An adolescent with delayed puberty and primary amenorrhoea has a 46,XY karyotype with streak gonads. During laparoscopic evaluation, bilateral gonadectomy is planned. Removal is advised chiefly because these dysgenetic gonads carry a significant risk of developing which tumour?
- A Juvenile granulosa cell tumour
- B Gonadoblastoma, with potential progression to dysgerminoma ✓
- C Immature teratoma
- D Small cell carcinoma of hypercalcaemic type
Correct answer: B. Gonadoblastoma, with potential progression to dysgerminoma
Explanation
Intra-abdominal dysgenetic gonads carrying Y chromosomal material have a high incidence of gonadoblastoma, a mixed germ cell and sex cord-stromal tumour that itself can progress to invasive dysgerminoma. This underpins the recommendation for prophylactic gonadectomy at diagnosis in patients with complete androgen insensitivity or pure gonadal dysgenesis with a Y chromosome. The remaining options are not specifically linked to dysgenetic Y-containing gonads.
Reference: Williams Gynecology, 3rd ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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