Testicular juvenile granulosa cell and Sertoli cell tumours: A clinicopathological study of 29 cases from the Kiel Paediatric Tumour Registry

Abstract

Testicular Sertoli cell tumours (SCT) and juvenile granulosa cell tumours (JGCT) are rare in childhood. This study was designed to investigate the clinical picture, morphology and disease course in a comparatively large series of cases (total number = 29). Of 198 cases of childhood testicular tumour documented in the Kiel Paediatric Tumour Registry 18 were cases of infantile SCT (9.1%) and 11 of JGCT (5.6%). The average age at the time of diagnosis was 4.2 months for infantile SCT and 0.4 months for IGCT. SCT and JGCT often showed infiltrative growth into adjacent testicular tissue, dense cellularity and considerable proliferation activity. Immunohistochemically all cases expressed vimentin intermediate filaments in both tumour types. Next in frequency of expression were cytokeratins (SCT: 7/16; JGCT: 7/10) and smooth-muscle actin (SCT: 9/15; JGCT: 4/10). Follow-up studies (24/29) showed that in cases of tumour manifestation in infancy and after complete tumour removal (usually orchiectomy) no local recurrences and no metastases occurred. The most important conclusion for diagnosis and therapy is that despite infiltrative growth, incomplete differentiation, dense cellularity and considerable proliferation activity, after surgical excision infantile SCT and JGCT have a good prognosis. Adjuvant chemotherapy or more extensive operations with lymphadenectomy are thus not indicated.

Juvenile Granulosa Cell Tumors of the Testis

“…31 However, some might interpret the corded patterns seen in some of our JGCTs as belonging in the Sertoli cell group, which may explain why some have reported "Sertoli cell tumors" as more common than JGCTs in the testis of children. 7 The follicles observed in JGCTs are typically larger than the lumens of tubules in Sertoli cell tumors; the former is also commonly multilayered in contrast to the single cell lining of the latter. In our opinion, an immature nuclear appearance and brisk mitotic rate are much more typical of JGCT than Sertoli cell tumor as is the lobular arrangement of tumor cells and myxoid stroma.…”

“…[5][6][7][8][9][10][11][12] We have encountered patterns and cytologic features that have not been described in the literature or only to a minimal degree, which may potentially cause confusion with other neoplasms, most often yolk sac tumor (YST) or, rarely, other sex cord stromal tumors. 13,14 We therefore undertook a comprehensive morphological analysis of testicular JGCT to highlight not only the classic features but also unusual aspects and those useful in differentiating it from other neoplasms.…”

Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems

“…[6][7][8][9] Low-power examination of the former often shows a striking nodular pattern, with the nodules separated by acellular, often vascular fibrous stroma, the vessels sometimes being dilated (Figure 2). This results in a relatively distinctive profile on low-power examination and on the basis of personal experience probably accounts for the morphology of approximately 50% of the low-grade neoplasms.…”

“…[44][45][46][47][48][49] The better known yolk sac tumor of the testis of young boys peaks after 6 months of age. 8 The tumors are uncommon in older children and are exceptionally rare in adults. Occasional juvenile granulosa cell tumors have occurred in undescended testes of infants with intersexual disorders.…”

“…In one large series of testicular tumors in children, these tumors were much more often cystic than Sertoli cell tumors. 8 Microscopic examination reveals follicular or solid patterns or both (Figure 33). The follicles vary from large and round to oval to small and irregular.…”