Case Report pissn 1738-2637 / eissn 2288-2928 J Korean Soc Radiol 2013;69(5):399-403 http://dx.doi.org/10.3348/jksr.2013.69.5.399 Contrast-Enhanced Ultrasound and Computed Tomography Findings of Recurrent Ovarian Steroid Cell Tumor Presenting with Peritoneal Seeding: A Case Report 1 복막파종으로재발한난소스테로이드세포종양의조영증강초음파및전산화단층촬영소견 : 증례보고 1 A Lan Im, MD 1, Young Hwan Lee, MD 1, Hye Won Kim, MD 1, Han Ah Lee, MD 1, Keum Ha Choi, MD 2 Departments of 1 Radiology, 2 Pathology, Wonkwang University School of Medicine & Hospital, Iksan, Korea We present ultrasonography and computed tomography (CT) findings of a case of recurrent ovarian steroid cell tumor presenting with peritoneal seeding in a 45-year-old woman. On abdominal ultrasonography, there were multiple hypoechoic round masses in the peritoneal cavity including the perihepatic area. Contrast-enhanced ultrasonography showed intense homogenous enhancement on the arterial phase and delayed prolonged enhancement of the masses. CT revealed multiple peritoneal solid masses with strong enhancement. Five years ago, the patient had been diagnosed with a steroid cell tumor of the left ovary. At that time, the CT showed a well-enhancing, lobulating, large solid mass at the left adnexa. Imaging findings of the peritoneal masses suggested peritoneal seeding from the preexisting ovarian steroid cell tumor. For treatment of the metastatic lesions in the perihepatic area, ultrasonography-guided radiofrequency ablation (RFA) was performed, and debulking surgery for the peritoneal masses was done. Six months later, complete ablation of the perihepatic metastases by RFA and a marked decrease in the peritoneal metastases by surgery were found on the follow-up CT. Index terms Ovarian Steroid Cell Tumor Recurrent Peritoneal Metastasis Peritoneal Seeding Treatment Received May 29, 2013; Accepted September 13, 2013 Corresponding author: Young Hwan Lee, MD Department of Radiology, Wonkwang University School of Medicine & Hospital, 895 Muwang-ro, Iksan 570-711, Korea. Tel. 82-63-859-1920 Fax. 82-63-851-4749 E-mail: yjyh@wonkwang.ac.kr This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. This paper was supported by Wonkwang University in 2012. 서론 스테로이드세포종양 (steroid cell tumors; 이하 SCTs) 은난소의성끈간질종양 (sex cord stromal tumors) 의드문하위집단에속하며 (1), 호르몬활성과남성화와관련된흥미로운임상양상을나타내는양성난소종양이다 (2). 대부분의스테로이드세포종양은초기에발견되어수술적치료가가능하나드물게재발이나전이를보일수있고, 항암화학요법이나방사선치료에대한이들의반응은거의알려져있지않다 (2). 또한전이의형태로재발한난소스테로이드세포종양의영상소견에대해이전에보고된바없다. 이에저자들은복막파종의형태로재발한 난소스테로이드세포종양의초음파검사 (ultrasonography) 와전산화단층촬영 (computed tomography; 이하 CT) 의영상의학적소견과그치료및경과를보고하고자한다. 증례보고 45세여자환자가복부팽만과다모증을주소로내원하였다. 내원당시혈청총테스토스테론은 0.046 ng/ml 로낮았고, cancer antigen 125 는 539.6 IU/mL 로매우높게측정되었다. 복부 CT에서좌측부속기에약 7.7 6.6 cm 크기의조영증강이잘되는분엽형의고형종괴가관찰되었고조영전영상에 Copyrights 2013 The Korean Society of Radiology 399
복막파종으로재발한난소스테로이드세포종양의조영증강초음파및전산화단층촬영소견 서종괴내뚜렷한지방성분은보이지않았으며, 다량의복수가동반되어있었다 (Fig. 1A). 혈청학적검사와영상학적소견으로좌측난소에서기원한악성종양이의심되었다. 이후자궁절제술과양측자궁부속기절제술을시행하였고, 병리학적으로좌측부속기에서기원한저등급의다른특정군에포함되지않는스테로이드세포종양 (grade 1 SCT, not otherwise specified) 으로진단되었다 (Fig. 1B). 5년 10개월뒤, 환자는건강검진으로시행한복부초음파검사에서간종괴가의심되어진단및치료를위해전원되었다. 본원에서시행한조영증강초음파검사에서간의 3분절과 6분절, 위간인대, 간십이지장인대, 대망과장간막을포함하여복강내에원형의저에코성종괴가다수발견되었다 (Fig. 2A, B). 이종괴들은조영증강초음파검사의동맥기에서강하고균질한조영증강을보였으며지연기까지지속적인조영증강을보였다 (Fig. 2C, D). 복부 CT에서도복강내에다수의경계가뚜렷한원형의종괴가관찰되었으며동맥기에서지연기까지강한조영증강을보였다. 원인대열 (fissure for ligamentum teres) 과간주위복막에위치한 1 cm와 2.4 cm 크기의두개의종괴는간과매우인접하고있어각각간의 3분절과 6분절에서발생한간종괴처럼보였다 (Fig. 2E-G). 조직학적진단을위하여간종괴에서침생검을시행하였고, 병리학적으로이전수술로진단된난소종양과동일한조직학적및면역염색결과를보여난소의스테로이드세포종양의복막전이로진단하였다. 다수의복막종괴에대한치료로서용적축소술 (debulking surgery) 을시행하기로하였고, 간과원인대열의병변에대하여 수술적간절제대신초음파유도하고주파소작술 (ultrasonography-guided radiofrequancy ablation) 을시행하였다. 복막종괴의수술적절제생검에서병리학적으로이전난소종양과같은소견을보여난소스테로이드세포종양의복막파종으로확진하였다 (Fig. 2H). 6개월후시행한추적관찰복부및골반 CT에서, 초음파유도하고주파소작술을시행한간내종괴는완전소작되어있었고, 용적축소술을시행한복막전이병변의수와크기가뚜렷하게감소되어있었다. 더이상의국소재발은보이지않았으며이후환자는추적관찰중이다. 고찰 스테로이드세포종양은모든난소종양의 0.1% 이하를차지하는드문종양으로, 성끈간질종양의하위집단으로분류된다. 대개양성이고일측성으로나타나며세포내지방증식이특징적이다 (1, 2). 스테로이드세포종양은다시기질황체종 (stromal leuteomas), 레이디히세포종양 (Leydig cell tumors), 그리고다른특정군에포함되지않는스테로이드세포종양 (SCTs, not otherwise specified) 의세가지로분류된다. 다른특정군에포함되지않는스테로이드세포종양은가장흔한종류로약 60% 정도를차지하며대부분남성화증상을나타내고, 25~45% 는임상적으로악성의경과를보인다 (3-5). 어느연령에서나발생할수있으나, 대개성인에서호발하며평균발생연령은 43세이다 (4). A B Fig. 1. Ovarian steroid cell tumor in a 45-year-old female patient. A. Initial abdominopelvic CT shows 7.7 6.6 cm sized well-enhancing solid mass with lobulating contour in left ovary suggesting ovarian cancer. Large amount of ascites was also noted. B. Photomicrograph of the ovarian mass ( ) shows round nuclei and clear cytoplasm suggesting steroid cell tumor. Note the waving appearance of the nuclei in normal ovarian stroma (*) (hematoxylin-eosin, 200). 400 대한영상의학회지 2013;69(5):399-403 jksronline.org
임아란외 A B C D E F G H Fig. 2. Recurrent ovarian steroid cell tumor presenting peritoneal seeding after 5 years and 10 months in same patient of Fig. 1. A, B. Abdominal ultrasonography shows multiple well-defined hypoechoic masses in the liver and peritoneal cavity including 3.0 2.1 cm sized mass in hepatic segment 6 (A) and 1.4 1.3 cm sized mass in hepatic segment 3 (not shown). Color Doppler ultrasound image shows slightly increased vascular flows at peripheral portion of the masses (B). C, D. Contrast-enhanced ultrasonography shows intense homogenous enhancement of the hepatic mass on arterial phase (C) and delayed prolonged contrast enhancement of the omental masses (D). E-G. Abdominopelvic CT shows multiple well-enhancing round masses (arrows) in the peritoneum, including perigastric, gastrohepatic and hepaticoduodenal ligaments, and mesenteric areas and fissure for ligamentum teres (E). Note another well-enhancing round mass (arrow) which is thought to be existing in segment 6 of the liver (F). But, axial image adapted from F shows that the mass is an extrahepatic mass (arrow) which is grown from accessory hepatic fissure and interrupting hepatic parenchyme (G). H. Photomicrographs of the omental mass ( ) shows round nuclei and clear cytoplasm suggesting metastatic steroid cell tumor from preexisting ovarian steroid tumor. Note rich vascularity (arrows) of the mass (hematoxylin-eosin, 200). 스테로이드세포종양은일반적으로무월경, 얼굴과사지에비정상적발모 (hair growth), 12~50% 정도에서는탈모등남성호르몬과관련된증상 (androgenic symptom) 이나타나며, 증상들이뚜렷해지기까지오랜시간이걸린다 (1). 징후와증상들은대체로다음과같은순서로나타난다. 질병초기에뜸한월경 (oligomenorrhea) 과신체에미미한비정상적발모가보이고, 이후에무월경, 유방과다른여성외부생식기의퇴행, 다모증, 여드름, 음핵비대 (clitoral hypertrophy), 그리고탈모가생긴다 (6). 난소스테로이드세포종양은조직학적으로세포내지방성분을많이포함하는특징을보인다. 풍부한투명세포질 (clear cytoplasm) 과다양한정도의세포내지방성분은스테로이드를분비하는부신피질세포 (adrenocortical cells) 와유사하고, 풍부한혈관분포를나타낸다 (7). 종양세포들은뚜렷한세포경계와중심핵을가진다각형이나원형의세포로, 두드러진핵소 jksronline.org 대한영상의학회지 2013;69(5):399-403 401
복막파종으로재발한난소스테로이드세포종양의조영증강초음파및전산화단층촬영소견 체를보이기도한다 (8). 영상의학적으로스테로이드세포종양은대부분경계가명확한일측성의고형종괴로나타나며, 작은낭성또는괴사부위가동반되기도한다 (7). CT와자기공명영상소견은포함된지방성분과섬유성기질의양에따라다양하다. 그러나대개종양세포내지방성분이많기때문에자기공명영상 T1 강조영상에서고신호강도를나타내며, 조영증강검사에서대부분강한조영증강을나타내는데이는종양의풍부한혈관분포를반영하는소견이다 (9, 10). 본증례에서도 CT뿐아니라조영증강초음파검사에서이러한과혈관성을반영하는강한조영증강을나타내었고, 조직학적소견에서풍부한혈관분포를확인할수있었다 (Fig. 2G). 이와비슷하게난소의과혈관성종양으로나타나는종양으로경화기질종양 (sclerosing stromal tumor) 이나카르시노이드 (carcinoid), 또는다른과혈관성형태의전이종양과의감별이필요하다. 경화기질종양의경우스테로이드세포종양과같은성끈간질종양에속하는종양으로서임상증상이비슷하게나타나나스테로이드세포종양과달리주로 10~20 대의비교적이른나이에발생하며, 고형성종괴에낭성부위가중앙에위치한형태가많다. 카르시노이드는보통폐경기이후여성에서생기며성숙기형종이나점액종양이동반되는경우가있다. 신세포암등에서기원한과혈관성전이종양의경우원발병소의유무와임상증상및진단검사의학적소견으로감별할수있다. 대부분의스테로이드세포종양이초기에발견되고재발이나전이를보이지않는다고알려져있으나 (3), 본증례는원발종양을수술적으로제거한뒤 5년 10개월후에광범위한복막파종형태로재발되었으며, 원인대열을포함하여간주위에발생한일부전이종양은간종양과감별을요할정도로간실질안에위치해있었다. 전이종양이모두경계가명확하며비교적주변조직으로의침습을보이지않는경우용적축소술을치료방침으로세울수있다. 본증례에서원인대열과간제6분절에위치한전이종양에대하여는간절제로치료하는대신초음파유도하고주파소작술을시행하였고, 나머지복막전이종괴에대하여수술적절제를시행하였다. 이후추적검사로시행한복부및골반 CT에서고주파소작부위에국소적재발의소견은보이지않았으며, 대부분의복막의다발성전이종양은용적축소술에의해성공적으로치료되어있었다. 결론적으로본증례는복강내에다발성의경계가명확한과혈관성전이성종괴로재발한난소스테로이드세포종양으로, 간내종괴형태로나타난전이병변에대하여간절제대신초음파유도하고주파소작술을통하여성공적으로치료하였기에조영증강초음파검사및 CT 등영상의학적소견을함께보고한다. 참고문헌 1. Young RH, Shully RE. Steroid cell tumors of the ovary. In Fox H, Wells M. Obstetric & Gynecological Pathology. Spain: Churchill Livingstone, 2003:845-856 2. Haji AG, Sharma S, Babu M, Vijaykumar D, Chitrathara K. Androgen secreting steroid cell tumor of the ovary in a young lactating women with acute onset of severe hyperandrogenism: a case report and review of literature. J Med Case Rep 2007;1:182 3. Ye L, Wu XL, Xu L, Huang Q, Sun L, He Y, et al. [Ovarian steroid cell tumor, not otherwise specified: a clinicopathologic study]. Zhonghua Bing Li Xue Za Zhi 2007;36:516-520 4. Kim YT, Kim SW, Yoon BS, Kim SH, Kim JH, Kim JW, et al. An ovarian steroid cell tumor causing virilization and massive ascites. Yonsei Med J 2007;48:142-146 5. Liu AX, Sun J, Shao WQ, Jin HM, Song WQ. Steroid cell tumors, not otherwise specified (NOS), in an accessory ovary: a case report and literature review. Gynecol Oncol 2005; 97:260-262 6. Boyraz G, Selcuk I, Yusifli Z, Usubutun A, Gunalp S. Steroid cell tumor of the ovary in an adolescent: a rare case report. Case Rep Med 2013;2013:527698 7. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex cord-stromal and steroid cell tumors of the ovary. Radiographics 1998;18:1523-1546 8. Al-Farsi A, Al-Talib A. Steroid cell tumor. Sultan Qaboos Univ Med J 2008;8:358-359 9. Jung SE, Rha SE, Lee JM, Park SY, Oh SN, Cho KS, et al. CT and MRI findings of sex cord-stromal tumor of the ovary. AJR Am J Roentgenol 2005;185:207-215 10. Saida T, Tanaka YO, Minami M. Steroid cell tumor of the ovary, not otherwise specified: CT and MR findings. AJR Am J Roentgenol 2007;188:W393-W394 402 대한영상의학회지 2013;69(5):399-403 jksronline.org
임아란외 복막파종으로재발한난소스테로이드세포종양의조영증강초음파및전산화단층촬영소견 : 증례보고 1 임아란 1 이영환 1 김혜원 1 이한아 1 최금하 2 45세여자환자에서복막파종으로재발한난소의스테로이드세포종양의초음파검사와전산화단층촬영을포함한영상의학적소견을보고하고자한다. 복부초음파검사에서복강내에원형의저에코로보이는종괴들이다수발견되었고, 조영증강초음파검사와역동적조영증강전산화단층촬영에서동맥기에서지연기까지강하고균질한조영증강을보였다. 5 년전, 환자는좌측난소의스테로이드세포종양으로수술적치료를받은기왕력이있었으며, 당시전산화단층촬영에서좌측부속기에조영증강이매우잘되는분엽형종괴와다량의복수가관찰되었다. 복강내종괴의영상의학적소견은난소스테로이드세포종양에서재발한복막파종을시사했다. 간주변에위치한전이병변에대해초음파유도하고주파소작술을시행하였고, 그외의복막전이병변에대한용적축소술을시행하였다. 6개월후전산화단층촬영에서고주파소작술과수술을통한복막전이병변의성공적인치료를확인할수있었다. 원광대학교의과대학원광대학교병원 1 영상의학과, 2 병리과 jksronline.org 대한영상의학회지 2013;69(5):399-403 403