09(07)-이종서/

Size: px

Start display at page:

Download "09(07)-이종서/"

준현 탁
5 years ago
Views:

1 대한척추외과학회지제 16 권제 4 호 Journal of Korean Spine Surgery Vol. 16, No. 4, pp 294~298, 2009 DOI: /jkss 요추골화섬유점액종의수술적치료 - 증례보고 - 이종서 정성수 오성균 # 성균관대학교의과대학정형외과, 원광대학교산본병원정형외과 # Surgical Treatment of Ossifying Fibromyxoid Tumor Invading the Lumbar Spine - A Case Report- Chong Suh Lee, M.D., Sung Soo Chung, M.D., Sung Kyun, Oh, M.D. # Department of Orthopedic Surgery, Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul, Korea Department of Orthopedic Surgery, Wonkwang University Sanbon Hospital, Gunpo, Korea # Abstract Ossifying fibromyxoid tumor is rare soft tissue neoplasm of an uncertain histogenesis, and this was first described in The majority of the reported cases have involved the soft tissue of the extremities. We present here on a case of atypical ossifying fibromixoid tumor that had invaded the spine and we report on its management and outcome. We also review the relevant literature. Key Words: Ossifying fibromyxoid tumor, Lumbar spine, Spinal invasion 서 론 골화섬유점액종은 1989년 Enzinger 등이처음기술한연부조직양성종양으로조직학적으로골혹은유골및연골조직을포함한점액양기질부에단일한타원형세포들로구성되는특징을가진다 1). 2008년까지영어논문을기준으로약 220례가보고돼있으나대부분피하조직이나근육같은연부조직을침범한것으로골조직을침범하거나후복막강을침범한증례는단 2례에불 과하다 2,3). 임상적으로악성혹은비전형적인양상을보이는증례들이최근보고되고있으며저자역시요추를침범하여방사통을야기하는거대골화섬유점액종의치험예를보고하고자한다 2,3,4,5). 증례보고 약 4 개월전부터발생한우측하지방사통및요통을주소로 37 세여자환자가내원하였으며이학적검사상 Address reprint requests to Sung Kyun,Oh,M.D. Department of Orthopaedic Surgery, Wonkwang University Sanbon Hospital, Sanbondong, Gunpo city, Gyeongido, Korea Fax: Tel: niceo@daum.net Received: Accepted:

요추골화섬유점액종의수술적 - 증례보고 - 이종서외 천추 1,2,3 번피부영역의감각감소및우측무지의신전및굴곡근력약화소견을보였다. 단순방사선사진상우측골반내의점상석회화를동반한거대한연부조직음영이관찰되었고, CT 검사상거대한연부조직종괴가제 5 요추를침범하고있으며크기는약 13 cm 직경을보였고가장자리에불규칙적인점상석회화소견을보였다 (Fig. 1).

CT 조영하세침조직검사소견상영상의학과판독과도일치한악성도가높은연골육종소견보였다. 전이유무를위해검사한전신골조영및흉부 CT 검사상전이소견은보이지않았다.

2 요추골화섬유점액종의수술적 - 증례보고 - 이종서외 천추 1,2,3 번피부영역의감각감소및우측무지의신전및굴곡근력약화소견을보였다. 단순방사선사진상우측골반내의점상석회화를동반한거대한연부조직음영이관찰되었고, CT 검사상거대한연부조직종괴가제 5 요추를침범하고있으며크기는약 13 cm 직경을보였고가장자리에불규칙적인점상석회화소견을보였다 (Fig. 1). 자기공명검사소견상 cm 크기의거대한종괴가우측골반내후복막강에위치하고있으며제 5 요추를침범하여척추관내의경막외공간까지침범하여신경관을압박하는소견을보이고있었다. 종괴의조영은 T1 강조영상에서근육과동일한정도의조영증강을보였고,T2 강조영상에서이형질을띠며고음영을보였다 (Fig. 2). CT 조영하세침조직검사소견상영상의학과판독과도일치한악성도가높은연골육종소견보였다. 전이유무를위해검사한전신골조영및흉부 CT 검사상전이소견은보이지않았다. 수술시출혈을줄이기위해술전색전술을시행한후수술을시행하였고, 악성도가높아고식적수술 (palliative surgery) 을계획하고경복막접근을통해종괴의광 범위절제와제 5 요추절제술을시행한후골시멘트충진된 cage 를이용하여전방지지를시행하였고, 동시에후방에서척추경나사를이용한고정술을시행하였다 (Fig. 3A). 종괴는육안상주위조직으로의침윤이없이비교적뚜렷한변연을가진회백색을띄었다. 수술후최종조직학적진단은술전과는다르게골화섬유점액종소견을보여장기생존을고려하여추가적으로자가비골을이용한후방골유합술을시행하였고약 3 주후국소재발방지를위해방사선치료를시행하였다. 그러나술후 8 개월째달리기운동중발생한통증으로응급실내원하여검사한방사선소견상 cage 전방탈출소견보여전방으로접근하여 cage 제거후전방골이식술시행하였다 (Fig. 3B). 종괴의광학현미경소견상경계가비교적분명한불완전한골섬유양경계를가진원형의종양세포가로제트 (rosettes) 를형성하고있으며이는가는유리화된섬유띠로분리되어있었다 (Fig. 4). 면역조직화학적소견상 S-100 단백과 neuron-specific enolase 에양성소견을보였다 (Fig. 4, Table 1). 수술후외래추시상 6 년째국소재발및전이소견보이지않고양호한경과를보이고있으나방사선상국소후만및후방금속봉의파손소견보여불유합의심하에 CT 검사시행하였으며골유합소견보이고증세없어외래추시중이다 (Fig. 3C). 고 찰 Fig. 1. Precontrast CT scan of level L5 vertebra revealed a large soft tissue mass measuring about 13cm in length invading the L5 vertebral body. 골화섬유점액종은연부조직에발생하는드문양성종양으로주로피부나사지의근육을침범하는것으로알려져있다. 이증례처럼골조직을침범하는경우는매우드물고후복막강과대퇴골을침범한 2 례가보고된적이있다 2,3). 술전증상은평균 4 년으로전형적으로서서히자라는무통성종괴였고 104 명중 12 명의경우술전 10 년간관 Fig. 2. (A) T2 weighted axial MR shows the mass is a heterogeneous and isointense to muscle. (B) T1 weighted coronal MR shows the mass is isointense with psoas muscle. (C) T2 weighted saggital MR shows the mass extend to the canal with epidural mass formation

(hypocelluar metaplastic bone shell) 이변연에존재한다 1,4,5). 면역조직화학적으로 S-100 단백양성을보이는경우가 60~94% 로보고자마다상이하나이는골화섬유점액종의기원을어느쪽으로여기는지에따라이견이존재한다.

3 대한척추외과학회지 Vol. 16, No. 4, 2009 찰하였으며, 발견후 35 년후에수술한경우가최장관찰기간이었다 4,5). 골화섬유점액종의조직학적인특징은점액양기저부에타원형혹은방추형세포들이소엽이 Table 1. Immunohistochemical profile Antigen +/- S100pretein + Neuron-specific enolase + Leu-7 - Glial fibrillary acidic protein - Desmin - Smooth muscle actin - Cytokeratin AE1/3 - 나결절을형성하는것으로일반적으로저세포성골조직 (hypocelluar metaplastic bone shell) 이변연에존재한다 1,4,5). 면역조직화학적으로 S-100 단백양성을보이는경우가 60~94% 로보고자마다상이하나이는골화섬유점액종의기원을어느쪽으로여기는지에따라이견이존재한다. 골화섬유점액종의기원은 Schwann 세포, 평활근, 연골, 골, 그리고근상피세포 (myeoepithelial cell) 라는여러이견과보고들이있다 5,6,7,8,9). 조직학적으로도감별해야할질환은신경초종, 연골육종, 평활근종, 섬유성골이형성증그리고골육종등이다. 일반적인골화섬유점액종은양성경과를보인다. 그러나전이나재발을보이는비전형적혹은악성골화섬유점액종에대한보고들이최근증가하였고이들용어 Fig. 3. (A) Immediate post operative lateral view. (B) 2nd Post operative lateral view. (C) Postoperative 6year last follow up lateral view. Fig. 4. (A) The photomicrograph ( 100, Hematoxylin-eosin stain) shows a well circumscribed mass with an incompletely ossified fibrous rim. Tumor cells are relatively uniform round to oval shape arranged in small rosettes that were separated by delicate hyalinized fibrous bend. (B) By Immunohistochemistry, the tumor cells are positive for S

4 요추골화섬유점액종의수술적 - 증례보고 - 이종서외 에대한기준과치료에대해서는이견이있다. 70 례를보고한 Folpe 등 4) 은 high nuclear grade (defined as irregular nuclear contours,coarse chromatin,and macronucleoli) 혹은 high ceullarity 와 50 HPF (high power field) 당 2 개이상의유사분열 (mitotic figure) 을보이는경우악성경과를보일가능성이높으므로육종으로여기고치료할것을제안하였다. 골화섬유점액종의치료는크기가작은경우자유변연을얻는국소절제이며재발하였거나크기가큰경우혹은심부에위치한조직학적으로비전형혹은악성의특징을보이는경우광범위절제를요한다. 국소재발률은 27% 까지보고되었으나이는비전형적혹은악성증례들이다수포함된경우이며 Miettinen 등 4,5) 의경우에서는이보다낮게보고하고있고 3 cm 이상의크기와응고괴사, 그리고위성결절의존재나절제선상양성을보이는경우국소재발과도관계없다하였다. 보조치료 (adjuvant therapy) 의경우양성경과를보일것으로예상되는전형적인경우는필요치않으며, 본증례의경우심부에서거대종괴를형성하여신경관내까지파급한증례로신경관내국소재발에의해신경압박등의합병증이예상되므로술후방사선치료를시행하였다. 또한골종양및심부연부종양의경우술전조직검사를시행하여조직학적진단과병기결정을시행하여치료계획을수립하나본증례의경우처럼최종진단에서바뀌는경우가드물지않으므로수술중반복적인동결조직검사와병리의사와의충분한상의가요구된다. 또한근골격계종양의침생검의진단정확도가 80~90% 임을감안하여영상의학과소견과임상소견을종합하여술전치료방침을정하여야할것으로사료된다 10). 본증례의경우도악성도를지나치게높게평가하여환자의기대여명과활동에대해과소평가하여일차수술시충분한골이식과견고한골유합을얻지못한것이재수술로이어졌다고여겨진다. 요약하면전형적인골화섬유육종의경우양성의임상경과를보이나심부에위치한비전형적인양상의골화섬유점액종의경우잠재적인악성도를고려하여치료할수있을것으로사료된다. 참고문헌 01) EnzingerFM, Weiss SW, Liang CY: Ossifying fibromyxoid tumor of soft parts. A clinicopathological analysis of 59 cases. Am J Surg Pathol 1989; 13: ) Schaffler G,Raith J, Ranner G, Weybora W, Jeserschek R: Radiographic appearance of an ossifying fibromyxoid tumor of soft parts. Skeletal Radiol 1997; 26: ) Ogose A, Otsuka H, Morita T, Kobayashi H, Hirata Y: Ossifying fibromyxoid tumor resembling parosteal osteosarcoma. Skeletal Radiol 1998; 27: ) Folpe AL, Weiss SW: Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants. Am J Surg Pathol 2003; 27: ) Miettinen M, Finnell V, Fetsch JF: Ossifying fibromyxoid tumor of soft parts--a clinicopathologic and immunohistochemical study of 104 cases with long-term follow-up and a critical review of the literature. Am J Surg Pathol 2008; 32: ) Donner LR: Ossifying fibromyxoid tumor of soft parts: evidence supporting Schwann cell origin. Hum Pathol 1992; 23: ) Schofield JB, Krausz T, Stamp GW, Fletcher CD, Fisher C, Azzopardi JG: Ossifying fibromyxoid tumour of soft parts: immunohistochemical and ultrastructural analysis. Histopathology 1993; 22: ) Sovani V, Velagaleti GV, Filipowicz E, Gatalica Z, Knisely AS: Ossifying fibromyxoid tumor of soft parts: report of a case with novel cytogenetic findings. Cancer Genet Cytogenet 2001; 127: ) Nishio J, Iwasaki H, Ohjimi Y, et al.: Ossifying fibromyxoid tumor of soft parts. Cytogenetic findings. Cancer Genet Cytogenet 2002; 133: ) Oqilvie CM, Torbert JT, Finstein JL, et al.: Clinical utility of percutaneous biopsies of musculoskeletal tumors. Clin Orthop Relat Res 2006; 450:

5 대한척추외과학회지 Vol. 16, No. 4, 2009 국문초록 골화섬유점액종은매우드문연부조직양성종양으로척추같은골조직을침범하는것은매우드물다. 특히심부에위치하고크기가큰비전형적인경우조직학적인악성도에따라악성에준하여치료하여야한다. 요추를침범해방사통과요통을일으키는골화섬유점액종의수술적치료를시행하고 6년추시한결과재발및전이없이양호한임상경과를보여치험례를보고하고자한다. 색인단어 : 골화섬유점액종, 요추, 척추침범 통신저자 : 오성균경기도군포시산본동 1142 원광대학교산본병원정형외과 Tel: , Fax: , niceo@daum.net

Kaes017.hwp

Kaes017.hwp 갑상선의 Poorly Differentiated (Insular) Carcinoma Insular Carcinoma: An Aggressive Subtype of Differentiated Thyroid Neoplasms Seok-Jin Nam, M.D., Sang-Dal Lee, M.D., Hal-lin Park, M.D., Young-Ryun Oh, M.D.