An adolescent with delayed puberty and a 46,XY karyotype with complete androgen insensitivity is found to have bilateral inguinal gonadal masses. Histology shows nests of germ cells admixed with sex cord-derived granulosa/Sertoli cells and eosinophilic basement membrane deposits. Which complication must gonadectomy prevent, and why is it indicated?
- A Development of dysgerminoma from the germ cell component ✓
- B Endometrial carcinoma from unopposed oestrogen
- C Transformation into Leydig cell sarcoma
- D Recurrence of virilising adrenal rests
Correct answer: A. Development of dysgerminoma from the germ cell component
Explanation
The histology is gonadoblastoma, a benign mixed germ cell and sex cord-stromal tumour that arises almost exclusively in dysgenetic gonads carrying Y chromosomal material. Its importance lies in progression to invasive germ cell malignancy, most commonly dysgerminoma, which justifies prophylactic bilateral gonadectomy once puberty is complete. There is no endometrium in complete androgen insensitivity syndrome, so option B cannot occur.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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