An ovarian tumour removed from a 15-year-old girl with delayed puberty and a 46,XY karyotype contains both large primitive germ cells and smaller sex cord derivatives arranged in rounded nests, with focal calcifications. This tumour is best described as:

• A Sclerosing stromal tumour, uniformly benign
• B Mixed germ cell tumour requiring bleomycin-etoposide-cisplatin
• C Sex cord tumour with annular tubules, associated with Peutz-Jeghers syndrome
• D Gonadoblastoma, carrying high risk of progression to dysgerminoma ✓

Explanation

Gonadoblastoma is a benign in situ neoplasm mixing germ cells and sex cord stromal elements, occurring almost exclusively in dysgenetic gonads, typically 46,XY pure gonadal dysgenesis or androgen insensitivity, and frequently calcifies. Its importance lies in the substantial risk of overgrowth by dysgerminoma, so prophylactic gonadectomy is indicated. Sex cord tumour with annular tubules links to Peutz-Jeghers syndrome and calcifying variants, a different entity.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.