CT and MRI Findings of Sex Cord Stromal Tumor of the Ovary

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1 T and MRI of Ovarian Tumors Obstetric and Gynecologic Imaging Pictorial Essay Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Seung Eun Jung 1 Sung Eun Rha 1 Jae Mun Lee 1 Soo Youn Park 1 Soon Nam Oh 1 Kyoung Sik ho 2 Eun Ju Lee 3 Jae Young yun 1 Seong Tai Hahn 1 Jung SE, Rha SE, Lee JM, et al. Received July 9, 2004; accepted after revision September 22, Department of Radiology, St. Mary s Hospital, ollege of Medicine, The atholic University of Korea, #62, Youidodong, Youngdeungpo-gu, Seoul , South Korea. ddress correspondence to S. E. Jung (sejung@catholic.ac.kr). 2 Department of Radiology, san Medical enter, University of Ulsan, Seoul , South Korea. 3 Department of Radiology, jou University, ollege of Medicine, Paldal-gu, Suwon, Kyunggi-do, South Korea. JR 2005;185: X/05/ merican Roentgen Ray Society T and MRI Findings of Sex ord Stromal Tumor of the Ovary OJETIVE. The purpose of this article was to research the clinical and imaging features of sex cord stromal tumors of the ovary to help in specific diagnosis of ovarian tumors. Sex cord stromal tumors of the ovary are rare ovarian neoplasms, which arise from stromal cells and primitive sex cords in the ovary. The common types are granulosa cell tumors, fibrothecomas, sclerosing stromal tumors, and Sertoli-Leydig cell tumors. They account for most of the hormonally active ovarian tumors. They have characteristic imaging features in each type of the tumor. ONLUSION. linical and radiologic clues are helpful in differential diagnosis from the more common epithelial tumors; sex cord stromal tumors primarily are treated surgically and have generally good prognosis. ex cord stromal tumors of the S ovary are rare, making up approximately 8% of all ovarian neoplasms. These tumors arise from two groups of cells in the ovary: stromal cells and primitive sex cords. Stromal cells contain fibroblasts, theca cells, and Leydig cells, and primitive sex cords include granulosa cells in the normal ovary, Sertoli cells in the testis, and Sertoli cells in ovarian tumors [1]. They differ from the more common epithelial neoplasms in clinical and radiologic aspects. Understanding the clinical and imaging features of sex cord stromal tumor of the ovary is helpful in specific diagnosis of ovarian tumors. Sex cord stromal tumors of the ovary affect all age groups and account for most of the hormonally active ovarian tumors that show estrogenic effects or virilization. Unlike patients with common epithelial tumors, of which 75% are considered to be at stage III or IV at diagnosis, approximately 70% of patients with these tumors are classified as having stage I lesions at presentation. onsequently, sex cord stromal tumors are primarily treated surgically and have a generally good prognosis. In addition, these tumors may have characteristic imaging features [1, 2]. In this article, we show T and MRI findings of relatively common sex cord stromal tumors of the ovary and discuss points that differentiate these tumors from more common ovarian epithelial tumors. Granulosa ell Tumors Granulosa cell tumors of the ovary account for less than 5% of all malignant ovarian tumors but represent the most common malignant sex cord stromal tumor and the most common clinically estrogenic ovarian tumor. dult granulosa cell tumors appear more often than the juvenile type and occur usually in postmenopausal women. They can present with abnormal vaginal bleeding and can be associated with endometrial hyperplasia, polyps, and carcinoma (3 25% of cases). Granulosa cell tumors have potential for clinically malignant behavior. Prognosis correlates with stage and age at the time of the diagnosis. Most patients with these tumors have an excellent prognosis (> 90% having a 10-year survival rate). They have a tendency of late recurrence, even years after diagnosis [1 3]. dult granulosa cell tumors show a spectrum of imaging manifestations due to various histologic appearances and various arrangements of tumor cells. They can have the appearance of solid masses (Fig. 1), tumors with hemorrhagic or fibrotic changes, multilocular cystic lesions (Figs. 2 and 3), or completely cystic tumors. Heterogeneity within a solid tumor is caused by intratumoral bleeding, infarct, fibrous degeneration, and irregularly arranged tumor cells (Fig. 3). Multilocular cystic appearances are produced by a predominantly macrofollicular pattern of JR:185, July

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, fter contrast administration, T scan shows mass (arrows) as mildly and homogeneously enhanced.

In contrast to the more common epithelial tumors, granulosa cell tumors are confined to the ovary at the time of diagnosis with less propensity for peritoneal seeding Fig.

, xial T1-weighted image obtained after gadolinium administration shows marked enhancement of septa and solid portions with multiple small cystic areas within tumor, resulting in spongy

2 Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Fig year-old woman with granulosa cell tumor., Unenhanced T scan shows solid soft-tissue mass (arrows) in right adnexal area., fter contrast administration, T scan shows mass (arrows) as mildly and homogeneously enhanced. granulosa cell tumor, and these multiple cystic spaces are filled with watery fluid or hemorrhage. In contrast to the more common epithelial tumors, granulosa cell tumors are confined to the ovary at the time of diagnosis with less propensity for peritoneal seeding Fig year-old woman with granulosa cell tumor., xial T2-weighted image shows well-defined cystic tumor (arrows) with multiple small chambers., xial T1-weighted image obtained after gadolinium administration shows marked enhancement of septa and solid portions with multiple small cystic areas within tumor, resulting in spongy appearance. and are only rarely bilateral. They may rupture and result in hemoperitoneum [2 5]. Fibroma, Fibrothecoma, and Thecoma Fibroma, fibrothecoma, and thecoma form a spectrum of benign tumors of the ovary. These tumors constitute approximately 4% of all ovarian neoplasms and occur in both pre- and postmenopausal women. Fibroma is the most common sex cord tumor. lthough fibroma arising from nonfunctioning stroma shows no estrogenic activity, lipid-rich thecoma can show es- 208 JR:185, July 2005

T and MRI of Ovarian Tumors Downloaded from www.ajronline.org by 37.44.207.121 on 01/23/18 from IP address 37.44.207.121. opyright RRS. For personal use only; all rights reserved trogenic activity.

ecause fibromas have abundant collagen and fibrous contents, these tumors show relatively diagnostic imaging findings. The mass appears as a homogeneous solid tumor with delayed enhancement on T (Fig.

, xial T1-weighted image shows large well-defined mass with multiple cystic areas of extremely low signal intensity and tiny spots (arrows) with high signal intensity that represent intratumoral

, xial T1-weighted image obtained after gadolinium administration shows marked enhancement of solid components in tumor. Fig. 4 34-year-old woman with exophytic fibroma.

, fter contrast administration, T scan shows homogeneously enhanced anterior mass (arrows) as pedunculated fibroma and posterior heterogeneous structure as right ovary.

3 T and MRI of Ovarian Tumors Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved trogenic activity. Fibromas can be associated with Meigs syndrome (ascites, an ovarian tumor, and a right-sided pleural effusion). Fibrosarcomas are rare [1, 2, 6]. ecause fibromas have abundant collagen and fibrous contents, these tumors show relatively diagnostic imaging findings. The mass appears as a homogeneous solid tumor with delayed enhancement on T (Fig. 4) Fig year-old woman with granulosa cell tumor., xial T1-weighted image shows large well-defined mass with multiple cystic areas of extremely low signal intensity and tiny spots (arrows) with high signal intensity that represent intratumoral hemorrhage., xial T2-weighted image shows large multicystic mass with some solid portion. This is called bunch-of-grapes appearance of cystic tumor with multiple chambers., xial T1-weighted image obtained after gadolinium administration shows marked enhancement of solid components in tumor. Fig year-old woman with exophytic fibroma., Unenhanced T scan shows dumbbell-shaped mass in right adnexal area., fter contrast administration, T scan shows homogeneously enhanced anterior mass (arrows) as pedunculated fibroma and posterior heterogeneous structure as right ovary. and as a hypointense mass on T1-weighted MRI with very low signal intensity on T2- weighted imaging (Fig. 5). Dense calcifications are often seen. Scattered high-signal-intensity areas in the mass indicate edema or cystic degeneration [2] (Fig. 6). Fibroma can be a cause of adnexal torsion. The T and MRI findings in adnexal torsion with fibroma include tube thickening, ascites, deviation to the twisted side, hemorrhage in the thickened tube, and torsion knot. It is difficult to diagnose hemorrhagic infarction after adnexal torsion because the fibroma is a solid tumor. The finding of a high-signal-intensity area in the periphery of the mass on T1-weighted imaging is helpful in diagnosis of hemorrhagic infarction of fibroma [7] (Fig. 7). JR:185, July

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5 28-year-old woman with bilateral fibromas.

masses are noted in both adnexal regions on T2-weighted images.

Ovarian follicles (arrows) are noted in left adnexal area. Fig.

, xial T1-weighted image shows well-defined mass with low signal intensity in pelvic

4 Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Fig year-old woman with bilateral fibromas. and, Upper pelvic level () and lower pelvic level () multilobulated low-signal-intensity masses are noted in both adnexal regions on T2-weighted images. Masses have typical dark signal intensity. Ovarian follicles (arrows) are noted in left adnexal area. Fig year-old woman with fibrothecoma., xial T1-weighted image shows well-defined mass with low signal intensity in pelvic cavity., On axial T2-weighted image, mass shows markedly heterogeneous signal intensity., Gadolinium-enhanced fat-suppressed T1-weighted image reveals intense enhancement in most of mass. Some areas of high signal intensity on T2-weighted image are not enhanced, which represent cystic change and edema. 210 JR:185, July 2005

T and MRI of Ovarian Tumors Downloaded from www.ajronline.org by 37.44.207.121 on 01/23/18 from IP address 37.44.207.121. opyright RRS.

and, Sagittal () and axial () T2-weighted images show well-circumscribed solid mass with heterogeneous dark signal intensity in left adnexal region.

Note whorled structure (arrows) abutting anterior margin of ovarian mass, finding that suggests twisted vascular pedicle.

D, fter gadolinium administration, mass is faintly enhanced in areas of low signal intensity on T2-weighted image.

5 T and MRI of Ovarian Tumors Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Fig year-old woman with torsion of left ovarian fibroma. She presented with aggravation of back pain. and, Sagittal () and axial () T2-weighted images show well-circumscribed solid mass with heterogeneous dark signal intensity in left adnexal region. Increased signal intensity area of anterior aspect of mass represents edema. Note whorled structure (arrows) abutting anterior margin of ovarian mass, finding that suggests twisted vascular pedicle., xial T1-weighted image shows round homogeneous low-signal-intensity mass. D, fter gadolinium administration, mass is faintly enhanced in areas of low signal intensity on T2-weighted image. Ovarian mass proved to be fibroma without any congestion or necrosis at surgery. Torsion of pedicle was 270. D Sclerosing Stromal Tumor of Ovary Sclerosing stromal tumors are rare benign ovarian tumors that occur predominantly in young women. The most common clinical symptom is menstrual irregularity. scites may be seen but is rare. Surgical removal of the tumor is curative, and there is no local or distant recurrence [2, 8]. Imaging findings include a large mass with hyperintense cystic components (Fig. 8) or a heterogeneous solid mass of intermediate to high signal intensity on T2-weighted JR:185, July

Downloaded from www.ajronline.org by 37.44.207.121 on 01/23/18 from IP address 37.44.207.121. opyright RRS. For personal use only; all rights reserved MRI (Fig. 9).

8 and 9). On dynamic contrastenhanced images, the tumors reveal early peripheral enhancement with centripetal progression.

6 Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved MRI (Fig. 9). The thick peripheral hypointense rim on T2-weighted imaging is a compressed ovarian cortex due to a slowgrowing tumor. There is striking contrast enhancement with internal small cleft and cysts (Figs. 8 and 9). On dynamic contrastenhanced images, the tumors reveal early peripheral enhancement with centripetal progression. Striking early enhancement reflects the cellular areas with their prominent vascular networks, and an area of prolonged enhancement in the inner portion of the mass represents the collagenous hypocellular area. These findings can be useful in differentiating sclerosing stromal tumor from fibroma because fibroma shows absence of early enhancement and delayed accumulation of the contrast material [8 10]. Sertoli-Leydig ell Tumor Sertoli-Leydig cell tumors are the most common virilizing ovarian tumor, but they Fig year-old woman with sclerosing stromal tumor. She complained of vaginal spotting during ovulation., xial T1-weighted image shows well-defined pelvic mass with slightly hyperintense peripheral portion (arrows) and irregular central hypointense area., xial T2-weighted image revealed mass with marked hyperintense central area and slightly hyperintense periphery (arrows)., Gadolinium-enhanced fat-suppressed T1-weighted image reveals very intense enhancement of periphery of tumor. are very rare (< 0.5% of ovarian tumors). They occur usually in young women. In 40 50% of patients, the presenting symptoms are related to clinical signs of androgenic activity [1]. The tumors are microscopically ranged from well-differentiated to poorly differentiated. ehavior correlates with degree of differentiation and stage. Most of these tumors behave in a benign fashion [11]. In contrast to granulosa cell tumors, Sertoli-Leydig cell tumors tend to 212 JR:185, July 2005

She had menstrual irregularity and lower abdominal discomfort.

Mass has homogeneous intermediate signal intensity. Flow voids are also seen between mass and ovary.

t surgery, pedunculated sclerosing stromal tumor from ovary was confirmed. Fig.

7 T and MRI of Ovarian Tumors Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Fig year-old woman with sclerosing stromal tumor. She had menstrual irregularity and lower abdominal discomfort., Sagittal T2-weighted image shows round mass (arrows) protruding from ovary. Mass has homogeneous intermediate signal intensity. Flow voids are also seen between mass and ovary., Gadolinium-enhanced T1-weighted sagittal images show very intense enhancement of mass. t surgery, pedunculated sclerosing stromal tumor from ovary was confirmed. Fig year-old woman with poorly differentiated Sertoli-Leydig cell tumor., xial T1-weighted image shows lobulated solid mass with low signal intensity (arrows). and, xial () and sagittal () T2-weighted images show multilobulated mass with intermediate signal intensity with multiple tiny high-signal cysts (arrows). (Fig. 10 continues on next page) JR:185, July

and, xial () and sagittal () T2-weighted images show multilobulated mass with intermediate signal intensity with multiple tiny high-signal cysts (arrows).

11 41-year-old woman with steroid cell tumor. She presented with amenorrhea and weight gain. Laboratory findings revealed elevated testosterone level.

Ill-defined area of high signal intensity is noted, indicating lipid content., On T2-weighted image, mass (arrows) is heterogeneously hyperintense. (Fig.

8 Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved Fig. 10 (continued) 56-year-old woman with poorly differentiated Sertoli-Leydig cell tumor. and, xial () and sagittal () T2-weighted images show multilobulated mass with intermediate signal intensity with multiple tiny high-signal cysts (arrows). D, fter gadolinium administration, fat-suppressed T1-weighted image shows that mass is intensely and heterogeneously enhanced. Fig year-old woman with steroid cell tumor. She presented with amenorrhea and weight gain. Laboratory findings revealed elevated testosterone level., xial T1-weighted image shows small (~ 3 cm) ovoid hypointense mass (arrows) in left adnexal region. Ill-defined area of high signal intensity is noted, indicating lipid content., On T2-weighted image, mass (arrows) is heterogeneously hyperintense. (Fig. 11 continues on next page) D recur relatively soon after initial diagnosis. They are almost always unilateral tumors that can be solid, solid and cystic, and cystic or even papillary. The mass appears as a well-defined enhancing solid mass with intratumoral cysts on T and as hypointense with multiple variable-sized cystic areas on MRI (Fig. 10). Low signal intensity on T2- weighted imaging depends on the extent of 214 JR:185, July 2005

T and MRI of Ovarian Tumors Fig. 11 (continued) 41-year-old woman with steroid cell tumor. She presented with amenorrhea and weight gain. Laboratory findings revealed elevated testosterone level.

9 T and MRI of Ovarian Tumors Fig. 11 (continued) 41-year-old woman with steroid cell tumor. She presented with amenorrhea and weight gain. Laboratory findings revealed elevated testosterone level., fter gadolinium administration, coronal fat-suppressed T1-weighted image shows tumor (arrows) is very intensely enhanced. Downloaded from by on 01/23/18 from IP address opyright RRS. For personal use only; all rights reserved fibrous stroma. Multicystic areas develop as a result of heterologous elements [1, 2]. Steroid ell Tumor Steroid cell tumors are very rare ovarian tumors that affect patients from a wide range of ages but usually those in the fifth or sixth decade of life. Most steroid cell tumors are virilizing, and rare cases are associated with ushing s syndrome. pproximately one third of these tumors behave in a clinically malignant fashion. Steroid cell tumors are usually small (< 3 cm) nodules and virtually always unilateral. The tumor manifests as a small mass with hyperintense areas on T1- weighted images due to abundant intracellular lipid and intense enhancement due to rich vascularity [1, 12] (Fig. 11). Summary Sex cord stromal tumors of the ovary are rare ovarian neoplasms that arise from stromal cells and primitive sex cords in the ovary. These tumors may have characteristic clinical and radiologic features. They affect all age groups and account for most of the hormonally active ovarian tumors. Most of them are stage I lesions at presentation with good prognosis. Granulosa cell tumors are usually large multiloculated cystic masses with variable solid portions. The tumors are associated with endometrial abnormalities. Fibromas and thecomas are usually solid masses with dark signal intensity on T2-weighted MRI and variable extent of calcification or degeneration. Sclerosing stromal tumors show typical early peripheral enhancement with centripetal progression. Sertoli-Leydig cell tumors appear as well-defined, enhancing solid masses with variable-sized intratumoral cysts. Steroid cell tumors show a heterogeneous solid mass with internal areas of intracellular lipid. References 1. Outwater EK, Wagner J, Mannion, McLarney JK, Kim. Sex cord-stromal and steroid cell tumors of the ovary. RadioGraphics 1998; 18: Jung SE, Lee JM, Rha SE, yun JY, Jung JI, Hahn ST. T and MR imaging of ovarian tumors with emphasis on differential diagnosis. RadioGraphics 2002; 22: Ko SF, Wan YL, Ng SH, et al. dult ovarian granulosa cell tumors: spectrum of sonographic and T findings with pathologic correlation. JR 1999; 172: Kim SH, Kim SH. Granulosa cell tumor of the ovary: common findings and unusual appearances on T and MR. J omput ssist Tomogr 2002; 26: Morikawa K, Hatabu H, Togashi K, Kataoka ML, Mori T, Konishi J. Granulosa cell tumor of the ovary: MR findings. J omput ssist Tomogr 1997; 21: Troiano RN, Lazzarini KM, Scoutt LM, Lange R, Flynn SD, Mcarthy S. Fibroma and fibrothecoma of the ovary: MR imaging findings. Radiology 1997; 204: Rha SE, yun JY, Jung SE, et al. T and MR imaging features of adnexal torsion. RadioGraphics 2002; 22: Torricelli P, aruso Lombardi, oselli F, Rossi G. Sclerosing stromal tumor of the ovary: US, T and MRI findings. bdom Imaging 2002; 27: Joja I, Okuno K, Tsunoda M, et al. Sclerosing stromal tumor of the ovary: US, MR, and dynamic MR findings. J omput ssist Tomogr 2001; 25: Kim JY, Jung K, hung DS, Kim OD, Lee JH, Youn SK. Sclerosing stromal tumor of the ovary: MR-pathologic correlation in three cases. Korean J Radiol 2003; 4: Roth LM, nderson M, Govan D, Langley F, Gowing NF, Woodcock S. Sertoli-Leydig cell tumors: a clinicopathologic study of 34 cases. ancer 1981; 48: Wang PH, hao HT, Lee R, et al. Steroid cell tumors of the ovary: clinical, ultrasonic and MRI diagnosis a case report. Eur J Radiol 1998; 26: JR:185, July

Sclerosing Stromal Tumor of the Ovary: MR-Pathologic Correlation in Three Cases

Sclerosing Stromal Tumor of the Ovary: MR-Pathologic Correlation in Three Cases Jin Young Kim, MD 1 Kyung-Jae Jung, MD 1 Duck Soo Chung, MD 1 Ok Dong Kim, MD 1 Jin Hee Lee, MD 2 Sung Kook Youn, MD 3 Sclerosing