Testicular sex cord stromal tumors
- Mary-Ellen Taplin, MD
Mary-Ellen Taplin, MD
- Professor of Medicine
- Harvard Medical School
Sex cord stromal tumors (SCSTs) comprise approximately 5 percent of all testicular tumors, while the remainder are of germ cell origin . SCSTs, which arise from the supporting tissues of the testis, include Leydig, Sertoli, and granulosa cell tumors, as well as malignant mesothelioma of the tunica vaginalis, adenocarcinoma of the rete testis, and paratesticular rhabdomyosarcoma [2-5]. In 2017, the World Health Organization (WHO) published a review and update of the classification of testicular non-germ cell tumors .
The clinical presentation and management of SCSTs are discussed here. The pathology of SCSTs and an overview of testicular germ cell tumors are presented separately. (See "Anatomy and pathology of testicular tumors", section on 'Sex cord-stromal tumors' and "Overview of the treatment of testicular germ cell tumors".)
The most common clinical presentation of testicular SCSTs is with a mass, which often causes swelling and/or discomfort. Tumors may also be discovered on routine physical examination or during evaluation for endocrine symptoms due to sex hormone production [2,7]. In general, there is a wide age range for SCSTs, with ages in two contemporary series ranging between 14 and 87 years [8,9].
There are no known risk factors for SCSTs. Unlike germ cell tumors, there has been no definitive association with cryptorchidism, although such an association cannot be excluded [10,11].
SCSTs do not typically produce markers such as lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and human chorionic gonadotropin (hCG), which are more commonly elevated in testicular germ cell tumors.
Subscribers log in hereLiterature review current through: Sep 2017. | This topic last updated: Sep 27, 2017.References
- Dilworth JP, Farrow GM, Oesterling JE. Non-germ cell tumors of testis. Urology 1991; 37:399.
- Conkey DS, Howard GC, Grigor KM, et al. Testicular sex cord-stromal tumours: the Edinburgh experience 1988-2002, and a review of the literature. Clin Oncol (R Coll Radiol) 2005; 17:322.
- Murthy V, Fisher C, Horwich A. Rare tumors of the testis and paratesticular tissues. In: Textbook of Uncommon Cancer, 3rd, Raghavan D, Brecher M, Johnson D, et al (Eds), Wiley, 2006. p.66.
- Schure PJ, van Dalen KC, Ruitenberg HM, van Dalen T. Mesothelioma of the tunica vaginalis testis: a rare malignancy mimicking more common inguino-scrotal masses. J Surg Oncol 2006; 94:162.
- Plas E, Riedl CR, Pflüger H. Malignant mesothelioma of the tunica vaginalis testis: review of the literature and assessment of prognostic parameters. Cancer 1998; 83:2437.
- Idrees MT, Ulbright TM, Oliva E, et al. The World Health Organization 2016 classification of testicular non-germ cell tumours: a review and update from the International Society of Urological Pathology Testis Consultation Panel. Histopathology 2017; 70:513.
- Anderson MS, Brogi E, Biller BM. Occult Leydig cell tumor in a patient with gynecomastia. Endocr Pract 2001; 7:267.
- Cornejo KM, Young RH. Adult granulosa cell tumors of the testis: a report of 32 cases. Am J Surg Pathol 2014; 38:1242.
- Kao CS, Kum JB, Idrees MT, Ulbright TM. Sclerosing Sertoli cell tumor of the testis: a clinicopathologic study of 20 cases. Am J Surg Pathol 2014; 38:510.
- Al-Agha OM, Axiotis CA. An in-depth look at Leydig cell tumor of the testis. Arch Pathol Lab Med 2007; 131:311.
- Kim I, Young RH, Scully RE. Leydig cell tumors of the testis. A clinicopathological analysis of 40 cases and review of the literature. Am J Surg Pathol 1985; 9:177.
- Banerji JS, Odem-Davis K, Wolff EM, et al. Patterns of Care and Survival Outcomes for Malignant Sex Cord Stromal Testicular Cancer: Results from the National Cancer Data Base. J Urol 2016; 196:1117.
- Gabrilove JL, Nicolis GL, Mitty HA, Sohval AR. Feminizing interstitial cell tumor of the testis: personal observations and a review of the literature. Cancer 1975; 35:1184.
- Ulbright TM, Amin MB, Young RH. Ulbright TM, Amin MB, Young RH. In: Atlas of Tumor Pathology, 3rd, Rosai J, Sobin LH (Eds), Armed Forces Institute of Pathology, Washington, DC 1999.
- Proppe KH, Scully RE. Large-cell calcifying Sertoli cell tumor of the testis. Am J Clin Pathol 1980; 74:607.
- Washecka R, Dresner MI, Honda SA. Testicular tumors in Carney's complex. J Urol 2002; 167:1299.
- Ditonno P, Lucarelli G, Battaglia M, et al. Testicular granulosa cell tumor of adult type: a new case and a review of the literature. Urol Oncol 2007; 25:322.
- Al-Bozom IA, El-Faqih SR, Hassan SH, et al. Granulosa cell tumor of the adult type: a case report and review of the literature of a very rare testicular tumor. Arch Pathol Lab Med 2000; 124:1525.
- Arzola J, Hutton RL, Baughman SM, Mora RV. Adult-type testicular granulosa cell tumor: case report and review of the literature. Urology 2006; 68:1121.e13.
- Garrett JE, Cartwright PC, Snow BW, Coffin CM. Cystic testicular lesions in the pediatric population. J Urol 2000; 163:928.
- Gourlay WA, Johnson HW, Pantzar JT, et al. Gonadal tumors in disorders of sexual differentiation. Urology 1994; 43:537.
- Nicolai N, Necchi A, Raggi D, et al. Clinical outcome in testicular sex cord stromal tumors: testis sparing vs. radical orchiectomy and management of advanced disease. Urology 2015; 85:402.
- Featherstone JM, Fernando HS, Theaker JM, et al. Sex cord stromal testicular tumors: a clinical series--uniformly stage I disease. J Urol 2009; 181:2090.
- Silberstein JL, Bazzi WM, Vertosick E, et al. Clinical outcomes of local and metastatic testicular sex cord-stromal tumors. J Urol 2014; 192:415.
- Rove KO, Maroni PD, Cost CR, et al. Pathologic Risk Factors for Metastatic Disease in Postpubertal Patients With Clinical Stage I Testicular Stromal Tumors. Urology 2016; 97:138.
- Mosharafa AA, Foster RS, Bihrle R, et al. Does retroperitoneal lymph node dissection have a curative role for patients with sex cord-stromal testicular tumors? Cancer 2003; 98:753.
- Farkas LM, Székely JG, Pusztai C, Baki M. High frequency of metastatic Leydig cell testicular tumours. Oncology 2000; 59:118.
- Azer PC, Braunstein GD. Malignant Leydig cell tumor: objective tumor response to o,p'-DDD. Cancer 1981; 47:1251.
- van der Hem KG, Boven E, van Hennik MB, Pinedo HM. Malignant Leydig cell tumor of the testis in complete remission on o,p'-dichlorodiphenyl-dichloroethane. J Urol 1992; 148:1256.
- Grem JL, Robins HI, Wilson KS, et al. Metastatic Leydig cell tumor of the testis. Report of three cases and review of the literature. Cancer 1986; 58:2116.
- Hansen PV, Trykker H, Andersen J, Helkjaer PE. Germ cell function and hormonal status in patients with testicular cancer. Cancer 1989; 64:956.