Juvenile Granulosa Cell Tumors of the Testis

Abstract

All authors have no conflict of interest to declare. Abstract 2The clinical and pathologic features of 70 juvenile granulosa cell tumors of the testis are presented. The patients were from 30-weeks gestational age to 10 years old; 60 of 67 (90%) whose age is known to us were < 6 months old. Sixty-two underwent gonadectomy, 6 wedge excision, and 2 only biopsy. Twenty-six tumors were left-sided, and 22 right-sided. Six occurred in an undescended testis and 2 in dysgenetic gonads. The most common presentation was a testicular mass (65%) followed by an "enlarging testis" (25%). Six of 14 patients in whom it was measured had "elevated" serum AFP, likely physiologically, and 1 had gynecomastia. The tumors measured 0.5 to 5 cm (mean, 1.7; median, 1.5) and were most commonly wellcircumscribed and typically yellow-tan; approximately 2/3 had a cystic component, while 1/3 were entirely solid. Microscopic examination typically showed a lobular growth, punctuated in 67 cases by variably-sized and shaped follicles containing material that was basophilic (21%), eosinophilic (44%), or of both characters (35%); 3 lacked follicles. In non-follicular areas, the tumor cells typically grew diffusely, but occasionally had a corded arrangement (26%) or reticular appearance (29%). The stroma was either fibrous or fibromyxoid; hemorrhage associated with hemosiderin-laiden macrophages was focally seen in 16%. The tumor cells were mostly small to medium sized with round to oval nuclei containing inconspicuous nucleoli and moderate to abundant, but occasionally scant, pale to lightly eosinophilic, sometimes vacuolated, cytoplasm; nuclear grooves were infrequent (6%). Focal columnar morphology was seen in 27% of the tumors. Mitoses were plentiful in 37% and apoptosis was prominent in 46%. Intratubular tumor was seen in 43% and entrapped seminiferous tubules in 70%. Lymphovascular invasion was present in 2 cases, rete testis involvement in 4, and necrosis in 1. Rare features/patterns included: regressed tumor with hyalinization and prominent blood vessels (13%), papillary (4%), basaloid (1%), spindle cell predominant (1%), microcystic (1%), adult granulosa cell-like (1%) 3 patterns, and hyaline globules (1%). Inhibin (16/18), calretinin (8/9), WT1 (6/7), FOXL2 (12/12), SF-1 (12/12), and SOX9 (6/11) were positive, while SALL4 and glypican-3 were consistently negative in the neoplastic granulosa cells. Only one tumor was focally positive for AFP. Juvenile granulosa cell tumor is a rare neoplasm with a wide morphologic spectrum that also occurs rarely in undescended testes and dysgenetic gonads. The solid and reticular patterns may pose diagnostic challenges but the lobular appearance and follicular differentiation are characteristic. Immunohistochemical stains may aid in its distinction from other tumors of young males, particularly yolk sac tumor, a neoplasm that peaks at a somewhat later age. Twenty-four patients with follow-up, including 4 of 6 patients treated with wedge resection /biopsy, had no evidence of disease (2-348 months; mean, 83...

Juvenile Granulosa Cell Tumor of the Testicle – Report of a Neonatal Case with Positive Alpha-fetoprotein Immunohistochemical Staining

“…A clinicopathologic study of 70 cases of JGCT performed by Kao et al described 79% of cases with mixed follicular and solid patterns staining positive for inhibin, calretinin, WT1 and FOXL2. AFP staining was uniformly negative except for one case that showed focal reactivity 2 . Our case would be the second one reporting the same AFP positivity in this type of tumor.…”

Risk factors and treatment outcomes of 239 patients with testicular granulosa cell tumors: a systematic review of published case series data

“…According to the current World Health Organization (WHO) classification of Tumors of the Urinary System and Male Genital Organs, two histologic subtypes are distinguished: The juvenile tGrCT and the less frequent adult tGrCT (Idrees et al 2017 ). While the juvenile subtype accounts for 6% of all prepubertal testicular tumors and represents the most frequent congenital testicular tumor (Kao et al 2015 ), the adult tGrCT is rare and only reported in small case series and case reports. For both histological subtypes, the risk of metastatic spread is ill defined (Cecchetto et al 2010 ; Mostofi et al 1959 ).…”

Juvenile Granulosa Cell Tumor of the Testis: Prenatal Diagnosis and Management

“…1 It is frequently diagnosed in the neonatal period; it is uncommon in older children and exceptional in adults. 2 Typical presentation is a painless neonatal scrotal mass 3 ; occasionally it occurs in cryptorchidic testes 3 4 5 or in infants with abnormal karyotypes and ambiguous genitalia 3 ; all cases reported have had a benign outcome. 3 6 Inguinal orchiectomy was invariably considered the treatment of choice but new treatment trends advocate a trans-scrotal approach 7 and testis-sparing surgery where preoperative staging determines that this is safe.…”

“…2 Typical presentation is a painless neonatal scrotal mass 3 ; occasionally it occurs in cryptorchidic testes 3 4 5 or in infants with abnormal karyotypes and ambiguous genitalia 3 ; all cases reported have had a benign outcome. 3 6 Inguinal orchiectomy was invariably considered the treatment of choice but new treatment trends advocate a trans-scrotal approach 7 and testis-sparing surgery where preoperative staging determines that this is safe. 6 8 We report a case of JGCT of the testis prenatally diagnosed, an event described only twice in the literature so far, 7 9 followed by inguinal orchiectomy.…”