COW-2021-28

1. c; 2. d

Juvenile Granulosa Cell Tumor

Granulosa cell tumor of the testis is a sex cord-stromal cell tumor that exists as adult and juvenile type. Although rare, juvenile granulosa cell tumor (JGCT) of the testis is the most common neoplasm of the testis during the first 6 months of life. It typically presents as a scrotal mass which on ultrasound evaluation shows as circumscribed tumor with multicystic appearance. Prenatal diagnosis is possible.

JGCT is a benign neoplasm, hormonally inactive and its prognosis is very good. The standard surgical therapy is inguinal orchiectomy. Microscopic examination typically shows lobular growth, with variably-sized and shaped follicles filled with mucoid material and lined by a single layer or multiple layers of rounded granulosa and theca-like cells. In non-follicular areas, the tumor cells typically grow diffusely as cords or have a reticular appearance. The stroma is fibrous or fibromyxoid. The tumor cells are mostly small to medium sized with round to oval nuclei containing inconspicuous nucleoli and moderate to abundant pale to lightly eosinophilic cytoplasm. The nuclei vary from pale to hyperchromatic and lack grooves, unlike adult type. Mitoses are abundant. Call-Exner bodies are uncommon, unlike the adult type.

The neoplastic granulosa cells are positive for inhibin, calretinin, WT1, FOXL2, SF-1, and SOX9 and negative for glypican-3, SALL4 and OCT3/4. In some cases of JGCT serum AFP is elevated, as in our case, which makes the clinical distinction from germ cell tumor difficult. However, in very young infants serum AFP level can be physiologically high with concentrations at birth ranging at 15,000-146,000 ng/ml so the serum levels of AFP are not helpful in this age group. In addition, there are non-germ cell tumors of the urological tract, such as ovarian juvenile granulosa cell tumor, that have been reported to produce AFP and thus, raised serum AFP is not pathognomonic of a germ cell tumor. Therefore, AFP levels need to be interpreted in the context of the patient’s age, clinical presentation, and histologic features of the lesion.

Bulotta, A.L., Molinaro, F., Angotti, R. et al. Juvenile granulosa cell tumor of the testis: prenatal diagnosis and prescrotal approach. Ital J Pediatr 38, 67 (2012). https://doi.org/10.1186/1824-7288-38-67

Zugor V, Labanaris AP, Witt J, Seidler A, Weingärtner K, Schott GE. Congenital juvenile granulosa cell tumor of the testis in newborns. Anticancer Res. 2010 May;30(5):1731-4. PMID: 20592370.

Kao, C.-S., Cornejo, K. M., Ulbright, T. M., & Young, R. H. (2015). Juvenile Granulosa Cell Tumors of the Testis: A Clinicopathologic Study of 70 Cases with Emphasis on Its Wide Morphologic Spectrum. The American Journal of Surgical Pathology, 39(9), 1159–1169. http://doi.org/10.1097/PAS.0000000000000450

El-Bahrawy M. α-Fetoprotein-Producing Non-Germ Cell Tumors of the Urological System. Rev Urol. 2011;13(1):14-9. PMID: 21826124; PMCID: PMC3151583.

Bader D, Riskin A, Vafsi O, Tamir A, Peskin B, Israel N, Merksamer R, Dar H, David M. Alpha-fetoprotein in the early neonatal period–a large study and review of the literature. Clin Chim Acta. 2004 Nov;349(1-2):15-23. doi: 10.1016/j.cccn.2004.06.020. PMID: 15469851.

Radiana Trifonova; Andrea Lightle
Albany Medical Center, Albany
USA

Testis

testis, granulosa cell tumor, pediatric pathology, AFP