An 11-year-old girl with delayed puberty is found to have bilateral streak gonads and a 46,XY karyotype. Prophylactic gonadectomy is performed. Histology of one gonad shows a tumour with nests of large germ cells admixed with smaller sex cord-derived cells and eosinophilic hyaline Call-Exner-like bodies, with foci of calcification. The diagnosis is:
- A Juvenile granulosa cell tumour
- B Pure dysgerminoma
- C Gonadoblastoma ✓
- D Sex cord tumour with annular tubules
Correct answer: C. Gonadoblastoma
Explanation
Gonadoblastoma is a mixed germ cell and sex cord-stromal tumour arising almost exclusively in dysgenetic gonads carrying Y chromosomal material, such as 46,XY complete gonadal dysgenesis or androgen insensitivity. It contains germ cells resembling dysgerminoma cells plus sex cord elements with hyaline bodies and frequent calcification. Pure dysgerminoma lacks the sex cord component, while sex cord tumour with annular tubules is associated with Peutz-Jeghers syndrome, not dysgenesis.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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