Case Report J Korean Orthop Assoc 2012; 47: 정중신경에재발한지방섬유종성과오종 Recurrent Lipofibromat

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1 Case Report J Korean Orthop Assoc 2012; 47: Recurrent Lipofibromatous Hamartoma of the Median Nerve 김철홍 김보건 임영훈동아대학교의과대학정형외과학교실 지방섬유종성과오종 (lipofibromatous hamartoma) 은지방및결체조직이신경을과도하게침범하는것을특징으로하는드문말초신경종양이며문헌에서의보고도많지않다. 종양의치료방법으로부분적절제술이나신경감압술등을고려할수있는데저자들은정중신경에서발생한지방섬유종성과오종으로인해부분적절제술을시행했으나재발하여수근관증후군등의신경학적증상을일으킨 1예를보고하면서그특징과유의해야할사항들에대해보고하고자한다. 색인단어 : 정중신경, 지방섬유종성과오종, 수근관증후군 지방섬유종성과오종 (lipofibromatous hamartoma) 은지방및결체 조직이신경을과도하게침범하는것을특징으로하는드문말초 신경종양의일종으로주로전완부, 수근부, 수부의수장부에발 생한다. 1) 흔히정중신경을침범하고, 드물게척골신경을침범하 기도하며국내에서는드물게보고되고있다. 저자들은 19 세남자 환자에서종물감과수근관증후군을일으킨정중신경에서재발 한지방섬유종성과오종에대해수술적치료를시행한 1 예를보 고하고자한다. 증례보고 19 세남자환자로약 10 년전좌측수부종물및불편감으로지역 병원에서절제생검술을시행한병력이있으며이후로도가벼운 불편감이지속되다 5 년전부터증상이점점악화되어좌측전완 부와수부수장부종물및손저림을주소로내원하였다. 보호자 의기술에의하면처음수술적치료를시행한것은환자나이 8 세경이었고당시자기공명영상촬영을통해신경내지방섬유종 (intraneural lipofibroma) 이의심된다는설명을들었으며수장부 에서수근관절에이르는종물의크기를파악한후절제생검술 접수일 2011 년 11 월 3 일수정일 2011 년 12 월 16 일게재확정일 2012 년 2 월 14 일교신저자김보건부산시서구대신공원로 26, 동아대학교의료원정형외과 TEL , FAX cusem9@daum.net 을시행하였다고하나, 수술당시의상태나최종적인병리소견등의자료는남아있지않았다. 일차수술적치료후환자는가벼운불편감은있었으나종물의제거로보호자들은만족한상태였고, 특히운동및감각이상등의신경증상의합병증은관찰되지않았다고했다. 그러나환자는약 5년전부터수장부의종물재발을느꼈다고하였고, 본원외래내원후시행한이학적검사상좌측전완부원위부에서중수지관절원위수장부에이르는약 5 5 cm의종물이촉지되었으며경도의압통이관찰되었고간헐적으로 2, 3, 4번째수지에저림을호소하였으나운동및감각저하는없었으며수지의관절운동에도특이소견은없었다. 또한환자의과거병력상가족력등의특이소견도관찰되지않았다. 자기공명영상촬영에서좌측원위요골에서원위중수골부위까지정중신경의 ' 방추상비대 (fusiform enlargement)' 소견이관찰되었고, 내부에특징적인 ' 케이블모양 (cable like appearance)' 의소견 2,3) 이관찰되었으나주변조직으로의침범은관찰되지않았다 (Fig. 1). 환자는종물의비대로인하여불편감및간헐적인손저림을호소하여수술적치료를원하였고감각소실등신경학적합병증의가능성에대해충분한설명및동의를얻은후수술을시행하였다. 횡수근인대근위부 10 cm 상방에서중수지관절원위부까지지그재그형의피부절개를한후종물을노출시켰다. 횡수근인대근위부약 7 cm부터정중신경을따라종물이신경을감싸고있었으며원위부로갈수록비후는더심해지는양상을보였고횡수근인대아래에서정중신경의압박된부분을확인할수있었다 (Fig. 2). 근위부에서부터정중신경을따라종물외막에 대한정형외과학회지 : 제 47 권제 4 호 2012 Copyright 2012 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.

distributed fat, high signal intensity on T1-weighted sequences and low signal intensity on

T1 and T2 axial section at the base of metacarpals shows the typical 'cable-like appearance'

nerve surrounded by fat. Figure 2. Clinical photographs.

(B) Intra-operative view of the enlarged left median nerve with fibroadipose tissue

2 317 Figure 1. Magnetic resonance imaging scan showing the case of lipofibromatous hamartoma of the median nerve. (A, B) Low-intensity structures representing thickened nerve fascicles, surrounded by evenly distributed fat, high signal intensity on T1-weighted sequences and low signal intensity on T2-weighted sequences. T1 and T2 axial section at the base of metacarpals shows the typical 'cable-like appearance' (C) T1 coronal section of the wrist demonstrates 'fusiform-like enlargement' of the median nerve surrounded by fat. Figure 2. Clinical photographs. (A) Tumor in the left palm of the patient. (B) Intra-operative view of the enlarged left median nerve with fibroadipose tissue proliferation. (C) Photograph after a partial excision of the mass with epineurolysis and bleeding control. 절개를가한후, 섬유지방조직 (fibroadipose tissue) 이각각의신경속 (nerve fascicle) 을둘러싸고있음을확인하였다. 종물과정중신경의신경속사이의유착으로인해박리가매우어려운상태였고, 신경손상의위험때문에종물의완전절제는불가능하였고비후된신경외막 (epineurium) 에대한절개술및신경외막을둘러싸는종물의부분적절제와신경속사이로침습된종물의부분적제거술을시행하고수술을종료하였다. 수술이후환자는종물의 감소및저린증상의호전에는비교적만족하였으나정중신경의분지를따라 2, 3, 4 수지에서부분적인감각소실이발생하였다. 조직검사상지방섬유종성과오종으로확진되었고 (Fig. 3), 현재수술후 6개월째로외래를통한추시관찰중이다.

3 318 김철홍 김보건 임영훈 Figure 3. Charateristic histologic findings in lipofibromatous hamartoma (H&E stain, 20): The mass is composed of large nerve bundles (black arrow) surrounded by intermingled fibrous (A) and adipose tissue (B). 고찰 지방섬유종성과오종은주로소아기에전완부, 수근부, 수부의 수장부에발생하며 4) 신경외막과신경주위막 (perineurium) 에 ' 섬 유지방조직 (fibroadipose tissue)' 의과도한침착을특징으로하는 드문양성종양으로 2) 정중신경에호발하며, 신경내지방섬유종 (intraneural lipofibroma), 섬유지방증식증 (fibrofatty proliferation) 등으로명명되기도한다. 5) 임상증상으로진행하는수장부의종 물감과정중신경의압박으로인한신경증상이있고, 외상성신 경종, 결절종, 지방종및혈관기형등과감별이필요하다. 2) 지방섬유종성과오종은자기공명영상촬영상축상면상에서신 경은확장되어있으며각각의신경속이섬유지방조직내에서산 재되어 ' 케이블모양 ' 소견을보이며, 이것은지방섬유종성과오 종의특징적인소견이다. 2) 자기공명영상촬영상이러한특징적인 소견으로인해방사선학적추정진단이가능하므로 6) 자기공명영 상촬영은진단과수술적치료계획에필수적이다. 본증례에서 도자기공명영상촬영에서전형적인양상의 ' 케이블모양 ' 의소견 을보이고있다 (Fig. 1). 병태생리학적질병의원인으로횡수근인대로인한정중신경 의만성적인미세외상또는비정상적으로증식된굴곡근지대 (flexor retinaculum) 로부터압력이 ' 반응과정 (reactive process)' 을 유발하여종양을발생시킨다는보고가있으나, 1,2) 정확한발병원 인은아직밝혀져있지않다. 2) 조직학적으로는신경외막을침범하고각각의신경섬유를 분리시키는소견을보이며, 때때로광범위한신경주위섬유화 (perineural fibrosis) 를유발하여 7) ' 양파줄기모양 (onion bulb-like appearance)' 의소견을보인다. 2) 이러한특성때문에종양과신경 사이의유착이심하므로, 종양의완전한제거는위험하며수술적 치료의필요성에대한논란이있다. 수술적치료방법으로는신경감압술과섬유지방조직의축소술 (debulking operation), 미세수술을이용한신경박리및절제술, 그리고종양의근치적절제술등이있다. 8) 여러저자들은지방섬유종성과오종에서수근관감압술이나종양조직의축소술을통해증상을호전시켰음을보고하였으나, 2,4,7) 이러한술식후장기추시에서심각한신경손상이발생하였다는보고도많다. 7,9) 최근에 Clavijo-Alvarez 등 8) 이신경내신경속박리 (intraneural fascicular dissection) 및신경이식술 (nerve graft) 시행후신경학적기능이보존되었다고보고하였지만수술적박리술은현재까지는만족할만한결과를보이지않고있다. 5) 그러나원위전완부에서정중신경과척골신경간에교차되는운동신경인 ' 마틴- 그루버문합 (Martin-Gruber anastomosis)' 이존재하는소아기환자에서수술이시행된경우에는심각한신경학적손상이없이, 더광범위한절제가가능하다는보고도있다. 10) 본증례는이전수술에서종물의부분절제술시행후종물의크기가다시증가하여재발한예로서, 국내에서는정중신경에발생하고재발한지방섬유종성과오종의첫증례이다. 환자가수술적제거술을원하였고합병증에대해충분한설명을한후, 수술적제거술및수근관감압술을시행하였으나신경학적합병증을피할수는없었다. 현재술후 6개월의경과관찰에서술후감각소실증상등이호전을보이지않고있다. 이에저자들은본증례와같은환자들의치료에있어가능한보존적치료를권장하며, 수술적치료가불가피한경우수술적치료에대한충분한설명이반드시필요하고, 이환자의경우처럼종양이비교적서서히증식한다는점을고려하여필요한경우완전절제보다종물의종괴효과에대한부분절제술을시행하고수근관감압술등신경증상에대한감압술을시행하는최소한의수술적제거술이더나은선택이라생각한다. 참고문헌 1. Guthikonda M, Rengachary SS, Balko MG, van Loveren H. Lipofibromatous hamartoma of the median nerve: case report with magnetic resonance imaging correlation. Neurosurgery. 1994;35: Al-Jabri T, Garg S, Mani GV. Lipofibromatous hamartoma of the median nerve. J Orthop Surg Res. 2010;5: Nilsson J, Sandberg K, Søe Nielsen N, Dahlin LB. Magnetic resonance imaging of peripheral nerve tumours in the upper extremity. Scand J Plast Reconstr Surg Hand Surg. 2009;43: Johnson RJ, Bonfiglio M. Lipofibromatous hamartoma of the median nerve. J Bone Joint Surg Am. 1969;51:

4 Houpt P, Storm van Leeuwen JB, van den Bergen HA. Intraneural lipofibroma of the median nerve. J Hand Surg Am. 1989;14: Marom EM, Helms CA. Fibrolipomatous hamartoma: pathognomonic on MR imaging. Skeletal Radiol. 1999;28: Warhold LG, Urban MA, Bora FW Jr, Brooks JS, Peters SB. Lipofibromatous hamartomas of the median nerve. J Hand Surg Am. 1993;18: Clavijo-Alvarez JA, Price M, Stofman GM. Preserved neurologic function following intraneural fascicular dissection and nerve graft for digital and median nerve lipofibromatous hamartoma. Plast Reconstr Surg. 2010;125:120e-2e. 9. Louis DS, Hankin FM, Greene TL, Dick HM. Lipofibromas of the median nerve: long-term follow-up of four cases. J Hand Surg Am. 1985;10: Paletta FX, Senay LC Jr. Lipofibromatous hamartoma of median nerve and ulnar nerve: surgical treatment. Plast Reconstr Surg. 1981;68:

5 320 김철홍 김보건 임영훈 Recurrent Lipofibromatous Hamartoma of the Median Nerve Chul Hong Kim, M.D., Bo Kun Kim, M.D., and Young Hoon Lim, M.D. Department of Orthopedic Surgery, Dong-A University College of Medicine, Busan, Korea Lipofibromatous hamartoma is a rare tumor of the peripheral nerves which is characterised by an excessive infiltration of the epineurium and perineurium by fibroadipose tissue and very few cases have been described and reported in the literature. Surgical treatments of lipofibromatous hamartoma include partial excision, debulking operation, nerve decompression and so on. We report a case of recurrent lipofibromatous hamartoma of the median nerve that partial excision was done previously but causing secondary carpal tunnel syndrome and a review of the literature regarding the etiology, pathogenesis and surgical management of lipofibromatous hamartoma. Key words: median nerve, lipofibromatous hamartoma, carpal tunnel syndrome Received November 3, 2011 Revised December 16, 2011 Accepted February 14, 2012 Correspondence to: Bo Kun Kim, M.D. Department of Orthopedic Surgery, Dong-A University Medical Center, 26, Daesingongwon-ro, Seo-gu, Busan , Korea TEL: FAX: cusem9@daum.net

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