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1 양성전이성폐평활근종 1 예 국민건강보험공단일산병원내과, 가정의학과 1, 흉부외과 2, 해부병리과 3, 방사선과 4 강신애, 최상인 1, 김연아, 김정주, 양동규, 강정한 2, 기정혜 3, 홍용국 4, 이선민 A Case of Benign Metastasizing Pulmonary Leiomyoma Shin Ae Kang, M.D., Sang In Choi, M.D. 1, Yeon-A Kim, M.D., Chong Ju Kim, M.D., Dong Gyoo Yang, M.D., Jeong Han Kang, M.D. 2, Jeong Hae Kie, M.D. 3, Yong Kook Hong, M.D. 4, Sun-Min Lee, M.D. Department of Internal Medicine, Family medicine 1, Chest Surgery 2, Anatomical Pathology 3, and Radiology 4, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea A benign metastasizing pulmonary leiomyoma (BMPL) is a rare disease that usually occurs in women with a prior or coincident history of uterine leiomyoma. Although leiomyoma is histologically benign, it has the potential to metastasize to a distant site such as the lung. A 35 year old woman who had undergone a hysterectomy due to uterine leiomyoma 5 years prior was admitted for an investigation of multiple pulmonary nodules on a routine chest roentgenogram. An open lung biopsy was taken to make a pathological diagnosis. The microscopic finding of the nodules was leiomyoma and was similar to those of the uterine leiomyoma that had been resected 5 years ago. The woman underwent wedge resections of all pulmonary nodules. This is the first case of BMPL in Korea, which was treated with wedge resections of all multiple pulmonary nodules. (Tuberc Respir Dis 2005; 58: ) Key words : Benign metastasizing pulmonary leiomyoma, Uterine leiomyoma, Wedge resection 서 론 증 례 양성전이성폐평활근종 (Benign metastasizing pul monary leiomyoma, 이하 BMPL) 은자궁근종의과거력이있는여성에서발생하는폐의평활근세포증식성종양이다 1,2. BMPL 은조직학적으로양성이지만폐전이를하는독특한임상행태를취하며, 성호르몬수용체에양성을나타내는경우가많다 3,4. 대다수의환자가증상이없이우연히발견되며, 폐경전여성에서는드물게는호흡부전에이르는급성경과를밟기도하고, 폐경후여성에서는무활동상태로있거나자연히퇴화되기도한다 3-5. 저자들은우연히발견된폐의다발성결절을주소로내원한 35세여자환자에서양성전이성폐평활근종을진단하고, 치료로써폐의전결절제거술을시행한 1예를경험하였기에문헌고찰과함께이를보고하는바이다. Address for correspondence : Sun-Min Lee, M.D. 1232, Baeksok-dong, Ilsandong-gu, Koyang-shi, Kyonggi-do, NHIC Ilsan Hospital, Department of Internal Medicine, Korea Phone : Fax : minny@nhimc.or.kr Received : Feb Accepted : Jun 환자 : 김O숙, 여자 35세주소 : 단순흉부촬영에서우연히발견된폐의다발성결절과거력 : 7년전에자궁근종을진단받고추적관찰하던중, 5년전자궁근종의크기가증가하여서자궁적출술을시행받았으며, 혈압, 당뇨, 간염, 폐결핵과흡연의과거력은없었다. 현병력 : 본환자는단순흉부촬영에서우연히발견된폐의다발성결절을주소로내원하였다. 문진소견 : 기침, 객담, 발열은없었고, 식욕부진이나체중감소는없었다. 진찰소견 : 내원당시혈압은 110/80 mmhg, 맥박은 60회 / 분, 호흡수는 18회 / 분, 체온은 36.5 였다. 전신상태는양호하였고의식은명료하였다. 결막은창백하지않았으며공막의황달은없었고경부림프절은촉지되지않았다. 흉부청진상양측폐야의호흡음은깨끗하였고, 심음은규칙적이었으며, 심잡음은없었다. 간과비장은촉지되지않았다. 검사실소견 : 말초혈액검사상헤모글로빈 11.5g/dL, 헤마토크릿 33.7%, 백혈구 6,200/mm 3, 혈소판 217,000/ 614

Tuberculosis and Respiratory Diseases Vol. 58. No. 6, Jun. 2005 mm 3 이였고, 혈청생화학검사와특수혈액검사상특이사항없었다. 종양표지자검사상 CEA 0.4 ng/ml ( 정상 : 0~5 ng/ml), CA 125 15.7 U/mL ( 정상 : 0~35 U/mL) 이었다.

이병 변은 5년전절제되었던자궁근종과동일한병리소견을보였으며, 자궁근종에서폐로전이된양성전이성폐평활근종으로진단되었다. 5년전절제되었던자궁근종의진단이평활근육종을오인했을가능성을배재하기위해 2명의병리의사에의한합의에의해자궁근종으로판독하였다.

2 Tuberculosis and Respiratory Diseases Vol. 58. No. 6, Jun mm 3 이였고, 혈청생화학검사와특수혈액검사상특이사항없었다. 종양표지자검사상 CEA 0.4 ng/ml ( 정상 : 0~5 ng/ml), CA U/mL ( 정상 : 0~35 U/mL) 이었다. 방사선소견 : 내원당시에시행한단순흉부촬영에서다발성혈액성폐전이를의심하게하는다수의잘경계지워진결절들이양폐야에존재하였다 (Fig. 1). 흉부컴퓨터단층촬영상 0.5 cm 에서 1 cm 크기의결절들이양폐야에존재하였고, 종격동이나폐문부임파선의비대는관찰되지않았다 (Fig. 2). 병리학적소견 : 폐결절에대한쐐기절제술을시행하여조직병리검사에서평활근종으로진단되었다. 이병 변은 5년전절제되었던자궁근종과동일한병리소견을보였으며, 자궁근종에서폐로전이된양성전이성폐평활근종으로진단되었다. 5년전절제되었던자궁근종의진단이평활근육종을오인했을가능성을배재하기위해 2명의병리의사에의한합의에의해자궁근종으로판독하였다. 또한본종괴의증식정도를보기위해 Ki-67 과 p53에대한면역조직화학염색을시행하였으며그결과 KI-67양성세포는 1% 미만이었고 p53 에대한과발현도관찰되지않았다. 평활근종의폐병변은에스트로젠수용체는음성을프로게스테론수용체는양성을나타내었다 (Fig. 3). 치료및경과 : 환자는 9일간격으로두차례에걸쳐서전신마취하에개흉술을통한양폐야전이성결절의전제거술을시행받았으며, 호르몬치료를고려하며외래추적관찰중이다. 고 찰 Figure 1. Chest X-ray finding. Multiple well demarcated nodules suggesting a hema togenous lung metastasis BMPL 은자궁근종이있거나자궁근종으로자궁적출술을시행받았던여자환자에서폐에서발견되는증식성평활근세포로이루어진양성종양의일종으로, 1939년 Steiner 에의해처음보고되었다 1,2. 종양의크기는수밀리미터에서수센티미터에이르며, 폐뿐아니라피부, 종격동그리고뼈를침범하기도한다. BMPL 은조직학적으로유사분열이적고괴사가없으며조직의염증반응이적어서양성의형상을가지는 Figure 2. Computed tomography findings of the chest. A & B. Multiple variable sized nodules < 1 cm. There was no lymph node enlargement at the mediastinum or hilum. There was no evidence of an endobronchial lesion or obstruction. 615

A histology examination of the pulmonary mass showed it to be well demarcated and composed of benign smooth muscle (H&E, x200) B.

The hysterectomized uterine mass was histologically reviewed.

On immunohistochemical stain, the tumor cells show strong immunoreactivity for progesterone receptor on the nucleus (immunohistochemical

There were no over expression of p53 (immunohistochemical stain, X400).

BMPL 의발생기전에대해서는첫째로양성자궁근종으로수술받은환자에서미처발견되지못했던악성병변인자궁육종이폐로전이된것이라는설과, 둘째로양성인자궁근종이폐로색전된것이라는설,

3 SA Kang, et al.: A case of benign metastasizing pulmonary leiomyoma Figure 3. Pathologic findings. A. A histology examination of the pulmonary mass showed it to be well demarcated and composed of benign smooth muscle (H&E, x200) B. The pulmonary mass shows strong immunoreactivity for α-smooth muscle actin (immunohistochemical stain, x400). C. The hysterectomized uterine mass was histologically reviewed. The mass was composed of benign smooth muscle without evidence of necrosis, mitosis, cellular atypia or high cellularity (H&E, x200). D. On immunohistochemical stain, the tumor cells show strong immunoreactivity for progesterone receptor on the nucleus (immunohistochemical stain, x400). E. Ki-67 positive cells were < 1% (immunohistochemical stain, x400). F. There were no over expression of p53 (immunohistochemical stain, X400). 것으로생각되지만, 임상적으로는자궁병변의폐전이라는악성의행태를취하므로역설적인의미를갖는양성전이성폐평활근종이라는용어가사용되게되었다 3. BMPL 의발생기전에대해서는첫째로양성자궁근종으로수술받은환자에서미처발견되지못했던악성병변인자궁육종이폐로전이된것이라는설과, 둘째로양성인자궁근종이폐로색전된것이라는설, 셋째로비정상적으로과다한여성호르몬에의해서자궁이나폐등의다양한장기에서평활근세포가증식한병변이라는세가지가설이제기되고있다 년대이후 BMPL 은섬유평활근종성과오종의아류로서폐의원발성평활근종라는견해가지배적이었다 1, 년 Horstman 등은 BMPL 환자의 90% 이상에서자궁근종으로치료받은과거력이있고, 임신기간동안폐병변의자발적퇴화가있기도하기때문에 BMPL 이양성자궁근종이폐로색전된병변이라는견해를제시하였다 5. 그러나이후에 Horstman 등이보고하였 던예들을재검한결과양성자궁근종의일부분에서조직학적으로저단계이기는하지만악성을띠는육종이소수의예에서발견되었고, 이후에 BMPL 이자궁육종의폐전이라는가설이대두되기도하였다. 현재는 BMPL 을자궁과폐를주로관여하는전반적인평활근종증의아류로서분류하는것이일반적인견해이다. BMPL 의감별진단에있어서가장중요한점은조직학적으로악성인자궁육종이폐로전이되었을가능성을배제하는것인데, Klaus Kayser 등이 BMPL 10 예와자궁육종의폐전이 2 예와비교하여보고한바에따르면 BMPL은폐경전여성에서호발하며양폐를침범하는경우가드물고자궁적출술후평균 14.9 년후에폐병변이발견되는반면에, 자궁육종의폐전이는폐경후여성에서호발하고양폐를침범하는경우가대부분이며, 자궁적출술후 4.5 년이라는보다짧은시간이경과한후에폐병변이발견되었다 2. BMPL 은육종과비교시낮은증식활동을보이고에 616

4 Tuberculosis and Respiratory Diseases Vol. 58. No. 6, Jun 스트로겐과프로게스테론수용체발현률이높으며혈관형성도가낮았다 3,4. BMPL은성호르몬에의존적인종양으로생각된다 5. BMPL 을진단받은여성들중에서폐경후에, 임신과정중에, 또는피임약을중단한경우에폐결절이감소했다는보고가있으며, 폐경후여성에서는병의경과가느린반면에폐경전여성에서는급격한진행으로호흡부전까지발생한경우가보고되고있다 5-7. BMPL 의증상은매우다양하다. 보통은증상이없이우연히발견되는경우가대부분이며, 소수에서는열감이나경증의마른기침을호소하기도하고, 드물게는심장에침범해서호흡곤란을일으키거나폐에큰낭성근종을형성해서기관폐쇄로인한호흡부전으로병원에처음내원하기도한다 7. BMPL 의방사선적소견은전형적인데, 대부분의경우다발성의잘경계지워진폐결절로나타나며, 드물게는좁쌀양상또는낭성종양으로나타나기도한다 7. BMPL 은드문질환으로치료에대해표준적인지침이결정되어있지는않다. 병이진행하지않을경우추적관찰만하는방법과, 폐결절을수술적으로완전히제거하는방법, 자궁적출술과양측난소난관제거술을시행하는방법, 프로게스테론이나항에스트로겐물질, 또는 GnRH 유사체를사용하여화학적거세를시행하는방법등이사용되고있으며, 위의치료법들이복합적으로시도되기도한다 6,8,9. BMPL은호르몬에반응이있는종양이므로장기적인예후는양호한편이다. 본증례의경우가임기에있는젊은여성이었으며절제된폐결절에서에스트로겐수용체는음성이고프로게스테론수용체는양성을나타내었으므로호르몬의영향에의해폐의병변이진행할가능성이제기되었고, 따라서개흉술을시행하여 9일간에걸쳐서순차적으로우측과좌측폐의모든결절들을제거하였다. 호르몬에의한종양의성장을방지하기위하여자궁적출술과난소제거술이고려되어야했고, 환자는이미 5년전에자궁적출술을시행받은상태이었으며, 젊은여성이어서양측난소난관제거술을시행하기보다는호르몬치료를고려하기로하고퇴원하였으며현재외래에서추적관찰중이다. BMPL 에서다수의폐병변에대해개흉술을통한모든병변의제거를시 도한것은국내에서는처음보고하는바이다. 요 저자들은자궁근종으로자궁적출술을시행받은과거력이있는 35세여자환자가우연히발견된폐의다발성결절을주소로내원한후, 폐생검을통하여양성전이성폐평활근종을진단받고, 이후개흉술을통한전결절제거술을성공적으로시행받은드문 1 예를경험하였고, 이는국내에서는처음시도된바이기에문헌고찰과함께보고하는바이다. 약 감사의글 병리슬라이드판독에도움을주신이화여자대학교의과대학병리학교실조민선선생님께감사드립니다. 참고문헌 1. kayser K, Zink S, Schneider T, Dienemann H, Andre S, Kaltner H, et al. Benign metastasizing leiomyoma of the urerus: documentation of clinical, immunohis tochemical and lectin-histochemical data of ten cases. Virchows Arch 2000;437: Steiner PE. Metastasizing fibroleiomyoma of the uterusreport of a case and review of the literature. Am J Pathol 1939;15: Gal AA, Brooks JS, Pietra GG. Leiomyomatous neo plasm of the lung: a clinical, histologic, and immu nohistochemical study. Mod Pathol 1989;2: Esteban JM, Allen WM, Schaerf RH. Benign metas tasizing leiomyoma of the uterus: histologic and im munohistochemical characterization of primary and metastatic lesions. Arch Pathol Lab Med 1999;123: Horstmann JP, Pietra GG, Harman JA, Cole NG, Crinspan S. Spontaneous regression of pulmonary le iomyomas during pregnancy. Cancer 1977;39: Funakoshi Y, Sawabata N, Takeda S, Hayakawa M, Okumura Y Maeda H. Pulmonary benign metastas izing leiomyoma from the uterus in a postmeno pausal woman: report of a case. Surg Today 2004;34:

5 SA Kang, et al.: A case of benign metastasizing pulmonary leiomyoma 7. Maredia R, Snyder BJ, Harvey LA, Schwartz AM. Benign metastasizing leiomyoma in the lung. Radi ographics 1998;18: Jacobson TZ, Rainey EJ, Turton CW. Pulmonary be nign metastasizing leiomyoma: response to treatment with goserelin. Thorax 1995;50: Banner AS, Carrington CB, Emory WB, Kittle F, Leonard G, Rengus J, et al. Efficacy of oopherectomy in lymphangioleiomyomatosis and benign metastasizing leiomyoma. N Engl J Med 1981;305:

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h 박용범.hwp 객혈을동반한양성전이성폐평활근종 1 예 한림대학교강동성심병원내과, 흉부외과 1, 해부병리과 2 박소영, 임은주, 장지선, 김창환, 지현근 1, 조성진 2, 박용범, 이재영, 모은경 Benign Metastasizing Pulmonary Leiomyoma with Hemoptysis So Young Park, M.D., Eun-Ju Lim, M.D., Ji-Sun