Yolk Sac Tumor of the Testis in Infants and Children

Abstract

We report 33 pure yolk sac tumors of the testis from boys 5 to 71 months of age (mean 20.7 mo) diagnosed from 1918 to 2014. All except 1 underwent orchiectomy, with lymph node dissections (all negative) performed in 18; 21 also received chemotherapy and 12 radiotherapy. The tumors were 1.6 to 7.0 cm (mean 3.7 cm) and were nonencapsulated, with a gray to yellow, often mucoid, cut surface. The commonest pattern was reticular-microcystic, but macrocystic, papillary, endodermal sinus (Schiller-Duval bodies), labyrinthine, myxomatous, glandular, and solid patterns were also observed. Follow-up was available for 32 patients (mean 100.5 mo; range, 3 to 456 mo). Twenty-four patients (including 4 who did not receive adjuvant therapy) were without evidence of disease, 8 had metastatic disease; 5 of the latter died of tumor and 1 of treatment complications. Two patients with metastasis were cured with radiation with or without chemotherapy. Two or more of the following were associated with a poor outcome in patients presenting with stage I cases: tumor size >4.5 cm (4/6 tumors [67%]), invasion of rete testis and/or epididymis (3/7 tumors [43%]), and necrosis (6/17 tumors [35%]). In the nonmetastasizing group, 2 or more unfavorable features occurred in only 3/24 tumors (13%) (P=0.0001). It is crucial that this tumor be distinguished from the juvenile granulosa cell tumor, which occurs at a slightly younger age and has distinctive features, although there may be some morphologic overlap. The survival of young boys with testicular yolk sac tumor is very good because of both effective chemotherapy and likely, the inherent characteristics of the tumor in this age group.

Juvenile Granulosa Cell Tumors of the Testis

“…4 The differential diagnosis of the JGCT may include a number of entities in the sex cordstromal category as well as other categories. As the best known testicular tumor of the young is the YST, 14 we consider it first, and then discuss the differential with other sex cord-stromal tumors, and then, briefly, other entities less often an issue.…”

“…14,25,26 In the largest series of testicular YSTs, only 9% were less than 6 months old, and another 15% 6 months or older but less than 1 year old. 14 One pitfall is that the typical serum AFP elevation seen in cases of YST is occasionally seen in patients with JGCT due to the physiologic elevation seen in the early months of life; however, the levels are almost invariably lower than the pathologic levels associated with YST.…”

“…14,25,26 In the largest series of testicular YSTs, only 9% were less than 6 months old, and another 15% 6 months or older but less than 1 year old. 14 One pitfall is that the typical serum AFP elevation seen in cases of YST is occasionally seen in patients with JGCT due to the physiologic elevation seen in the early months of life; however, the levels are almost invariably lower than the pathologic levels associated with YST. 25,27 Grossly, JGCTs more often exhibit a solid and cystic appearance, Although greater difficulty in differential diagnosis of the JGCT occurs with other sex cord tumors, particularly Sertoli cell tumor, it is worth briefly pointing out at this juncture the important differences from the adult type of testicular granulosa cell tumor.…”

“…[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.…”

Perspectives on testicular germ cell neoplasms

“…Prepubertal type yolk sac tumor has a more favorable prognosis and can be treated less aggressively than the adult type [65].…”