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1 혈관육종의낭성폐전이에의한기흉 1 예 연세대학교의과대학 1 내과학교실, 2 병리학교실, 3 폐질환연구소 이찬주 1, 최준정 2, 전한호 1, 정경수 1, 박병훈 1, 박선철 1, 신상윤 1, 정우영 1, 변민광 1, 문지애 1, 김영삼 1,3, 김세규 1,3, 장준 1,3, 김성규 1,3, 박무석 1,3 A Case of Pneumothorax Caused by Cystic Lung Metastasis of Angiosarcoma Chan Joo Lee, M.D. 1, Jun Jeong Choi, M.D. 2, Han Ho Jeon, M.D. 1, Kyung Soo Jung, M.D. 1, Byung Hoon Park, M.D. 1, Seon Cheol Park, M.D. 1, Sang Yun Shin, M.D. 1, Wou Young Chung, M.D. 1, Min Kwang Byun, M.D. 1, Ji Ae Moon, M.D. 1, Young Sam Kim, M.D. 1,3, Se Kyu Kim, M.D. 1,3, Joon Chang, M.D. 1,3, Sung Kyu Kim, M.D. 1,3, Moo Suk Park, M.D. 1,3 Departments of 1 Internal Medicine, 2 Patholgy, 3 The Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea Angiosarcoma is a rare but highly malignant tumorthat usually arises in the scalp or face of elderly males. Distant metastases favor the lung, liver, lymph nodes and skin. Metastatic pulmonary angiosarcoma commonly takes the form of a nodule but can sometimes appear as a thin-walled cyst. We report a case of 65 years-old male with a spontaneous pneumothorax, who underwent excision and radiotherapy for an angiosarcoma of the scalp 2 years ago. A chest CT scan revealed multiple cysts in the lung. The video-assisted thoracoscopic lung biopsy demonstrated subpleural cysts without tumor cells. A skin biopsy of the scalp showed an angiosarcoma. This case was diagnosed as a recurrence of an angiosarcoma with a supposed lung metastasis. This case suggests that a spontaneous pneumothorax in elderly people may be secondary to a pulmonary metastasis from an angiosarcoma of the scalp. (Tuberc Respir Dis 2008;64: ) Key Words: Angiosarcoma, Cyst, Lung metastasis, Pneumothorax 서 혈관육종은매우드문악성종양으로고령남자의두피나얼굴에발생하는것으로알려져있다. 임상적으로하나또는여러군데, 파란색또는자색의결절, 판상등으로다양하게나타난다 1,2. 전이는림프및혈액을통해이루어져림프절, 간, 폐에주로생기며, 폐전이의경우에는낭성병변을형성하여폐기종과의감별을요하기도한다 4,5. 저자들은두피의혈관육종을진단받고치료받았던경우에서객혈, 기침등의호흡기증상이새롭게나타나폐의낭성병변및기흉, 혈흉을발견하고혈관육종의전이로 Address for correspondence: Moo Suk Park, M.D. Department of Internal Medicine, Yonsei University College of Medicine, 134, Shinchon-dong, Seodaemun-gu, Seoul , Korea Phone: , Fax: pms70@yuhs.ac Received: Mar. 10, 2008 Accepted: Apr. 21, 2008 론 판단하여치료한증례를경험하였기에문헌고찰과함께보고하는바이다. 증례환자 : 조, 남자, 65세주소 : 호흡곤란현병력 : 20일간의객혈을주소로내원하여시행한흉부전산화단층촬영결과다발성폐낭종이발견되어검사도중내원 1일전부터호흡곤란이발생하여입원하였다. 과거력 : 2년전두피의혈관육종으로절제수술받았으며방사선치료받았다. 가족력 : 특이사항없었다. 사회력 : 특이사항없었다. 이학적소견 : 입원시활력징후는혈압 90/40 mmhg, 맥박 107회 / 분, 호흡수 25회 / 분, 체온 36.6 o C였다. 의식은명료하였으며전신무력감및급성병색보였으며, 결막은 374

Tuberculosis and Respiratory Diseases Vol. 64. No. 5, May. 2008 Figure 1. When angiosarcoma of the scalp was diagnosed, chest CT scans showed no cavitary lesions. Figure 2.

장음은정상이었고복부팽만은없었으며, 간및비장은촉지되지않았다. 늑골요추각압통및하지에함요부종은없었다. 검사실소견 : 말초혈액검사에서백혈구 11,040/mm 3, 혈색소 10.8 g/dl, 혈소판 314,000/mm 3 이었다. 혈청생화학검사에서 BUN 20.8 mg/dl, creatinine 1.1 mg/dl, total protein 6.

2 mmol/l, Cl 106 mmol/l이었고, 종양표지자검사상 CEA 4.19 ng/ml, CA 125 15.7 U/ml, CA 19-9 5.3 U/ml이었다. 방사선소견 : 2년전혈관육종진단당시에시행한흉부 전산화단층촬영에서낭종등의특이소견이보이지않았다 (Figure 1).

2 Tuberculosis and Respiratory Diseases Vol. 64. No. 5, May Figure 1. When angiosarcoma of the scalp was diagnosed, chest CT scans showed no cavitary lesions. Figure 2. When patient visited outpatient department due to repiratory symptom, Chest CT scans showed multiple cysts and ground glass opacity. 창백하지않았고공막에황달소견은보이지않았다. 호흡음은좌폐에서감소되어있었으며, 심음은규칙적이었다. 장음은정상이었고복부팽만은없었으며, 간및비장은촉지되지않았다. 늑골요추각압통및하지에함요부종은없었다. 검사실소견 : 말초혈액검사에서백혈구 11,040/mm 3, 혈색소 10.8 g/dl, 혈소판 314,000/mm 3 이었다. 혈청생화학검사에서 BUN 20.8 mg/dl, creatinine 1.1 mg/dl, total protein 6.0 mg/dl, albumin 4.0 mg/dl, total bilirubin 0.7 mg/dl, AST 21 IU/L, ALT 21 IU/L, alkaline phosphatase 88 IU/L, calcium 8.4 mg/dl, phosphate 4.1 mg/dl이었다. 전해질검사상 Na 141 mmol/l, K 4.2 mmol/l, Cl 106 mmol/l이었고, 종양표지자검사상 CEA 4.19 ng/ml, CA U/ml, CA U/ml이었다. 방사선소견 : 2년전혈관육종진단당시에시행한흉부 전산화단층촬영에서낭종등의특이소견이보이지않았다 (Figure 1). 객혈을주소로내원하여시행한흉부전산화단층촬영상양측폐실질에다발성낭종이관찰되었으며, 입원일주일전에시행한양전자방출단층촬영-전산화단층촬영상양폐야의출혈을동반한낭종들이더커진상태였다 (Figure 2). 내원당시시행한단순흉부 X-선상좌측흉부의기흉관찰되었다 (Figure 3). 임상경과및치료 : 자발성기흉으로흉관삽입하였으며, 낭종들에대하여비디오유도하흉강경조직검사를시행받았다. 조직검사상혈관육종의전이가의심되나확진되지않은상태로퇴원하였다. 퇴원 2일후에다시좌측의기흉발생하여입원하여흉관삽입하였으며, 비디오유도하흉강경쐐기절제술을시행받으면서동시에 2년전치료받았던두피의피부병변에대하여조직검사를시행받았다 (Figure 4, 5). 두피의조직검사상혈관육종재발을진단받 375

CJ Lee et al: Cystic lung metastasis of angiosarcoma Figure 3. On admission, Chest X-ray showed left pneumothorax.

Subpleural cyst formation was seen in viedoassited thoracoscopic lung biopsy, but tumor cells were not seen (H&E stain,

Skin biopsy showed that cysts and intracytoplasmic spaces were formed by tumor cells (A, H&E stain, 200), which were

았으며, 임상적으로폐로의낭성전이로판단하고 paclitaxel (70 mg/m 2 ) 로항암치료시작하였으나, 암전이가진행하여객혈및기흉지속되어사망하였다 (Figure 6).

3 CJ Lee et al: Cystic lung metastasis of angiosarcoma Figure 3. On admission, Chest X-ray showed left pneumothorax. Figure 4. Subpleural cyst formation was seen in viedoassited thoracoscopic lung biopsy, but tumor cells were not seen (H&E stain, 10). Figure 5. Skin biopsy showed that cysts and intracytoplasmic spaces were formed by tumor cells (A, H&E stain, 200), which were positive for CD 31, marker of endothelial cells, on the immunohistochemical stain (B, 400). 았으며, 임상적으로폐로의낭성전이로판단하고 paclitaxel (70 mg/m 2 ) 로항암치료시작하였으나, 암전이가진행하여객혈및기흉지속되어사망하였다 (Figure 6). 고찰혈관육종은매우드문악성종양으로혈관의내피세포에서기원한다 1. 전체악성종양의 1% 이하를차지하며, 모든연조직육종의 2% 정도를이루는것으로알려져있다 2. 주로피부와표면의연조직에서발생하여, 노년의남성의얼굴, 두피, 목부위에서호발한다 2,3. 혈관육종의진 단시에이미전이가되어있는경우가많아서예후가불량하며, 주된전이부위는폐, 간, 림프절, 피부이다 4. 혈관육종이폐로전이되는경우객혈, 기흉, 혈흉등의호흡기증상을일으킬수도있다 5. Ukihide 등 6 의혈관육종의폐전이에대한흉부전산화단층촬영결과에대한연구에따르면폐전이가결절성병변을이루는경우가 63% 이고, 얇은벽의낭종을보이는경우가 21% 이었다. 병이진행하면서낭종의크기는커지며흉막하에위치하는경우에는기흉이나혈흉을일으키게된다. 낭종안에공기-액체층이 13% 에서관찰되었으며이는전이성병변의취약성으로인한출혈로생각된다. 376

Tuberculosis and Respiratory Diseases Vol. 64. No. 5, May. 2008 Figure 6.

During disease progression, increased ground glass opacity was observed and pneumothorax was recurred. 낭성변화를일으키는기전에대해서는여러가지가설이제시되고있다.

셋째로는, 원래존재하던폐낭포주변으로종양세포가침윤한다는이론이있고, 넷째로종양이작은기관지를눌러서체크밸브역활을하여폐포를과다팽창시켜폐실질의낭성변화를일으킨다는것이다 7.

Kitagawa 등 5 은 95예의부검을통해두피에서기원한혈관육종이다른부위에서기원한경우보다폐전이가더잘되어호흡기계합병증을더잘일으키는것으로보고하였으며, 특히기흉은두피기원의혈관육종환자에서만발견되었고매우불량한예후를나타낸다고하였다.

4 Tuberculosis and Respiratory Diseases Vol. 64. No. 5, May Figure 6. Chest CT scans revealed that multiple cysts have increased in size and number. During disease progression, increased ground glass opacity was observed and pneumothorax was recurred. 낭성변화를일으키는기전에대해서는여러가지가설이제시되고있다. 첫째, 결절성종양형성후에혈류장애로인해괴사되고괴사조직은빠져나가낭종이형성된다는것과, 둘째로종양색전에의해원위부폐실질이괴사되어낭종이형성된다는주장이있다. 셋째로는, 원래존재하던폐낭포주변으로종양세포가침윤한다는이론이있고, 넷째로종양이작은기관지를눌러서체크밸브역활을하여폐포를과다팽창시켜폐실질의낭성변화를일으킨다는것이다 7. 본증례의경우는폐의조직생검에서낭종의벽을이루는종양세포가관찰되지않아네번째이론에의한낭성변화로생각되며, 이로인해이차성기흉이발생한것으로여겨지나, 이런변화에대한연구는아직도진행중이다. Kitagawa 등 5 은 95예의부검을통해두피에서기원한혈관육종이다른부위에서기원한경우보다폐전이가더잘되어호흡기계합병증을더잘일으키는것으로보고하였으며, 특히기흉은두피기원의혈관육종환자에서만발견되었고매우불량한예후를나타낸다고하였다. 본 증례는 2년전두피의혈관육종을진단받고광범위절제술및수술후방사선치료를받은병력이있었으며당시시행하였던흉부전산화단층촬영상에서폐전이를시사하는소견은없었다. 2년후호흡기계증상이계속되어외래내원시촬영하였던양전자단층촬영-전산화단층촬영상에서폐의낭종이형성되어있었고병이진행하면서낭종이터져기흉이발생하여응급실로내원한경우였다. 기흉이재발하여다시내원했을때시행한신체검진에서두피의병변이새로이발견되어폐의쐐기절제술및두피조직검사를함께시행하여두피에서혈관육종의재발을확인하였으나폐조직검사에서는종양세포를관찰할수없었다. 혈관육종의폐전이를조직검사상에서확진할수없었으나병의진행경과를보고임상적으로폐전이를진단하였다. 두피의혈관육종을처음진단받았을당시의흉부전산화단층촬영상에서폐에서낭종이관찰되지않았으나호흡기증상과함께낭종이발견되기시작하였으며, 추적검사상에서폐의낭종이커지고출혈과기흉이반복되며진행하였기때문이다. 377

5 CJ Lee et al: Cystic lung metastasis of angiosarcoma 혈관육종의치료로는광범위절제술및수술후방사선요법이지만, 종양의범위를결정하기어렵고제거하기힘든위치에발생하는경우때문에완전히절제하기가어렵다. Pawlik 등 8 에따르면두피의혈관육종으로광범위절제술을받은 28명의환자중에서 6명 (21%) 만이절제후가장자리에서종양세포가발견되지않았다. 이연구에서 28명중 23명이방사선요법을받았으며, 방사선요법이국소부위의치료에도움이된다고주장하였다. 전신항암약물요법이혈관육종에효과가있다는것은입증되지않았으나, paclitaxel 이피부병변및폐전이에효과가있다는최근의연구보고들이있다 9,10. 본증례에서도 paclitaxel 로전신항암약물요법을 12회투여하였으나, 암전이가진행하여사망하였다. 본증례는혈관육종을진단받고치료받아관해를이룬상태에서객혈등의호흡기계증상을보여시행한영상학적검사상폐의낭종을진단받고이후낭종이터져기흉이발생되었던경우였다. 노년층에서기흉발생시에종양에의한이차성기흉을꼭감별하여야하며, 폐조직검사상종양세포가발견되지않더라도혈관육종의경우는폐전이소견이낭성변화일수있으므로이에대한확인이필요하다고판단되며문헌고찰과함께보고하는바이다. 요약혈관육종은매우드문악성종양으로고령남자의두피나얼굴에주로발생하며, 폐로전이되는경우가있다. 폐전이의경우객혈, 기흉등의호흡기계증상을일으킬수있다. 저자들은혈관육종의폐전이가낭성변화를일으켜기흉및혈흉을초래한 1예를경험하였기에문헌고찰과함께보고하는바이다. 1. Orchard GE, Zelger B, Jones EW, Jones RR. An immunocytochemical assessment of 19 cases of cutaneous angiosarcoma. Histopathology 1996;28: Mendenhall WM, Mendenhall CM, Werning JW, Reith JD, Mendenhall NP. Cutanoues angiosarcoma. Am J Clin Oncol 2006;29: Holden CA, Spittle MF, Jones EW. Angiosarcoma of the face and scalp, prognosis and treatment. Cancer 1987; 59: Mark RJ, Tran LM, Sercarz J, Fu YS, Calcaterra TC, Juillard GF. Angiosarcoma of the head and neck. The UCLA experience 1955 through Arch Otolaryngol Head Neck Surg 1993;119: Kitagawa M, Tanaka I, Takemura T, Matsubara O, Kasuga T. Angiosarcoma of the scalp: report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data. Virchows Arch A Pathol Anat Histopathol 1987;412: Tateishi U, Hasegawa T, Kusumoto M, Yamazaki N, Iinuma G, Muramatsu Y, et al. Metastatic angiosarcoma of the lung: spectrum of CT findings. AJR Am J Roentgenol 2003;180: Sakurai H, Hada M, Miyashita Y, Tsukamoto K, Oyama T, Ashizawa I. Simultaneous bilateral spontaneous pneumothorax secondary to metastatic angiosarcoma of the scalp: report of a case. Surg Today 2006;36: Pawlik TM, Paulino AF, McGinn CJ, Baker LH, Cohen DS, Morris JS, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer 2003;98: Fata F, O'Reilly E, Ilson D, Pfister D, Leffel D, Kelsen DP, et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 1999;86: Budd GT. Management of angiosarcoma. Curr Oncol Rep 2002;4: 참고문헌 378

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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