Advertisement

Central European Journal of Medicine

, Volume 9, Issue 5, pp 653–656 | Cite as

Sertoli-Leydig tumor and male pseudohermaphroditism discovered during inguinal hernia surgery

  • Anna Pallisera
  • Rosa Jorba
  • Luis Ortiz de Zárate
  • Jordi Català
  • Irene Moysset
  • Myreia Jimeno
Case Report
  • 33 Downloads

Abstract

The diagnosis of inguinal hernia is usually clinical and it is performed with high sensitivity and specificity. Very occasionally, it may be confused with other diseases (lymphadenopathy, testicular pathology,etc). We report a rare case of a 80-year-old woman with a clinical diagnosis of hernia, which was underwent surgery and a tumor from the hernia orifice was found. After histological analysis we discovered that the misdiagnosed hernia was actually a tumor on a rudimentary testis. After radiological, gynecological and cytogenetic assessment we obtained an unexpected diagnosis: Male psheudohermaphroditism and Sertoli-Leydigtumor (SLCT) development on the testis. Diagnostic guidance for disorders of sexual development is based almost entirely on pediatric experience and very few guidelines are available for adults. Male pseudohermaphroditism is an intersex condition in which the carriers show a phenotype that includes external female genitalia, but a male genetic and gonadal sex. SLCT are sex-cord stromal tumors which develop in ovary and very rarely in the testis, representing 0.1–0.5% of ovarian tumors and less than 0.2% of testicular tumors. Thus far 24 case have been reported in the literature in which SLCT tumor has developed on testis.

Keywords

Male pseudohermaprhoditism Sertoli-Leydig tumor Inguinal Hernia 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. [1]
    Rey RA, Grinspon RP. Normal male sexual differentiation and aetiology of disorders of sex development. Best Pract Res ClinEndocrinolMetab 2011; 25(2):221–238CrossRefGoogle Scholar
  2. [2]
    Liao X, Liang D, Li Y, et al. Mutation analysis of the SRY, NR5A1 and DHH genes in six Chinese 46. XY women. J MaternFetalNeonaltal Med 2011;24(6):863–866Google Scholar
  3. [3]
    Larson A, Nokoff NJ, Travers S. Disorders of sex development: clinically relevant genes involved in gonadal differentiation. Discov Med 2012;14(78):301–309PubMedGoogle Scholar
  4. [4]
    Manrique-Hurtado H, Calderoón-Ticona J, Medina-Sánchez C, et al. Pseudohermafroditismomasculino: insensibilidadandrogénicacompleta. Reporte de uncaso. Rev Soc Peru Med Interna 2007;20(1):26–28Google Scholar
  5. [5]
    Jarzabek K, Philibert P, Koda M, et al. Primary amenorrhea in a young Polish woman with complete androgen insensitivity syndrome and sertolileydig cell tumor: identification of a new androgen receptor gene mutation and evidence of aromatase hyperactivity and apoptosis dysregulation within the tumor. Gynecological Endocrinology 2007;23(9):499–504PubMedCrossRefGoogle Scholar
  6. [6]
    Kriplari A, Savithrisowmya S, Argwal N, et al. A rare case of large epididymal cyst in androgen insensitivity syndrome removed laparoscopically. J Minim Invasive Gynecol 2009;16(4)504–506CrossRefGoogle Scholar
  7. [7]
    Rutger JKL. The case reported as bilateral Sertoli-Leydig cell tumor in a 61-year old woman with uterine apalasia may instead represent complete androgen insensitivity syndrome. Int J GynecolPathol 2011;30:395Google Scholar
  8. [8]
    Dell’Edera D, Malvasi A, Vitullo E, et al. Androgen insensitivity syndrome (or Morris Syndrome) and other associated pathologies. Eur Rev Med PaharmacolSci 2010;14(11):947–957Google Scholar
  9. [9]
    Subramaniam A, Singh R, Tilak P, et al. Androgen insensitivity syndrome: ten years of our experience. Front Brosci 2013;5:779:84Google Scholar
  10. [10]
    Berra M, Williams EL, Muroni B, et al. Recognition of 5α-reductasa-2 deficiency in an adult female 46XY DSD clinic. Eur J Endocrinol 2011;164(6):1019–1025PubMedCrossRefGoogle Scholar
  11. [11]
    Paliwal P, Sharma A, Birla S, et al. Identification of novel SRY mutations and SF1 (NR5A1) changes in patients with pure gonadal dysgenesis and 46,XY karyotype. Mol Hum Reprod 2011;17(6):372–378PubMedCrossRefGoogle Scholar
  12. [12]
    Pérez-Becerra R, Santana-Ríos Z, Hulda-Graus S, et al. Tumores del Estroma Gonadal Sertoli-Leydig en el Hospital General Dr Manuel GeaGonzález. Rev MexUrol 2009;69(3):174–177Google Scholar
  13. [13]
    Metzinger DS, Webb MJ. Surgical management of Sertoli-Leydig cell tumors of the ovary. CME J GinecolOncol 2002;7:140–142Google Scholar
  14. [14]
    Young RH. Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems. Modern Pathology 2005;18:S81–S98PubMedCrossRefGoogle Scholar
  15. [15]
    Caringella A, Loizzi V, Resta L, et al. A cause of Sertoli-Leydig cell tumor in a postmenopausal woman.Int J Gynecol Cancer 2006;16:435–438PubMedCrossRefGoogle Scholar
  16. [16]
    Sachdeva P, Arora R, Dubey C, et al. Sertoli-Leydig cell tumor: a rare ovarian neoplasm. Case report and review of littérature.GynecolEndocrinol 2008;24(4):230–234Google Scholar
  17. [17]
    Lou W, Cao D, Yang J, et al. RetiformSertoli-Leydig cell tumor of ovary in a 9-year-old girl: case report and review of the literature. Int J ClinOncol 2011;16(6):705–708Google Scholar
  18. [18]
    Guo L, Yang X, Zun H, et al. Sertoli-Leydig cell tumor presenting hyperestrogenism in a postmenopausal woman: a case report and review of the literature. Taiwan J ObstetGynecol 2012;51(4):620–624CrossRefGoogle Scholar
  19. [19]
    Gui T, Cao D, Shen K, et al. A clinicopathological analysis of 40 cases of ovarian Sertoly-Leydig cell tumors.GynecolOncol 2012;127(2):384–389Google Scholar
  20. [20]
    Xiao H, Li H, Zuo J. Ovarian Sertoli-Leydig cell tumor: a report of severe cases and a review fo the literature. GynecolEndocrinol 2012 DOI: 10.3109/09513590.2012.738723 [Epub ahead of print]Google Scholar

Copyright information

© Versita Warsaw and Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Anna Pallisera
    • 1
  • Rosa Jorba
    • 1
  • Luis Ortiz de Zárate
    • 1
  • Jordi Català
    • 2
  • Irene Moysset
    • 3
  • Myreia Jimeno
    • 3
  1. 1.Department of Digestive and General SurgeryHospital General de l’HospitaletCataloniaSpain
  2. 2.Department of RadiologyHospital General de l’HospitaletCataloniaSpain
  3. 3.Department of PahtologyHospital General de l’HospitaletCataloniaSpain

Personalised recommendations