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Vol 20, No 5 (2024)
Review paper
Published online: 2024-01-16

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Sex cord-stromal tumors of the testis—a rare group of benign and malignant gonadal neoplasms. Literature review

Krzysztof Kowalik1, Katarzyna Hetman2, Krystian Kasperowicz3, Andrzej Modrzejewski1
DOI: 10.5603/ocp.97184
Oncol Clin Pract 2024;20(5):343-350.

Abstract

This article discusses a less common group of testicular tumors, including sex cord-stromal tumors and gonadal stromal tumors. Among the sex cord-stromal tumors, we can distinguish androblastoma (Leydig cell tumor and Sertoli cell tumor), fibroma-thecoma group tumors, stromal tumors, and sex cord tumors. Based on a literature review, we present the epidemiology, diagnosis, and treatment of these testicular tumors. In our opinion, due to the rarity of this group of tumors and limited data in the EAU guidelines, this topic deserves attention, which is why we chose to explore it in our study.

Keywords: testicular tumorsex cord-stromal tumorsleydig testicular tumorsertoli tetsicular tumordiagnosistreatmentorchidectomytestis sparing surgery

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References

  1. Baird DC, Meyers GJ, Hu JS. Testicular Cancer: Diagnosis and Treatment. Am Fam Physician. 2018; 97(4): 261–268.
    PubMed
  2. EAU Guidelines 2023: Testicular cancer. https://uroweb.org/guidelines/testicular-cancer/chapter/epidemiology-aetiology-amp-pathology.
  3. Moch H, Humphrey PA, Ulbright TM, et al. WHO Classification of Tumors of the Urinary System and Male Genital. World Health Organization, Geneve 2016.
  4. World Health Organization (WHO) classification of testicular tumors. https://www.uptodate.com/contents/image?imageKey=ONC%2F81626.
  5. Berney DM, Cree I, Rao V, et al. An introduction to the WHO 5th edition 2022 classification of testicular tumours. Histopathology. 2022; 81(4): 459–466.
    CrossRefPubMed
  6. Kapoor M, Leslie SW. Sex Cord Stromal Testicular Tumor [Updated 2023 May 30]. In: StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL) 2023.
  7. Al-Agha OM, Axiotis CA. An in-depth look at Leydig cell tumor of the testis. Arch Pathol Lab Med. 2007; 131(2): 311–317.
    CrossRefPubMed
  8. 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(3): 177–192.
    CrossRefPubMed
  9. McGlynn KA, Trabert B. Adolescent and adult risk factors for testicular cancer. Nat Rev Urol. 2012; 9(6): 339–349.
    CrossRefPubMed
  10. Maizlin ZV, Belenky A, Kunichezky M, et al. Leydig cell tumors of the testis: gray scale and color Doppler sonographic appearance. J Ultrasound Med. 2004; 23(7): 959–964.
    CrossRefPubMed
  11. Kong Q, Yu Y, Tian T, et al. Leydig cell tumor of a testis with azoospermia: A case report and literature review. Medicine (Baltimore). 2020; 99(36): e22085.
    CrossRefPubMed
  12. Agarwal PK, Palmer JS. Testicular and paratesticular neoplasms in prepubertal males. J Urol. 2006; 176(3): 875–881.
    CrossRefPubMed
  13. Del Real OJ, Calvo de la Barra CI, Jiménez JA, et al. Predicting malignancy in small testicular lesions. Cent European J Urol. 2022; 75(1): 47–51.
    CrossRefPubMed
  14. Giannarini G, Mogorovich A, Menchini Fabris F, et al. Long-term followup after elective testis sparing surgery for Leydig cell tumors: a single center experience. J Urol. 2007; 178(3 Pt 1): 872–6; quiz 1129.
    CrossRefPubMed
  15. Carmignani L, Salvioni R, Gadda F, et al. Long-term followup and clinical characteristics of testicular Leydig cell tumor: experience with 24 cases. J Urol. 2006; 176(5): 2040–3; discussion 2043.
    CrossRefPubMed
  16. Suardi N, Strada E, Colombo R, et al. Leydig cell tumour of the testis: presentation, therapy, long-term follow-up and the role of organ-sparing surgery in a single-institution experience. BJU Int. 2009; 103(2): 197–200.
    CrossRefPubMed
  17. Loeser A, Vergho DC, Katzenberger T, et al. Testis-sparing surgery versus radical orchiectomy in patients with Leydig cell tumors. Urology. 2009; 74(2): 370–372.
    CrossRefPubMed
  18. Bozzini G, Picozzi S, Gadda F, et al. Long-term follow-up using testicle-sparing surgery for Leydig cell tumor. Clin Genitourin Cancer. 2013; 11(3): 321–324.
    CrossRefPubMed
  19. 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(4): 1117–1122.
    CrossRefPubMed
  20. Mortensen MS, Lauritsen J, Gundgaard MG, et al. A nationwide cohort study of stage I seminoma patients followed on a surveillance program. Eur Urol. 2014; 66(6): 1172–1178.
    CrossRefPubMed
  21. Ulbright TM, Emerson RE. Neoplasms of the testis. In: Bostwick DG, Cheng L. ed. Urologic surgical pathology. 3rd ed. Elsevier, Philadephia 2013: 738–828.
  22. Song DH, Jeong SM, Park JT, et al. Large cell calcifying sertoli cell tumor of the testis: a case study and review of the literature. Korean J Pathol. 2014; 48(1): 50–53.
    CrossRefPubMed
  23. Kawamura M, Nakazawa S, Ueda N, et al. [Sertoli cell tumor of the testis : a case report and questionnaire survey on the reported cases in Japan]. Hinyokika Kiyo. 2014; 60(6): 295–298.
    PubMed
  24. Akagi N, Suzuki T, Kaizuka Y, et al. [SERTOLI CELL TUMOR OF TESTIS: CASE REPORT]. Nihon Hinyokika Gakkai Zasshi. 2021; 112(3): 146–149.
    CrossRefPubMed
  25. Berney D, Ulbright T. Difficult or Newly Described Morphologic Entities in Testicular Neoplasia. Genitourinary Pathology. 2015: 471–481.
    CrossRef
  26. Guha P, Sarkar D, Ray A, et al. Malignant large cell calcifying sertoli cell tumor of testis with skip metastasis to lung presented with peutz-jeghers syndrome. Tanaffos. 2012; 11(4): 63–68.
  27. Ulbright TM, Young RH. Pseudo-"solid pseudopapillary neoplasms" of the testis: in reality Sertoli cell tumors. Hum Pathol. 2019; 83: 228–230.
    CrossRefPubMed
  28. Gourgari E, Saloustros E, Stratakis CA. Large-cell calcifying Sertoli cell tumors of the testes in pediatrics. Curr Opin Pediatr. 2012; 24(4): 518–522.
    CrossRefPubMed
  29. Anderson WJ, Gordetsky JB, Idrees MT, et al. Large cell calcifying Sertoli cell tumour: a contemporary multi-institutional case series highlighting the diagnostic utility of PRKAR1A immunohistochemistry. Histopathology. 2022; 80(4): 677–685.
    CrossRefPubMed
  30. Lan T, Zhuang H, Zhang H. Large cell calcifying sertoli cell tumour of the testis: a case report. Int J Clin Exp Pathol. 2016; 9(3): 3972–3977.
  31. Kumar H, Tanveer N, Gupta N, et al. Large Cell Calcifying Sertoli Cell Tumour of Testis-A Rare Case Report. J Clin Diagn Res. 2016; 10(11): ED03–ED05.
    CrossRefPubMed
  32. Kuroda N, Senzaki T, Yamasaki Y, et al. Sertoli cell tumor of the testis (not otherwise specified) with the expression of neuroendocrine markers and without the expression of inhibin-alpha. Pathol Int. 2004; 54(9): 719–724.
    CrossRefPubMed
  33. Yu S, Sholl LM, Siegmund S, et al. Large cell calcifying Sertoli cell tumour: molecular and immunohistochemical assessment of a series comprising non-metastasising and metastasising neoplasms. Histopathology. 2023; 82(7): 1079–1088.
    CrossRefPubMed
  34. Tang X, Hu Y, Zhou H, et al. The sclerosing sertoli cell tumor of the testis: a case report. Diagn Pathol. 2023; 18(1): 61.
    CrossRefPubMed
  35. Hanson JA, Ambaye AB. Adult testicular granulosa cell tumor: a review of the literature for clinicopathologic predictors of malignancy. Arch Pathol Lab Med. 2011; 135(1): 143–146.
    CrossRefPubMed
  36. Dieckmann KP, Bertolini J, Wülfing C. Adult Granulosa Cell Tumor of the Testis: A Case Report with a Review of the Literature. Case Rep Urol. 2019; 2019: 7156154.
    CrossRefPubMed
  37. Grogg JB, Schneider K, Bode PK, et al. Risk factors and treatment outcomes of 239 patients with testicular granulosa cell tumors: a systematic review of published case series data. J Cancer Res Clin Oncol. 2020; 146(11): 2829–2841.
    CrossRefPubMed
  38. Cornejo KM, Young RH. Adult granulosa cell tumors of the testis: a report of 32 cases. Am J Surg Pathol. 2014; 38(9): 1242–1250.
    CrossRefPubMed
  39. Wahlstedt ER, Allison DB, Bell JR. Adult granulosa cell tumour of the testis: an uncommon tumour. BMJ Case Rep. 2022; 15(12).
    CrossRefPubMed
  40. Ory J, Blankstein U, Gonzalez DC, et al. Outcomes of organ-sparing surgery for adult testicular tumors: A systematic review of the literature. BJUI Compass. 2021; 2(5): 306–321.
    CrossRefPubMed
  41. Paffenholz P, Pfister D, Heidenreich A. Testis-preserving strategies in testicular germ cell tumors and germ cell neoplasia . Transl Androl Urol. 2020; 9(Suppl 1): S24–S30.
    CrossRefPubMed
  42. Vatta F, Raffaele A, Pasqua N, et al. Juvenile Granulosa Cell Tumor of the Testis: Prenatal Diagnosis and Management. European J Pediatr Surg Rep. 2019; 7(1): e93–e95.
    CrossRefPubMed
  43. Lin KH, Lin SE, Lee LM. Juvenile granulosa cell tumor of adult testis: a case report. Urology. 2008; 72(1): 230.e11–230.e13.
    CrossRefPubMed
  44. Shukla AR, Huff DS, Canning DA, et al. Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis. J Urol. 2004; 171(5): 1900–1902.
    CrossRefPubMed
  45. Anraku T, Hashidate H, Nakahara A, et al. A 40-year-old man with a rapidly growing intrascrotal tumor in the fibroma-thecoma group. IJU Case Rep. 2022; 5(3): 175–178.
    CrossRefPubMed
  46. Algarni AM, Junejo NN, Alkhateeb SS, et al. Testicular fibroma: A case report and literature review. Urol Ann. 2021; 13(3): 308–311.
    CrossRefPubMed
  47. Tsili AC, Bertolotto M, Rocher L, et al. Sonographically indeterminate scrotal masses: how MRI helps in characterization. Diagn Interv Radiol. 2018; 24(4): 225–236.
    CrossRefPubMed
  48. Allen DC, Moorehead RJ. Mixed sex-cord stromal tumour of the testis. Ulster Med J. 1997; 66(1): 54–56.
    PubMed
  49. Michalova K, Michal M, Kazakov DV, et al. Primary signet-ring stromal tumor of the testis. Virchows Arch. 2005; 447(1): 107–110.
    CrossRefPubMed
  50. Kuo CY, Wen MC, Wang J, et al. Signet-ring stromal tumor of the testis: a case report and literature review. Hum Pathol. 2009; 40(4): 584–587.
    CrossRefPubMed
  51. Fichtner A, Fisseler-Eckhoff A, Kramer W, et al. Primary signet-ring stromal tumour of the testis: case report with literature review. APMIS. 2019; 127(1): 45–49.
    CrossRefPubMed
  52. Kao CS, Ulbright TM. A Morphologic and Immunohistochemical Comparison of Nuclear β-Catenin Expressing Testicular Sertoli Cell Tumors and Pancreatic Solid Pseudopapillary Neoplasms Supporting Their Continued Separate Classification. Am J Surg Pathol. 2020; 44(8): 1082–1091.
    CrossRefPubMed
  53. Renne SL, Valeri M, Tosoni A, et al. Myoid gonadal tumor. Case series, systematic review, and Bayesian analysis. Virchows Arch. 2021; 478(4): 727–734.
    CrossRefPubMed
  54. Kao CS, Ulbright TM. Myoid gonadal stromal tumor: a clinicopathologic study of three cases of a distinctive testicular tumor. Am J Clin Pathol. 2014; 142(5): 675–682.
    CrossRefPubMed
  55. Du S, Powell J, Hii A, et al. Myoid gonadal stromal tumor: a distinct testicular tumor with peritubular myoid cell differentiation. Hum Pathol. 2012; 43(1): 144–149.
    CrossRefPubMed
  56. Roth LM, Ramzy I. Perspectives on signet ring stromal cell tumor and related signet ring cell lesions of the gonads. Adv Anat Pathol. 2014; 21(6): 443–449.
    CrossRefPubMed
  57. Gupta P, Mehta V. Sex cord stromal tumors-general. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testissexcordgeneral.html (20.10.2023).
  58. Michalova K, Michal M, Kazakov DV, et al. Primary signet ring stromal tumor of the testis: a study of 13 cases indicating their phenotypic and genotypic analogy to pancreatic solid pseudopapillary neoplasm. Hum Pathol. 2017; 67: 85–93.
    CrossRefPubMed
  59. Madsen E, Hultberg B. Metastasizing Sertoli Cell Tumours of the Human Testis—a Report of Two Cases and a Review of the Literature. Acta Oncol. 2009; 29(7): 946–949.
    CrossRefPubMed
  60. Athanasiou AE, Barbounis V, Dimitriadis M, et al. Successful chemotherapy for disseminated testicular Sertoli cell tumour. Brit Jo Urol. 1998; 61(5): 456–457.
    CrossRefPubMed
  61. Dieckmann KP, Loy V. Response of metastasized sex cord gonadal stromal tumor of the testis to cisplatin-based chemotherapy. J Urol. 1994; 151(4): 1024–1026.
    CrossRefPubMed
  62. Morinaga S, Aoki S, Tsuzuki T, et al. Malignant testicular unclassified sex cord stromal tumor: a case report. J Med Case Rep. 2022; 16(1): 406.
    CrossRefPubMed
  63. DeVita VT, Rosenberg S, Lawrence TS. DeVita, Hellman, and Rosenberg's Cancer: Principles & Practice of Oncology. Lippincott Williams & Wilkins 2008: 1482–1483.

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