imagings show cortical disruption and the presence of a soft

Note the anterior soft tissue mass with cortical disruption.

(F) The anaplastic component showed a high-grade sarcoma in which the transformation origin was difficult to specify (H&E, 400).

Dedifferentiation of low-grade chondrosarcomas. Cancer. 1971;28:461-6. 2. Frassica FJ, Unni KK, Beabout JW, Sim FH.

1986;68:1197-205. 3. Grimer RJ, Gosheger G, Taminiau A, et al.

6 169 Dedifferentiated Chondrosarcoma A B C E D F Figure 3. A 60-year-old man with right thigh mass that developed 2 months ago (case 8). (A) The initial plain radiograph shows chondroid calcification with endosteal scalloping. (B, C) The sagittal T2 and axial enhanced T1 weighted magnetic resonance imagings show cortical disruption and the presence of a soft tissue mass. (D) An intercalary resection was performed at Korea Cancer Center Hospital. Note the anterior soft tissue mass with cortical disruption. (E) This high-power histologic photomicrograph shows the grade 1 chondrosarcoma component (H&E, 400). (F) The anaplastic component showed a high-grade sarcoma in which the transformation origin was difficult to specify (H&E, 400). CONFLICTS OF INTEREST The authors have nothing to disclose. REFERENCES 1. Dahlin DC, Beabout JW. Dedifferentiation of low-grade chondrosarcomas. Cancer. 1971;28: Frassica FJ, Unni KK, Beabout JW, Sim FH. Dedifferentiated chondrosarcoma. A report of the clinicopathological features and treatment of seventy-eight cases. J Bone Joint Surg Am. 1986;68: Grimer RJ, Gosheger G, Taminiau A, et al. Dedifferentiated chondrosarcoma: prognostic factors and outcome from a European group. Eur J Cancer. 2007;43: Liu C, Xi Y, Li M, et al. Dedifferentiated chondrosarcoma: radiological features, prognostic factors and survival statistics in 23 patients. PLoS One. 2017;12:e Staals EL, Bacchini P, Bertoni F. Dedifferentiated central chondrosarcoma. Cancer. 2006;106: Hwang PG, Won JK, Kim MA, Kim HS, Lee SH, Kim CJ. Dedifferentiated chondrosarcoma with giant cell-rich sarcoma-

7 170 Chang-Bae Kong, et al. tous component resembling giant cell tumor: a case report. Korean J Pathol 2004;38: Park JH, Koh HS, Lee SY. Dedifferentiated chondrosarcoma from low grade chondrosarcoma. J Korean Bone Joint Tumor Soc. 2005;11: Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980;153: Mercuri M, Picci P, Campanacci L, Rulli E. Dedifferentiated chondrosarcoma. Skeletal Radiol. 1995;24: MacSweeney F, Darby A, Saifuddin A. Dedifferentiated chondrosarcoma of the appendicular skeleton: MRI-pathological correlation. Skeletal Radiol. 2003;32: Bovée JV, Cleton-Jansen AM, Rosenberg C, Taminiau AH, Cornelisse CJ, Hogendoorn PC. Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. J Pathol. 1999;189: Têtu B, Ordóñez NG, Ayala AG, Mackay B. Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). II. An immunohistochemical and electron microscopic study. Cancer. 1986;58: Dickey ID, Rose PS, Fuchs B, et al. Dedifferentiated chondrosarcoma: the role of chemotherapy with updated outcomes. J Bone Joint Surg Am. 2004;86:

8 171 pissn : , eissn : Original Article J Korean Orthop Assoc 2019; 54: 역분화연골육종의임상결과 공창배 이승용 송원석 조완형 고재수 * 전대근 원자력병원정형외과, * 병리과 목적 : 역분화연골육종은저악성도의연골육종과골육종, 섬유육종등고등급의육종이같이관찰되는매우드문악성골종양이다. 저자들은원자력병원에서경험한역분화연골육종환자들의임상양상과종양학적결과에대해알아보고자하였다. 대상및방법 : 2007년부터 1월부터 2016년 12월까지본원에서역분화연골육종으로진단된이후치료받은 11명의환자를대상으로하였다. 이들의나이, 성별, 증상발현기간, 종양의위치및크기, 병기, 자기공명영상, 수술적절제연, 병리소견을후향적으로분석하였고, 국소재발및원격전이발생시간, 추시기간및종양학적결과를분석하였다. 생존율분석에는 Kaplan-Meier 생존율분석법을이용하였다. 결과 : 남자가 7예, 여자가 4예였으며, 평균연령은 54세 (33-80세) 였다. 종양의위치는대퇴골이 6예, 골반골이 4예, 중족골이 1예였으며종양의평균크기는 12.7 cm ( cm) 였다. 진단당시증상은 8예에서동통, 2예에서종괴였으며, 나머지 1예는방사선에서우연히발견된골병변이었다. 진단시병적골절이동반된환자는 2명이었으며, 병기는 1예에서 IIA기, 9예에서 IIB기, 1예에서 III 기였다. 8예가원발성, 3예가속발성역분화연골육종이었으며, 원발성중 1예는방사선적으로저등급의연골육종으로오인되어소파술을시행받은후진단되었다. 평균추시기간은 17개월 (5-56개월) 이었고, 국소재발이 8예, 원격전이가 10예에서발생했다. 국소재발은평균 8개월 (2-23개월), 원격전이는평균 7개월 (1-32개월) 에서관찰되었다. 역분화연골육종환자의 Kaplan-Meier 3년생존율은 18% 였으며, 10명의환자가질병으로인해사망하였다. 결론 : 역분화연골육종은조기에폐전이가발생하여매우좋지않은예후를갖는치사율이높은악성골종양으로확인되었다. 색인단어 : 역분화연골육종, 병리소견, 병적골절, 예후 접수일 2018 년 3 월 28 일수정일 2018 년 6 월 4 일게재확정일 2018 년 6 월 14 일책임저자조완형 01812, 서울시노원구노원로 75, 원자력병원정형외과 TEL , FAX , chowanda@naver.com, ORCID 대한정형외과학회지 : 제 54 권제 2 호 2019 Copyright 2019 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.

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