Sertoli-Leydig tumor and male pseudohermaphroditism discovered during inguinal hernia surgery
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.
KeywordsMale pseudohermaprhoditism Sertoli-Leydig tumor Inguinal Hernia
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- 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
- 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
- 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
- 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
- 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
- Subramaniam A, Singh R, Tilak P, et al. Androgen insensitivity syndrome: ten years of our experience. Front Brosci 2013;5:779:84Google Scholar
- 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
- Metzinger DS, Webb MJ. Surgical management of Sertoli-Leydig cell tumors of the ovary. CME J GinecolOncol 2002;7:140–142Google Scholar
- 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
- 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
- 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
- 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