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Case Report
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A case of bilateral Sertoli cell adenoma in androgen insensitivity syndrome

Year 2017, Volume: 56 Issue: 3, 154 - 156, 01.09.2017

Zeynep Öztürk İnal Nursadan Gergerlioğlu İlknur Küçükosmanoğlu Meryem İlkay Karanis

https://doi.org/10.19161/etd.393585

Abstract

Androgen insensitivity syndrome, previously referred to as testicular feminization syndrome, is an X-linked recessive disorder that is characterized by a mutation in the q11-q12 region of the X chromosome, which results in a deformed androgen receptor gene. Patients with androgen insensitivity syndrome may develop testicular tumors, especially seminomas after puberty. A 35-year-old female patient presented with the complaint of primary amenorrhea and had masses of approximately 4 cm size palpated in the bilateral inguinal regions. The masses were excised and the histopathological examination was reported as bilateral Sertoli cell adenoma. Although the risk of bilateral gonadal tumor development is low in patients with androgen insensitivity syndrome, such malignancies should not be disregarded.

Keywords

Sertoli cell adenoma androgen insensitivity syndrome

References

  • Siminas S, Kokai G, Kenny SE. Complete androgen insensitivity syndrome associated with bilateral Sertoli cell adenomas and paratesticular leiomyomas: Case report and review of the literature. J Pediatr Urol 2013;9(1):31-4.
  • Hughes IA, Deeb A. Androgen resistance. Best Pract Res Clin Endocrinol Metab 2006;20(4):577-98
  • Baksu A, Kabukcuoglu F, Baksu B, Goker N. Bilateral Sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome. Europ Jour of Obst and Gynand Reproductive Biol 2004;114(1):104-7.
  • Cools M, Drop SLS, Wolffenbuttel KP, Oosterhuis JW, Looijenga LH. Germ cell tumours in the intersex gonad: Old paths, new directions, moving frontiers. Endocr Rev 2006;27(5):468-84.
  • Boehmer AL, Brinkmann O, Brüggenwirth H, et al. Genotype versus phenotype in families with androgen insensitivity syndrome. J Clin Endocrinol Metab 2001;86(9):4151-60.
  • Fallat ME, Donahoe PK. Intersex genetic anomalies with malignant potential. Curr Opin Pediatr 2006;18(3):305-11.
  • Bouvattier C. Androgen receptor defects: Syndromes of androgen insensitivity. Disorders of sex development: endocrine aspects. In: Gearhart, Rink, Mouriquand, editors. Pediatric Urology. 2nd ed. Philadelphia: Saunders Elsevier; 2010:472-3.
  • Slijper FM, Frets PG, Boehmer AL, Drop SL, Niermeijer MF. Androgen insensitivity syndrome(AIS): Emotional reactions of parents and adult patients to clinical diagnosis of AIS and its confirmation by androgen receptor gene mutation analysis. Horm Res 2000;53(1):9-15.
  • Vega IF, Juanes JS, Pravia CG. Bilateral Sertoli cell adenoma in gonads, associated with serous cystadenoma. Pol J Pathol 2014;65(2):154-6.

Androjen duyarsızlık sendromunda bilateral Sertoli hücreli adenom olgusu

Year 2017, Volume: 56 Issue: 3, 154 - 156, 01.09.2017

Zeynep Öztürk İnal Nursadan Gergerlioğlu İlknur Küçükosmanoğlu Meryem İlkay Karanis

https://doi.org/10.19161/etd.393585

Abstract

Önceden testiküler feminizasyon sendromu olarak isimlendirilen androjen duyarsızlık sendromu androjen reseptör gen üzerinde lokalize X kromozomundaki q11-q12 mutasyonunun bulunduğu, X’e bağlı geçiş gösteren resesif bir hastalıktır. Androjen duyarsızlık sendromlu hastalarda puberteden sonra testiküler tümörler, özellikle de seminom gelişebilmektedir. Primer amenore şikayeti ile başvuran 35 yaşındaki hastamızın bilateral inguinal bölgede palpasyonla ele gelen yaklaşık 4 cm’lik kitlenin, eksizyon sonucu histopatolojik değerlendirilmesinde bilateral Sertoli hücreli adenom olarak rapor edilmiştir. Androjen duyarsızlık sendromlu hastalarda bilateral gonadal tümör gelişim riski düşük olsa da bu tür malignitelerin de görülebileceği göz ardı edilmemelidir.

Keywords

Sertoli hücreli adenom androjen duyarsızlık sendromu

References

  • Siminas S, Kokai G, Kenny SE. Complete androgen insensitivity syndrome associated with bilateral Sertoli cell adenomas and paratesticular leiomyomas: Case report and review of the literature. J Pediatr Urol 2013;9(1):31-4.
  • Hughes IA, Deeb A. Androgen resistance. Best Pract Res Clin Endocrinol Metab 2006;20(4):577-98
  • Baksu A, Kabukcuoglu F, Baksu B, Goker N. Bilateral Sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome. Europ Jour of Obst and Gynand Reproductive Biol 2004;114(1):104-7.
  • Cools M, Drop SLS, Wolffenbuttel KP, Oosterhuis JW, Looijenga LH. Germ cell tumours in the intersex gonad: Old paths, new directions, moving frontiers. Endocr Rev 2006;27(5):468-84.
  • Boehmer AL, Brinkmann O, Brüggenwirth H, et al. Genotype versus phenotype in families with androgen insensitivity syndrome. J Clin Endocrinol Metab 2001;86(9):4151-60.
  • Fallat ME, Donahoe PK. Intersex genetic anomalies with malignant potential. Curr Opin Pediatr 2006;18(3):305-11.
  • Bouvattier C. Androgen receptor defects: Syndromes of androgen insensitivity. Disorders of sex development: endocrine aspects. In: Gearhart, Rink, Mouriquand, editors. Pediatric Urology. 2nd ed. Philadelphia: Saunders Elsevier; 2010:472-3.
  • Slijper FM, Frets PG, Boehmer AL, Drop SL, Niermeijer MF. Androgen insensitivity syndrome(AIS): Emotional reactions of parents and adult patients to clinical diagnosis of AIS and its confirmation by androgen receptor gene mutation analysis. Horm Res 2000;53(1):9-15.
  • Vega IF, Juanes JS, Pravia CG. Bilateral Sertoli cell adenoma in gonads, associated with serous cystadenoma. Pol J Pathol 2014;65(2):154-6.
There are 9 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Case Reports
Authors

Zeynep Öztürk İnal 0000-0002-8766-2079

Nursadan Gergerlioğlu 0000-0003-3637-8710

İlknur Küçükosmanoğlu 0000-0002-5181-6152

Meryem İlkay Karanis 0000-0002-1097-4592

Publication Date September 1, 2017
Submission Date August 2, 2016
Published in Issue Year 2017 Volume: 56 Issue: 3

Cite

Vancouver Öztürk İnal Z, Gergerlioğlu N, Küçükosmanoğlu İ, Karanis Mİ. A case of bilateral Sertoli cell adenoma in androgen insensitivity syndrome. EJM. 2017;56(3):154-6.
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