Testis & paratestis
Sex cord stromal tumors
Juvenile granulosa cell tumor
Author: Swapnil U. Rane, M.D.
Last author update: 1 July 2013
Last staff update: 15 September 2022 (update in progress)
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Juvenile granulosa cell tumor testis
Related topics: Adult granulosa cell tumor
Table of Contents
Definition / general | Epidemiology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Immunohistochemistry & special stains | Electron microscopy description | Molecular / cytogenetics descriptionCite this page: Rane S. Juvenile granulosa cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testisgranulosajuvenile.html. Accessed September 22nd, 2023.
Definition / general
- Tumor with cells resembling the granulosa cells of ovary
Epidemiology
- Most frequent congenital testicular tumor
- 6% of all prepubertal testicular tumors
- Most tumors present in the perinatal period, occurrence after first year is rare
Clinical features
- Scrotal or abdominal asymptomatic mass
- More common in left testis (Tumori 2002;88:72)
- 30% involve abdominal testis; often associated with undescended contralateral testis
- Associated with ambiguous genitalia in 20%, most frequently mixed gonadal dysgenesis and hypospadias - these cases have abnormal karyotype: 45 / 46XY mosaicism, trisomy 12 or structural anomalies of Y chromosome
- Not associated with gynecomastia or other endocrine disorders
- Occasionally paraneoplastic hypercalcemia (Klin Padiatr 2013;225:170)
Radiology images
Images hosted on other servers:
Ultrasound
Prognostic factors
- Do not metastasize or recur, unlike ovarian or adult counterpart
Case reports
- Newborn with intra-abdominal testis (Urology 2012;79:1152)
- 2 cases in newborns (Anticancer Res 2010;30:1731)
- 3 month old boy (Can Urol Assoc J 2012;6:E101)
- 3 cases in infants (J Pediatr Hematol Oncol 2010;32:e158)
Treatment
- Orchiectomy
- Enucleation if the tumor is small in view of good prognosis since there have been no reports of metastases or recurrence (Am J Surg Pathol 1985;9:87)
Gross description
- Multicystic tumors with intervening solid areas
- Partly encapsulated
- Slightly smaller tumors as compared to the adult type (0.5 cm to 5 cm)
Gross images
Images hosted on other servers:
Juvenile granulosa cell tumor of testis
Microscopic (histologic) description
- Solid areas intermixed with follicle-like structures filled with mucoid material
- Macrofollicular pattern is common, with multilayered tumor cells surrounded by spindle cell stroma
- Cells are pale and can have abundant luteinized cytoplasm
- Nuclei are round, hyperchromatic and lack grooves, unlike adult type; nucleoli are prominent
- Mitoses are common and sometimes can be numerous, unlike the adult type
- Cellular atypia is typically less than that ovarian granulosa cell tumor
- Call-Exner bodies are uncommon
Immunohistochemistry & special stains
Electron microscopy description
Molecular / cytogenetics description
- Many constitutional chromosomal anomalies, most associated with genital abnormalities of undescended testis, hypospadias or ambiguous genitalia (Trisomy 12, del(4), Pediatr Dev Pathol 2011;14:224) and sex chromosome mosaicism (Am J Surg Pathol 1994;18:316, Am J Surg Pathol 1986;10:577, Am J Surg Pathol 1985;9:737)
- KRAS activation and PTEN loss appear to be causal in ovarian and testicular tumors (Oncogene 2012;31:1504)
- Recently nuclear expression of FOXL2 has been demonstrated in testicular granulosa cell tumors (Arch Pathol Lab Med 2012;136:825); FOXL2 is normally expressed in normal ovarian granulosa cells
- SOX9, normally expressed in nucleus, is sequestered in cytoplasm (J Urol 2008;180:1810)
- FOG2, GATA4 and GATA6 may be important in commitment of undifferentiated gonad towards ovarian development and truncation of testicular development; GATA4 and FOG2 expressed in pediatric ovarian granulosa cell tumors (PLoS One 2012;7:e45914) and mouse testicular granulosa cell tumors (Int J Cancer 2009;124:1122); loss of GATA4 may be favorable prognostic factor
