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Presented by Dr. Epstein and prepared by Dr. Daniel Miller
A 11 year old boy underwent an orchiectomy for an undescended testicle (cryptorchidism).
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Question 1 of 1
1. Question
A 11 year old boy underwent an orchiectomy for an undescended testicle (cryptorchidism).
Choose the Correct Diagnosis:
Correct
Answer: A. Germ cell neoplasia in situ
Discussion: Germ cell neoplasia in situ (GCNIS) is the new nomenclature for what was formerly called intratubular germ cell neoplasia. It is the precursor lesion to invasive germ cell tumors in post-pubertal males. GCNIS is associated with up to a 50% risk of developing invasive germ cell tumor within 5 years if untreated and up to 90% risk in 7 years. The risk of germ cell tumor is also increased in the contralateral testis. Associated risk factors include cryptorchidism (4% risk) and infertility (1%–2% of biopsies) and much higher in intersex disorders. GCNIS is almost always seen adjacent to malignant germ cell tumors in post-pubertal males. It is absent adjacent to pre-pubertal-type teratomas, epidermoid cysts, and prepubertal yolk sac tumors. The average age where GCNIS is seen is 15–35 years, and extremely rarely seen in prepubertal testes apart from an intersex disorder. Consequently, the finding of GCNIS in the current case is unusual. GCNIS occurs only in tubules lacking spermatogenesis. Within these tubules are enlarged cells along the periphery of the tubules with clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli, resembling seminoma cells. In contrast, immature germ cells in prepubertal testes are small with round and regular nuclear contours, densely packed chromatin and no nucleoli. Occasionally, spermatogonia are otherwise identical, yet can be binucleated or have enlarged nuclei (giant spermatogonia). PLAP and OCT4 can sometimes be seen in infants but subsequently are negative in nonneoplastic germ cells, where PLAP and OCT4 positivity are excellent specific markers of GCNIS. Surgically bringing the testis into the scrotum even before 2 years of age is not entirely protective of subsequent neoplasia. In some cases, especially if the cryptorchid testis is intra-abdominal where there is a higher risk of developing germ cell tumors compared to cryptorchid testes closer to the inguinal canal, orchiectomy may be performed.
Reference:
Williamson SR, Delahunt B, Magi-Galluzzi C, Algaba F, Egevad L, Ulbright TM, Tickoo SK, Srigley JR, Epstein JI, Berney DM; Members of the ISUP Testicular Tumour Panel. Histopathology. 2017 Feb;70(3):335-346. doi: 10.1111/his.13102. PMID: 27747907Incorrect
Answer: A. Germ cell neoplasia in situ
Discussion: Germ cell neoplasia in situ (GCNIS) is the new nomenclature for what was formerly called intratubular germ cell neoplasia. It is the precursor lesion to invasive germ cell tumors in post-pubertal males. GCNIS is associated with up to a 50% risk of developing invasive germ cell tumor within 5 years if untreated and up to 90% risk in 7 years. The risk of germ cell tumor is also increased in the contralateral testis. Associated risk factors include cryptorchidism (4% risk) and infertility (1%–2% of biopsies) and much higher in intersex disorders. GCNIS is almost always seen adjacent to malignant germ cell tumors in post-pubertal males. It is absent adjacent to pre-pubertal-type teratomas, epidermoid cysts, and prepubertal yolk sac tumors. The average age where GCNIS is seen is 15–35 years, and extremely rarely seen in prepubertal testes apart from an intersex disorder. Consequently, the finding of GCNIS in the current case is unusual. GCNIS occurs only in tubules lacking spermatogenesis. Within these tubules are enlarged cells along the periphery of the tubules with clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli, resembling seminoma cells. In contrast, immature germ cells in prepubertal testes are small with round and regular nuclear contours, densely packed chromatin and no nucleoli. Occasionally, spermatogonia are otherwise identical, yet can be binucleated or have enlarged nuclei (giant spermatogonia). PLAP and OCT4 can sometimes be seen in infants but subsequently are negative in nonneoplastic germ cells, where PLAP and OCT4 positivity are excellent specific markers of GCNIS. Surgically bringing the testis into the scrotum even before 2 years of age is not entirely protective of subsequent neoplasia. In some cases, especially if the cryptorchid testis is intra-abdominal where there is a higher risk of developing germ cell tumors compared to cryptorchid testes closer to the inguinal canal, orchiectomy may be performed.
Reference:
Williamson SR, Delahunt B, Magi-Galluzzi C, Algaba F, Egevad L, Ulbright TM, Tickoo SK, Srigley JR, Epstein JI, Berney DM; Members of the ISUP Testicular Tumour Panel. Histopathology. 2017 Feb;70(3):335-346. doi: 10.1111/his.13102. PMID: 27747907
