Hamartomatous nodule, sertoli cell adenoma in complete androgen insensitivity syndrome with Wolffian/Müllerian duct remnants: An unusual case report

  • Prashant Vijay Kumavat Assitant Professor LTMMC and LTMGH sion mumbai . Mahrashtra.
  • Chetan S Chaudhari
  • Anita Padmanabhan
  • Nitin M Gadgil
  • Sangita Margam
  • Ganesh R Kshirsagar
Keywords: Complete androgen insensitivity syndrome, Gonads, Hamartoma, Müllerian, Wolffian.


Complete Androgen insensitivity syndrome is a disorder of hormone resistance characterized by a female phenotype with an XY karyotype and testes producing age-appropriate normal or higher concentrations of androgens. 26 year old unmarried phenotypically female presented with left inguinal swelling and never consulted for amenorrhea. MRI finding revealed bilateral inguinal masses, uterus cervix not visualized and hypoplatic vagina was noted. She had advised karyotyping which was 46 XY.  Hormonal investigation showed testosterone, estradiol and LH was increase and FSH was within normal limits.  Patient underwent laparoscopic bilateral gonadectomy with left open hernia repair. Histopathology examination revealed hamartomatous nodule, sertoli cell adenoma, leydig cell hyperplasia, which are more pronounced as age advances as result of absent activity of androgen. Fallopian tube, underdeveloped vas deference, Wolffian/ Müllerian cysts lined by cuboidal epithelium was also noted which may be reminiscent of Wolffian/ Müllerian structure. IHC revealed PLAP and CD 117 which were negative. The clinical, MRI, laboratory and histopathology findings confirmed diagnosis of complete androgen insensitivity syndrome. DOI: 10.21276/APALM.1132 Key words: Complete androgen insensitivity syndrome, Gonads, Hamartoma,   Müllerian, Wolffian. 

Author Biography

Prashant Vijay Kumavat, Assitant Professor LTMMC and LTMGH sion mumbai . Mahrashtra.
Assitant Professor, Department of pathology,  LTMMC AND LTMGH SION mumbai


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Case Report

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