Sertoli-Leydig Cell Tumor of the Ovary in a Young Female: A Case Report and Literature Review

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1 Cse Report Sertoli-Leydig Cell Tumor of the Ovry in Young Femle: A Cse Report nd Literture Review Mdn M. Gupt, Nndini U. Bhri, Rthod Ketn Deprtment of Rdiodignosis nd Imging, Gurugovind Singh Hospitl, M. P. Shh Medicl College, Jmngr, Gujrt, Indi Correspondence: Dr. Mdn Mohn Gupt, Deprtment of Rdiodignosis nd Imging, Gurugovind Singh Hospitl, M.P. Shh Medicl College, Jmngr, Gujrt, Indi. E-mil: mdnmohngupt05@gmil.com ABSTRACT Sertoli-Leydig cell tumor (SLCT) of the ovry is n exceedingly unusul neoplsm tht elongs to group of sex-cordstroml tumors of ovry nd ccounts for <0.5% of ll primry ovrin neoplsms. We present cse of primry ovrin SLCT in 21-yer-old femle, who presented with 6-month history of pelvic pin, cne, hirsutism, nd oligomenorrhe with elevted testosterone nd dehydroepindrosterone levels. Ultrsound nd contrst computed tomogrphy reveled well-defined hyper vsculr heterogeneous solid lesion in left dnex. Mgnetic resonnce imging demonstrted ovrin origin of tumor, which turned out SLCT on histopthology. In this cse, we discuss multimodlity imging findings of SLCT nd its mngement spects with review of literture. Key words: Acne, hirsutism nd oligomenorrhe, primry ovrin neoplsms, Sertoli-Leydig cell tumor ملخص البحث : تعتبر خاليا سيرتولى اليدج السرطانية بالمبيض من األورام النادرة وتشكل اقل من 0.5% من كل سرطانات المبيض. يستعرض الباحثون حالة لمريضة في الواحد والعشرين من عمرها تشكو من الم في الحوض حب الشباب اضطراب ونقص في الدورة الشهرية ظهور الشعر في غير مواضعه مع ارتفاع هرمون التستيرون في الدم. بينت األشعة الصوتية والمقطعية وجود ورم بالقرب من المهبل كما وضح الفحص بالرنين المغنطيسي أن منشأ الورم هو المبيض وأظهر الفحص المجهري خاليا سيرتولي السرطانية بالمبيض. يناقش الباحثون الجوانب واألدبيات. INTRODUCTION Sertoli-Leydig cell tumor (SLCT) is rre ovrin tumor tht elongs to the group of sex-cord-stroml tumors. These constitute <0.5% of ovrin tumors. [1] Most tumors re unilterl, confined to the ovries, nd re seen during the second nd third decdes of life. These tumors re chrcterized y the presence of testiculr structures tht produce ndrogens. Hence, mny ptients hve symptoms of viriliztion depending on the quntity of ndrogen production. We present cse of SLCT with multimodlity imging findings nd its mngement issues. Access this rticle online Quick Response Code: Wesite: DOI: / X CASE REPORT A 21-yer-old nulliprous femle presented with complints of pelvic pin, cne, hirsutism, horseness of voice nd oligomenorrhe for the lst 6 months. No history of norexi, weight loss, incresed liido, or rest recession ws noted. Her medicl nd fmily history ws unremrkle. Vginl exmintion reveled clitoromegly nd firm nd moile solid mss in the left dnex. Lortory tests showed elevted serum testosterone 27.6 ng/ml (norml rnge: ng/ml) nd dehydroepindrosterone of 16.3 ng/ ml (norml rnge: ng/ml), nd norml CA- 125 of 22 U/mL (norml rnge <35 U/mL). All other lortory tests including complete lood count, renl, one, heptic nd cogultion profiles, lkline phosphtse, crcinoemryonic ntigen, luteinizing hormone, follicle stimulting hormone levels were within norml rnges. The ptient ws referred to imging workup to our deprtment. Ultrsound showed lrge wellcircumscried, well-mrginted heterogeneous mixed Sudi Journl of Medicine & Medicl Sciences Vol. 3 Issue 3 Septemer

2 Gupt, et l.: Sertoli-Leydig Cell Tumor of the Ovry solid-cystic lesion [Figure 1] in left dnex with internl vsculrity on color Doppler [Figure 1]. The right ovry nd the uterus were norml [Figure 1c]. Susequently, cross-sectionl imging ws performed; unenhnced computed tomogrphy (CT) demonstrted lrge well-circumscried solid lesion in left side of pelvic cvity [Figure 2]. On contrst-enhnced computed tomogrphy, the lesion showed heterogeneous enhncement more in peripherl prt (corresponds to solid prt of lesion on ultrsound) nd less enhncing centrl prt (corresponds to cystic prt of lesion on ultrsound). Left ovry is not distinctly visulized from the lesion. Blood supply to lesion ws coming from vsculr pedicle on its nterior spect [Figure 2]. The lesion cuses displcement of uterus inferiorly nd towrd right side. Right dnex ppered unremrkle [Figure 3]. No evidence of prortic lymphdenopthy ws noted [Figure 3]. Non-enhnced mgnetic resonnce imging (MRI) of the pelvis T2-weighted imges showed heterogeneous signl intensity lesion with few centrl cystic res in left dnex. The lesion cuses compression nd peripherl displcement of follicles suggestive of ovrin origin [Figure 4]. The lesion showed multiple signl voids. Figure 1: A 21-yer-old femle with left ovrin sertoli-leydig cell tumor, () Ultrsound shows heterogeneous mixed solid (thin rrow) nd cystic (lrge rrow) lesion in left dnex (white rrow), left ovry is not visulized seprtely. () On color Doppler ultrsound, the left dnexl lesion shows internl vsculrity (white rrow). (c) Ultrsound shows norml uterus (white rrow). c Figure 2: A 21-yer-old femle with left ovrin sertoli-leydig cell tumor, () Non enhnced computed tomogrphy domen-xil section shows lrge well-defined, well-circumscried solid soft tissue lesion in left side of pelvic cvity (white rrow). () On contrst-enhnced computed tomogrphy domen-xil section, the lesion shows heterogeneous enhncement in peripherl prt (corresponds to solid prt of lesion on ultrsound [smll rrow]) nd less enhncing centrl prt (corresponds to cystic prt of lesion on ultrsound [lrge rrow]). Blck rrow shows vsculr pedicle to the lesion on its nterior spect. Figure 3: A 21-yer-old femle with left ovrin sertoli-leydig cell tumor, On Contrst enhnced computed tomogrphy (CECT) domen-() xil section the lesion cuses displcement of uterus towrd right side (smll rrow). Right dnex pper unremrkle (lrge rrow). () Coronl section the lesion cuses displcement of uterus inferiorly nd towrd right side (lrge rrow). Right dnex pper unremrkle (smll rrow); however, right ovry is not distinctly visulized on CECT. No evidence of pr-ortic lymphdenopthy. Figure 4: A 21-yer-old femle with left ovrin sertoli-leydig cell tumor, nonenhnced mgnetic resonnce imging of pelvis-t2-weighted xil section shows lrge well-circumscried heterogeneous signl intensity (lck rrow) with few centrl cystic res (smll rrow) in left dnex cusing compression nd peripherl displcement of follicles (lrge rrow), suggest ovrin origin. 234 Sudi Journl of Medicine & Medicl Sciences Vol. 3 Issue 3 Septemer 2015

3 Gupt, et l.: Sertoli-Leydig Cell Tumor of the Ovry Uterus nd right ovry ppered unremrkle [Figure 5]. T1-weighted imge demonstrted isointense signl nd multiple signl voids [Figure 5]. On the sis of ove clinicl, iochemicl nd imging findings dignosis of ndrogenic neoplstic primry ovrin tumor ws mde. The ptient underwent for explortory lprotomy. Intropertively left ovry is replced y lrge cpsulted solid grey-white mss [Figure 6]. The right ovry nd uterus were found unremrkle. Left slpingo-oophorectomy ws performed. Gross exmintion of the pthologic specimen [Figure 6] showed n ovrin mss with smooth externl gryish surfce. A cut-section of the specimen [Figure 6c] reveled solid s well s cystic res filled with cler fluid. The histopthologicl exmintion showed tumor composed of poorly formed cords, nests, nd tuules of tumor cells. Tumor cells hd hyperchromtic nuclei, moderte mount of cytoplsm (Sertoli cells), nd occsionl mitosis [Figure 6d]. Interspersed etween these were nests of polygonl cells with round nuclei nd undnt grnulr cytoplsm (Leydig cells). Finl dignosis ws intermeditely differentited SLCT on histopthology. After her consent, one cycle for four cycles of chemotherpy with leomycin, etoposide, nd cispltin regimen were dministered every 3 weeks. The ptient tolerted the chemotherpy well, with no mjor complictions. During 10-month of follow-up, the ptient hs resumed her periods, with resolution of her viriliztion symptoms. There is lso no increse of her hirsutism. Repet testosterone levels on follow-up were within norml rnge. After 2 yers of opertion, t present the ptient leds symptom free life. DISCUSSION Sertoli-Leydig cell tumor is very rre ovrin tumor tht included in the group of sex-cord-stroml tumors nd they ccount for <0.5% of ll primry ovrin tumors. [1] It is usully seen during reproductive ge group (second nd third decdes of life). [1-5] Clinicl presenttion is relted to either hormonl production (mostly ndrogen nd rrely estrogen) [6] or presence of mssoccupying lesion. [1-5] Androgen-excess mnifesttions include virilism, hirsutism, nd horseness of voice, clitoromegly, oligomenorrhe, nd menorrhe. [7,8] Imging studies cn e utilized in the dignosis of ovrin SLCTs. Sonogrphy remins the est imging modlity of preference for initil ssessment of dnexl msses, due to its high sensitivity, suitility, nd cost-effectiveness. [7,9] In generl, SLCTs exhiit solid sonogrphic ppernce [7,9] nd mostly unilterl tumors. [1-3,5] Mixed (solid nd cystic) components re most commonly encountered in roughly 60% of ll ovrin SLCTs. [1] Color Doppler sonogrphy offers further ctegoriztion nd evlution of neoplstic msses. Moderte-to-rich ovrin vsculr msses with low-resistnce indices highly suggest mlignnt rther thn enign lesions. Other imging modlities such s Figure 5: A 21-yer-old femle with left ovrin sertoli-leydig cell tumor, nonenhnced mgnetic resonnce imging of pelvis-() T2- weighted xil section, the left ovrin lesion shows multiple signl voids (lrge rrow) nd cuses displcement of uterus towrd right side (curved rrow). Uterus nd right ovry ppers unremrkle (smll rrow). () T1-weighted xil section, the left ovrin lesion isointense signl intensity (smll rrow) nd multiple signl voids (lrge rrow). c Figure 6: () Intropertive highly vsculr ovrin lesion (smll rrow) nd fllopin tue (lrge rrow) nd uterus (lck rrow). () Gross specimen shows well-encpsulted gryish solid mss. (c) Cut-section of specimen shows tn-yellow nodulr ppernce (white rrow). (d) Microscopiclly, Photomicrogrph showing hyperchromtic nuclei, moderte cytoplsm (Sertoli cells [lck rrow]) nd nests of polygonl cells with round nuclei nd undnt grnulr cytoplsm (Leydig cells with heterologous elements showing mucinous epithelium of the gstrointestinl type [white rrow]) (H nd E 40). d Sudi Journl of Medicine & Medicl Sciences Vol. 3 Issue 3 Septemer

4 Gupt, et l.: Sertoli-Leydig Cell Tumor of the Ovry CT, MRI, nd positron imging tomogrphy scns cn e used for etter chrcteriztion of ovrin SLCTs, detection of extr ovrin disese/metstsis. SLCTs pper hyper vsculr mixed solid-cystic lesion within dnex on CT or MRI. Cross-sectionl imging cn e used for stging of tumor preopertively. Mcroscopiclly, [10] SLCTs re well-encpsulted, solid, firm, nd yellow-gry mss. Cut-section surfce exhiits vrying degrees of gresy/fleshy consistency, strw-colored fluid, necrosis, hemorrhge, nd cystic spces seprted y firous septe. Microscopiclly, [10] SLCTs re mde up of uncontrolled prolifertion of differentition of tuules lined y Sertoli cells nd intervening nests of Leydig cells. Leydig cells re typiclly found in clusters in interstitil strom nd exhiit polygonl cells with well-defined mrgins, centric nuclei, prominent nucleoli, nd eosinophilic cytoplsm. Sertoli cells typiclly form tuulr structures lined y single or multiple lyers of cuoidl-columnr cells with well-ounded mrgins, ovl drk nuclei, inconspicuous nucleoli, nd eosinophilic or vcuolted cytoplsm. Pthologiclly, SLCT re divided into four sutypes: Well-(11%), intermeditely-(54%), nd poorly differentited (13%) nd contined heterologous elements (22%). Heterologous elements re vrious, such s crcinoid, mesenchyml, nd mucinous epithelil tissues with the commonest eing gstrointestinl-types. [10] Retiform pttern presents in 15% of SLCT, nd is found only in intermeditely nd poorly differentited SLCT, nd often occurs in younger women. [10] SLCT contins Sertoli cells, Leydig cells, gondl stroml cells nd heterologous cells in tumors with heterologous elements. Leydig cells sctter mong numerous Sertoli cells, which my rrnge into open or closed tuules. The most importnt prognostic fctors in these tumors re their stge nd degree of differentition. In review of 207 cses y Young nd Scully in 1985, [10] ll well-differentited tumors were enign, wheres 11% of tumors with intermedite differentition, 59% of tumors with poor differentition, nd 19% of those with heterologous elements were mlignnt. In nother study of 64 ptients who hd intermedite or poorly differentited SLCT, survivl rte of 92% ws noted t oth 5 nd 10 yers. [11] Most of these tumors re unilterl nd dignosed in Stge I, so conservtive surgery in young ptient is n pproprite tretment. There hve lso een cse reports of successful lproscopic mngement of these tumors. [11] Adjuvnt chemotherpy is considered for ptients who hve poor prognostic fctors. The mlignncy rte in tumors with heterologous elements is 15-20%. Adjuvnt chemotherpy in Stge I is given to those ptients who hve poorly differentited SLCT or SLCT with heterologous elements or metsttic tumor of ny histologic type. [12] Surgicl resection represents the minsty of mngement of ovrin SLCTs. [4] Therefore, fertilityspring surgery (unilterl slpingo-oophorectomy) cn e considered in ll ptients with well-differentited ovrin SLCTs. In generl, postopertive chemotherpy is considered for ptients with poor prognostic fctors such s dvnced disese stging, moderte-topoor tumor grding, high mitotic profile, existence of heterologous elements, nd tumor rupture. [1-3] Prognosis of ovrin SLCTs is significntly correlted with degree of tumor differentition (grding) nd tumor extent (stging). [1] Long-term follow-up is highly dvised in ll ptients. CONCLUSION Sertoli-Leydig cell tumor is rre ovrin sex-cord tumor of the ovry with good prognosis postopertively. Hence, imging findings my help in erly detection nd preopertive differentition nd stging of SLCT in young femle. Its mngement depends on degree of differentition nd stging of tumor, which mostly depend on histopthology. REFERENCES 1. Young RH, Scully RE. Ovrin Sertoli-Leydig cell tumors. A clinicopthologicl nlysis of 207 cses. Am J Surg Pthol 1985;9: Zloudek C, Norris HJ. Sertoli-Leydig tumors of the ovry. A clinicopthologic study of 64 intermedite nd poorly differentited neoplsms. Am J Surg Pthol 1984;8: Roth LM, Anderson MC, Govn AD, Lngley FA, Gowing NF, Woodcock AS. Sertoli-Leydig cell tumors: A clinicopthologic study of 34 cses. Cncer 1981;48: Weng CS, Chen MY, Wng TY, Tsi HW, Hung YC, Yu KJ, et l. Sertoli-Leydig cell tumors of the ovry: A Tiwnese Gynecologic Oncology Group study. Tiwn J Ostet Gynecol 2013;52: Young RH, Scully RE. Sex cord-stroml, steroid cell, nother ovrin tumors. In: Kurmn RJ, editor. Blustein s Pthology of Femle Genitl Trct. 5 th ed. New York, NY, USA: Springer; p Sudi Journl of Medicine & Medicl Sciences Vol. 3 Issue 3 Septemer 2015

5 Gupt, et l.: Sertoli-Leydig Cell Tumor of the Ovry 6. Znotti KM. The clinicl mnifesttions nd dignosis of Sertoli-Leydig cell tumors of the ovry. CME J Gynecol Oncol 2002;7: Osorn RH, Ynnone ME. Plsm ndrogens in the norml nd ndrogenic femle: A review. Ostet Gynecol Surv 1971;26: Prunty FT. Hirsutism, virilism nd pprent virilism nd their gondl reltionship. II. J Endocrinol 1967;38: de Oliveir Frnzin CM, Krft ML, Fundes D, Zeferino LC, Alvreng M, Mrussi EF. Detection of ovrin Sertoli-Leydig cell tumors exclusively y color Doppler sonogrphy. J Ultrsound Med 2006;25: Nourini M, Felix JC, Dueu L. Histogenesis nd histopthologicl chrcteristics of Sertoli-Leydig cell tumors. CME J Gynecol Oncol 2002;7: Kriplni A, Agrwl N, Roy KK, Mnchnd R, Singh MK. Lproscopic mngement of Sertoli-Leydig cell tumors of the ovry. A report of two cses. J Reprod Med 2001;46: Sood AK, Gershenson DM. Mngement of erly-stge ovrin cncer. In: Bristow RE, Krln BY, editors. Surgery for Ovrin Cncer Principles nd Prctice. London, UK: Tylor nd Frncis; p How to cite this rticle: Gupt MM, Bhri NU, Ketn R. Sertolileydig cell tumor of the ovry in young femle: A cse report nd literture review. Sudi J Med Med Sci 2015;3: Source of Support: Nil, Conflict of Interest: None declred. Sudi Journl of Medicine & Medicl Sciences Vol. 3 Issue 3 Septemer