Abstract
Sex cord-stromal tumors of the ovary (SCST) are uncommon ovarian tumors arising from sex cord and/or stromal cells of the ovaries. They may be nonfunctional and asymptomatic or functional presenting with hyperestrogenic, hyperandrogenic or cushingoid symptoms. They present in a wide age group of women, mostly in early stages and follow a nonaggressive clinical course after surgical resection. They differ from more prevalent epithelial ovarian tumors which tend to present in older women in advanced stages with poor prognosis. Some of SCSTs are associated with clinical syndromes. We will review imaging features on ultrasound, computed tomography and magnetic resonance imaging, epidemiology and clinical presentations of these tumors.
Similar content being viewed by others
References
Haroon S, Zia A, Idrees R, et al. (2013) Clinicopathological spectrum of ovarian sex cord-stromal tumors; 20 years’ retrospective study in a developing country. Journal of ovarian research 6(1):87. https://doi.org/10.1186/1757-2215-6-87
Quirk JT, Natarajan N (2005) Ovarian cancer incidence in the United States, 1992-1999. Gynecologic oncology 97(2):519–523. https://doi.org/10.1016/j.ygyno.2005.02.007
Kurman RJ, Carcangiu ML, Herrington CS, Young RH (2014) Classification of tumours of the ovary. In: WHO classification of tumours. vol 6, 4th edn. IARC, Lyon, pp 44–56
Pratt J (2004) Pathology of the ovary, 1st edn. Philadelphia: Sounders
Levin G, Zigron R, Haj-Yahya R, Matan LS, Rottenstreich A (2018) Granulosa cell tumor of ovary: A systematic review of recent evidence. European journal of obstetrics, gynecology, and reproductive biology 225:57–61. https://doi.org/10.1016/j.ejogrb.2018.04.002
Torre LA, Trabert B, DeSantis CE, Miller KD, Samimi G, Runowicz CD, Gaudet MM, Jemal A, Siegel RL (2018) Ovarian cancer statistics, 2018. CA: a cancer journal for clinicians 68 (4):284-296. https://doi.org/10.3322/caac.21456
Seagle BL, Ann P, Butler S, Shahabi S (2017) Ovarian granulosa cell tumor: A National Cancer Database study. Gynecologic oncology 146(2):285–291. https://doi.org/10.1016/j.ygyno.2017.05.020
Horta M, Cunha TM (2015) Sex cord-stromal tumors of the ovary: a comprehensive review and update for radiologists. Diagnostic and interventional radiology (Ankara, Turkey) 21(4):277–286. https://doi.org/10.5152/dir.2015.34414
Fuller PJ, Leung D, Chu S (2017) Genetics and genomics of ovarian sex cord-stromal tumors. Clinical genetics 91(2):285–291. https://doi.org/10.1111/cge.12917
Jung SE, Rha SE, Lee JM, et al. (2005) CT and MRI findings of sex cord-stromal tumor of the ovary. AJR American journal of roentgenology 185(1):207–215. https://doi.org/10.2214/ajr.185.1.01850207
Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B (1998) Sex cord-stromal and steroid cell tumors of the ovary. Radiographics: a review publication of the Radiological Society of North America, Inc 18 (6):1523-1546. https://doi.org/10.1148/radiographics.18.6.9821198
Clement PB, Young RH, Scully RE (1991) Clinical syndromes associated with tumors of the female genital tract. Seminars in diagnostic pathology 8(4):204–233
Ko SF, Wan YL, Ng SH, et al. (1999) Adult ovarian granulosa cell tumors: spectrum of sonographic and CT findings with pathologic correlation. AJR American journal of roentgenology 172(5):1227–1233. https://doi.org/10.2214/ajr.172.5.10227493
Lauszus FF, Petersen AC, Greisen J, Jakobsen A (2001) Granulosa cell tumor of the ovary: a population-based study of 37 women with stage I disease. Gynecologic oncology 81(3):456–460. https://doi.org/10.1006/gyno.2001.6183
Van Holsbeke C, Domali E, Holland TK, et al. (2008) Imaging of gynecological disease (3): clinical and ultrasound characteristics of granulosa cell tumors of the ovary. Ultrasound Obstet Gynecol 31(4):450–456. https://doi.org/10.1002/uog.5279
Zhang H, Zhang H, Gu S, et al. (2018) MR findings of primary ovarian granulosa cell tumor with focus on the differentiation with other ovarian sex cord-stromal tumors. J Ovarian Res 11(1):46. https://doi.org/10.1186/s13048-018-0416-x
Tanaka YO, Tsunoda H, Kitagawa Y, Ueno T, Yoshikawa H, Saida Y (2004) Functioning ovarian tumors: direct and indirect findings at MR imaging. Radiographics: a review publication of the Radiological Society of North America, Inc 24 Suppl 1:S147-166. https://doi.org/10.1148/rg.24si045501
Kim SH, Kim SH (2002) Granulosa cell tumor of the ovary: common findings and unusual appearances on CT and MR. Journal of computer assisted tomography 26(5):756–761. https://doi.org/10.1097/00004728-200209000-00016
Minutoli F, Blandino A, Gaeta M, Lentini M, Pandolfo I (2001) Twisted ovarian fibroma with high signal intensity on T1-weighted MR image: a new sign of torsion of ovarian tumors? European radiology 11(7):1151–1154. https://doi.org/10.1007/s003300000723
Chung BM, Park SB, Lee JB, et al. (2015) Magnetic resonance imaging features of ovarian fibroma, fibrothecoma, and thecoma. Abdominal imaging 40(5):1263–1272. https://doi.org/10.1007/s00261-014-0257-z
Yazdani S, Alijanpoor A, Sharbatdaran M, et al. (2014) Meigs’ syndrome with elevated serum CA125 in a case of ovarian fibroma/thecoma. Caspian journal of internal medicine 5(1):43–45
Riker D, Goba D (2013) Ovarian mass, pleural effusion, and ascites: revisiting Meigs syndrome. Journal of bronchology & interventional pulmonology 20(1):48–51. https://doi.org/10.1097/LBR.0b013e31827ccb35
Scalia AC, Farulla A, Fiocchi F, Alboni C, Torricelli P (2018) Imaging features of uterine and ovarian fibromatosis in Nevoid Basal Cell Carcinoma Syndrome. Journal of radiology case reports 12(9):21–30. https://doi.org/10.3941/jrcr.v12i9.3390
Zhao SH, Li HM, Qiang JW, Wang DB, Fan H (2018) The value of MRI for differentiating benign from malignant sex cord-stromal tumors of the ovary: emphasis on diffusion-weighted MR imaging. Journal of ovarian research 11(1):73. https://doi.org/10.1186/s13048-018-0444-6
Rha SE, Byun JY, Jung SE, Jung JI, Choi BG, Kim BS, Kim H, Lee JM (2002) CT and MR imaging features of adnexal torsion. Radiographics: a review publication of the Radiological Society of North America, Inc 22 (2):283-294. https://doi.org/10.1148/radiographics.22.2.g02mr02283
Oh SN, Rha SE, Byun JY, et al. (2008) MRI features of ovarian fibromas: emphasis on their relationship to the ovary. Clinical radiology 63(5):529–535. https://doi.org/10.1016/j.crad.2007.10.006
Zong L, Lin M, Fan X (2014) Mitotically active cellular fibroma of ovary should be differentiated from fibrosarcoma: a case report and review of literature. International journal of clinical and experimental pathology 7(11):7578–7582
Huang L, Liao LM, Wang HY, Zheng M (2010) Clinicopathologic characteristics and prognostic factors of ovarian fibrosarcoma: the results of a multi-center retrospective study. BMC cancer 10:585. https://doi.org/10.1186/1471-2407-10-585
Siekierska-Hellmann M, Sworczak K, Babińska A, Wojtylak S (2006) Ovarian thecoma with androgenic manifestations in a postmenopausal woman. Gynecological endocrinology: the official journal of the International Society of Gynecological Endocrinology 22(7):405–408. https://doi.org/10.1080/09513590600842539
Roth LM, Czernobilsky B (2011) Perspectives on pure ovarian stromal neoplasms and tumor-like proliferations of the ovarian stroma. The American journal of surgical pathology 35(3):15–33. https://doi.org/10.1097/PAS.0b013e31820acb89
Zhang H, Zhang GF, Wang TP, Zhang H (2013) Value of 3.0 T diffusion-weighted imaging in discriminating thecoma and fibrothecoma from other adnexal solid masses. Journal of ovarian research 6 (1):58. https://doi.org/10.1186/1757-2215-6-58
Li Z, Hu Q, Luo Z, et al. (2020) Analysis of magnetic resonance imaging features of ovarian thecoma. Medicine 99(21):e20358. https://doi.org/10.1097/md.0000000000020358
Bono Y, Mizumoto Y, Nakamura M, et al. (2017) FDG-PET-positive ovarian thecoma with GLUT5 expression: Five cases. The journal of obstetrics and gynaecology research 43(3):599–603. https://doi.org/10.1111/jog.13243
Spiegel GW, Swiger FK (1996) Luteinized thecoma with sclerosing peritonitis presenting as an acute abdomen. Gynecologic oncology 61(2):275–281. https://doi.org/10.1006/gyno.1996.0139
Staats PN, McCluggage WG, Clement PB, Young RH (2008) Luteinized thecomas (thecomatosis) of the type typically associated with sclerosing peritonitis: a clinical, histopathologic, and immunohistochemical analysis of 27 cases. The American journal of surgical pathology 32(9):1273–1290. https://doi.org/10.1097/PAS.0b013e3181666a5f
Bahar B, Hu Z, Szpaderska A, et al. (2014) Fatal case of luteinized thecoma with sclerosing peritonitis in a 40-year-old woman. International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists 33(1):30–34. https://doi.org/10.1097/PGP.0b013e31827d1a65
Levavi H, Sabah G, Heifetz M, Feinmesser M (2009) Sclerosing peritonitis associated with bilateral luteinized thecoma, linked to anticonvulsant therapy. European journal of gynaecological oncology 30(6):695–700
Duman E, Aslan A, Gunduz N, Inan I (2018) Sclerosing encapsulated peritonitis: typical imaging findings for easy diagnosis. Annals of Saudi medicine 38(3):230–232. https://doi.org/10.5144/0256-4947.2018.230
Zung A, Shoham Z, Open M, et al. (1998) Sertoli cell tumor causing precocious puberty in a girl with Peutz-Jeghers syndrome. Gynecologic oncology 70(3):421–424. https://doi.org/10.1006/gyno.1998.5063
Oliva E, Alvarez T, Young RH (2005) Sertoli cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 54 cases. The American journal of surgical pathology 29(2):143–156. https://doi.org/10.1097/01.pas.0000149692.21205.9c
Tavassoli FA, Norris HJ (1980) Sertoli tumors of the ovary. A clinicopathologic study of 28 cases with ultrastructural observations. Cancer 46 (10):2281-2297. https://doi.org/10.1002/1097-0142(19801115)46:10%3c2281::aid-cncr2820461028%3e3.0.co;2-4
Young RH, Welch WR, Dickersin GR, Scully RE (1982) Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer 50(7):1384–1402. 10.1002/1097-0142(19821001)50:7<1384::aid-cncr2820500726>3.0.co;2-5
Moon WK, Kim SH, Kim WS, et al. (1995) Case report: ovarian sex cord tumour with annular tubules: imaging findings. Clinical radiology 50(8):581–582. https://doi.org/10.1016/s0009-9260(05)83200-3
Swanger RS, Brudnicki A (2007) Ultrasound of ovarian sex-cord tumor with annular tubules. Pediatric radiology 37(12):1270–1271. https://doi.org/10.1007/s00247-007-0600-4
Grechi G, Clemente N, Tozzi A, Ciavattini A (2015) Laparoscopic Treatment of Sclerosing Stromal Tumor of the Ovary in a Woman With Gorlin-Goltz Syndrome: A Case Report and Review of the Literature. Journal of minimally invasive gynecology 22(5):892–895. https://doi.org/10.1016/j.jmig.2015.03.002
Chang YW, Hong SS, Jeen YM, Kim MK, Suh ES (2009) Bilateral sclerosing stromal tumor of the ovary in a premenarchal girl. Pediatric radiology 39(7):731–734. https://doi.org/10.1007/s00247-009-1190-0
Ismail SM, Walker SM (1990) Bilateral virilizing sclerosing stromal tumours of the ovary in a pregnant woman with Gorlin’s syndrome: implications for pathogenesis of ovarian stromal neoplasms. Histopathology 17(2):159–163. https://doi.org/10.1111/j.1365-2559.1990.tb00688.x
Lee MS, Cho HC, Lee YH, Hong SR (2001) Ovarian sclerosing stromal tumors: gray scale and color Doppler sonographic findings. Journal of ultrasound in medicine: official journal of the American Institute of Ultrasound in Medicine 20(4):413–417. https://doi.org/10.7863/jum.2001.20.4.413
Torricelli P, Caruso Lombardi A, Boselli F, Rossi G (2002) Sclerosing stromal tumor of the ovary: US, CT, and MRI findings. Abdominal imaging 27(5):588–591. https://doi.org/10.1007/s00261-001-0096-6
Vang R, Bagué S, Tavassoli FA, Prat J (2004) Signet-ring stromal tumor of the ovary: clinicopathologic analysis and comparison with Krukenberg tumor. International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists 23(1):45–51. https://doi.org/10.1097/01.pgp.0000101081.35393.5f
Forde GK, Harrison C, Doss BJ, Forde AE, Carlson JW (2010) Bilateral and multinodular signet-ring stromal tumor of the ovary. Obstetrics and gynecology 116(Suppl 2):556–558. https://doi.org/10.1097/AOG.0b013e3181e9b410
Chen PH, Hui P, Buza N (2020) Bilateral Signet-ring Stromal Tumor of the Ovary: A Case Report With Next-generation Sequencing Analysis and FOXL2 Mutation Testing. International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists 39(2):193–198. https://doi.org/10.1097/pgp.0000000000000579
Liu J, Hou Y, Bao L, et al. (2019) Ovarian microcystic stromal tumors: clinical, radiological, and pathological studies of two cases. International journal of clinical and experimental pathology 12(6):2241–2248
Jeong D, Hakam A, Abuel-Haija M, Chon HS (2018) Ovarian microcystic stromal tumor: Radiologic-pathologic correlation. Gynecologic oncology reports 25:11–14. https://doi.org/10.1016/j.gore.2018.05.004
Hayes MC, Scully RE (1987) Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases. The American journal of surgical pathology 11 (11):835-845. https://doi.org/10.1097/00000478-198711000-00002
Donovan JT, Otis CN, Powell JL, Cathcart HK (1993) Cushing’s syndrome secondary to malignant lipoid cell tumor of the ovary. Gynecologic oncology 50(2):249–253. https://doi.org/10.1006/gyno.1993.1202
Oz M, Ozgü E, Türker M, Erkaya S, Güngör T (2014) Steroid cell tumor of the ovary in a pregnant woman whose androgenic symptoms were masked by pregnancy. Archives of gynecology and obstetrics 290(1):131–134. https://doi.org/10.1007/s00404-014-3165-0
Saida T, Tanaka YO, Minami M (2007) Steroid cell tumor of the ovary, not otherwise specified: CT and MR findings. AJR American journal of roentgenology 188(4):393–394. https://doi.org/10.2214/ajr.06.0867
Morani AC, Mubarak AI, Bhosale HR, et al. (2019) Steroid Cell Ovarian Tumor in a Case of von Hippel-Lindau Disease: Demonstrating Lipid Content of the Mass with MR Imaging. Magnetic resonance in medical sciences: MRMS: an official journal of Japan Society of Magnetic Resonance in Medicine 18(4):251–252. https://doi.org/10.2463/mrms.ci.2018-0104
Sakamoto K, Fujimitsu R, Ida M, et al. (2009) MR diagnosis of steroid cell tumor of the ovary: value of chemical shift imaging. Magnetic resonance in medical sciences: MRMS: an official journal of Japan Society of Magnetic Resonance in Medicine 8(4):193–195. https://doi.org/10.2463/mrms.8.193
Chen VW, Ruiz B, Killeen JL, et al. (2003) Pathology and classification of ovarian tumors. Cancer 97(10 Suppl):2631–2642. https://doi.org/10.1002/cncr.11345
Okamura K, Yoshizako T, Yoshida R, et al. (2020) Diagnosis of a small Leydig cell tumor by dynamic contrast-enhanced and diffusion-weighted magnetic resonance imaging. Radiol Case Rep 15(7):875–878. https://doi.org/10.1016/j.radcr.2020.04.019
Cai S-Q, Zhao S-H, Qiang J-W, et al. (2013) Ovarian Sertoli-Leydig cell tumors: MRI findings and pathological correlation. Journal of ovarian research 6(1):73. https://doi.org/10.1186/1757-2215-6-73
Bhat RA, Lim YK, Chia YN, Yam KL (2013) Sertoli-Leydig cell tumor of the ovary: analysis of a single institution database. The journal of obstetrics and gynaecology research 39(1):305–310. https://doi.org/10.1111/j.1447-0756.2012.01928.x
Funding
This review article is not supported by any grant funding and authors do not have any financial disclosures.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Human and animal rights
This article does not contain any studies with human participants performed by any of the authors.
Informed consent
For this review article, formal consent was not required.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
CME activity This article has been selected as the CME activity for the current month. Please visit https://ce.mayo.edu/node/103132 and follow the instructions to complete this CME activity.
Rights and permissions
About this article
Cite this article
Javadi, S., Ganeshan, D.M., Jensen, C.T. et al. Comprehensive review of imaging features of sex cord-stromal tumors of the ovary. Abdom Radiol 46, 1519–1529 (2021). https://doi.org/10.1007/s00261-021-02998-w
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00261-021-02998-w