Juvenile Granulosa Cell (Sex Cord-Stromal) Tumor of Fetal Testis

Crump, W. Donald

doi:10.1016/s0022-5347(17)52540-9

Cited by 32 publications

(13 citation statements)

References 6 publications

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“…[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.…”

Section: Testicular Juvenile Granulosa Cell Tumormentioning

confidence: 99%

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

2005

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Gonadal sex cord-stromal tumors contain some of the most morphologically interesting neoplasms of the gonads and these lead to many important issues in differential diagnosis. The pathology of these tumors is reviewed with emphasis on new information, similarities and differences in the two gonads, and diagnostic problems. Sertoli cell tumors occur in both gonads being more common in the testis where they usually exhibit a lobular pattern of hollow or solid tubules. In the ovary, tubular differentiation is usually the predominant feature but the lobulation typically seen in the testis is generally not as striking. One variant of Sertoli cell tumor, the large cell calcifying form, appears to be restricted to the male gonad and in contrast to other sex cord tumors is much more frequently bilateral and is associated in many cases with unusual clinical manifestations. In both sexes, patients with Peutz-Jeghers syndrome often have distinctive gonadal pathology. In females, it is in the form of the sex cord with annular tubules whereas in males, the lesion has features that are often intermediate between those of a sex cord tumor with annular tubules and a large cell calcifying Sertoli cell tumor. Sertoli-Leydig cell tumors are more morphologically diverse than pure Sertoli cell tumors and for practical purposes are an issue only in ovarian pathology being exceptionally rare in the testis. The classification proposed by Meyer into well, intermediate, and poor differentiation, remains important prognostically. More recently, heterologous and retiform differentiation has been described. Heterologous tumors most often contain mucinous epithelium, sometimes with small foci of carcinoid or less commonly, and generally in poorly differentiated neoplasms, rhabdomyosarcoma or fetal-type cartilage. Such tumors should be distinguished from pure sarcomas and teratomas. The retiform neoplasms, which tend to occur in young females, may mimic serous borderline tumors or even serous carcinomas. Granulosa cell tumors are much more common in females and in both gonads are divided into adult and juvenile forms. In females, granulosa cell tumors and other sex cord tumors may have markedly bizarre nuclei potentially leading to overdiagnosis as more malignant neoplasms. The juvenile granulosa cell tumor of the testis tends to occur in the first 6 months of life and should be carefully distinguished from the yolk sac tumor of the testis, which usually occurs in a slightly older age group. Occasional sex cord-stromal tumors cannot be readily categorized into the Sertoli or granulosa families and are diagnosed as sex cord-stromal tumors unclassified. In females, this is a relatively common placement for a neoplasm in a pregnant patient. Unclassified tumors are overall more common in males and may entrap residual normal germ cells potentially leading to the erroneous placement of the tumor in the category of a mixed germ cell sex cord-stromal tumor. From the practical viewpoint, the most helpful immunohistochemical findings are the negative s...

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Section: Testicular Juvenile Granulosa Cell Tumormentioning

confidence: 99%

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

2005

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“…Logically he referred to the testicular tumors by the same name. Soon afterwards the first example of juvenile granulosa cell tumor of the testis was reported in the literature, 2 Dr. Scully's experience with 14 cases was described two years later. 3 Over the years it has become apparent that criteria for the diagnosis of this neoplasm in the testis are not applied uniformly by individual investigators and the spectrum acceptable for this designation has been debated.…”

Section: Introductionmentioning

confidence: 99%

Juvenile Granulosa Cell Tumors of the Testis

Kao

Cornejo

Ulbright

et al. 2015

American Journal of Surgical Pathology

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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...

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“…In 1983, Crump8 first described a case of a SCST in a 30-week-old fetus, but juvenile granulosa cell tumor (JGCT) was recognized as a distinct entity in 1985 due to its similarity to the ovarian granulosa cell tumor 9 , 10. The juvenile type accounts for 1.2% of all prepubertal testis tumors, representing the most common stromal sex cord neoplasm of the testis in infancy, and the most common neoplasm of the testis in the first 6 months of life 11 - 13.…”

Section: Discussionmentioning

confidence: 99%

Granulosa cell tumor of the testis in a newborn

Claros¹,

Sakai²,

Consolmagno³

et al. 2014

ACR

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Testicular neoplasms are uncommon tumors of childhood. These tumors comprise the germ cell tumors, and other tumors that may originate from histological testicular components, which are unrelated to the germinal lineage. Among the latter are the sex cord-stromal tumors (SCST), an important entity in newborns. SCSTs comprise, among others, granulosa cell tumors, which are more common in the ovary, but in rare cases may develop in the testis. The prognosis is excellent since it is universally benign. Diagnosis, which is sometimes challenging, is usually made after orchiectomy and pathological examination, which is characterized by morphological features and positive expression of inhibin, calretinin, and vimentin, and negative for alpha-fetoprotein. The authors present the case of a newborn with a right enlarged testis detected during the first examination after birth. Ultrasonography showed a heterogeneous solid/cystic mass in the right testis, without retroperitoneal lymphadenopathy. A right inguinal orchiectomy was performed 21 hours after birth. Pathologic examination revealed a juvenile granulosa cell tumor of the right testicle. After 4 years of follow-up, as expected, the child presented an uneventful outcome.

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Juvenile Granulosa Cell (Sex Cord-Stromal) Tumor of Fetal Testis

Abstract: A case is reported of a juvenile variant of the rare, testicular granulosa cell (sex cord-stromal) tumor in a fetus. The variant has been reported in the ovary but not in the testis. Sex cord-stromal tumors in general have not been reported in fetal testes.

Cited by 32 publications

References 6 publications

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

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

Juvenile Granulosa Cell Tumors of the Testis

Granulosa cell tumor of the testis in a newborn

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