A case report of Sertoli cell tumor in a patient with testicular feminization: many dilemmas for the pathologist

Adv Clin Path. 2002 Apr;6(2):105-10.

Abstract

Aims: The aim of this case report is to present the pathological, clinical, and ethic problems due to the diagnosis of an adnexal mass in a female patient with an undetected androgen insensitivity syndrome.

Methods: We employed both commonly used pathologic techniques, such as hematoxylin-eosin staining and immunohystochemistry, both fluorescence in situ hybridization on paraffin-embedded tissue sections using probes for chromosomes X and Y.

Main results: At pathologic evaluation, a diagnosis of Sertoli cell tumor was made. Fluorescence in situ hybridization showed an XY karyotype of tumor cells. The final diagnosis was bilateral Sertoli cell adenoma in a patient with testicular feminization syndrome.

Conclusion: Androgen insensitivity syndrome is caused by insensitivity of end organs to androgen caused by a non-functioning receptor. The diagnosis of testicular feminization has been made as late as the ninth decade. In this case, two kinds of problem derived from the pathologist point of view: first, the demonstration of chromosomal sex to confirm the diagnosis, and second, the transfer of diagnosis to the patient and to the clinicians.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Androgen-Insensitivity Syndrome / pathology*
  • Androgen-Insensitivity Syndrome / surgery
  • Chromosomes, Human, X
  • Chromosomes, Human, Y
  • Female
  • Feminization
  • Humans
  • In Situ Hybridization, Fluorescence
  • Male
  • Omentum / surgery
  • Ovarian Neoplasms / pathology*
  • Ovarian Neoplasms / surgery
  • Ovariectomy
  • Sertoli Cell Tumor / pathology*
  • Sertoli Cell Tumor / surgery
  • Treatment Outcome