Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project

Cecchetto, Giovanni; Alaggio, Rita; Bisogno, Gianni; Virgone, Calogero; Dall’Igna, Patrizia; Terenziani, Monica; Boldrini, Renata; Donofrio, Vittoria; Ferrari, Andrea; Bernini, Gabriella

doi:10.1016/j.jpedsurg.2010.02.120

Cited by 41 publications

(55 citation statements)

References 29 publications

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“…The stroma was fibrous or fibromyxoid in an equal number of cases (Figs. [8][9] and occasionally had hemorrhage associated with hemosiderin-laiden macrophages (16%).…”

Section: Pathologic Featuresmentioning

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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“…The stroma was fibrous or fibromyxoid in an equal number of cases (Figs. [8][9] and occasionally had hemorrhage associated with hemosiderin-laiden macrophages (16%).…”

Section: Pathologic Featuresmentioning

confidence: 99%

Juvenile Granulosa Cell Tumors of the Testis

Kao

Cornejo

Ulbright

et al. 2015

American Journal of Surgical Pathology

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“…Em um adolescente acometido, essa neoplasia apresentou comportamento agressivo, com metástases a distância, embora seja descrito em geral como de bom prognóstico e raramente se manifestar por alterações endocrinológicas 22 . Na nossa série, tumor desmoplásico na região paratesticular foi diagnosticado em um paciente.…”

Section: Discussionunclassified

Testicular tumors in children and adolescents

Neto¹,

Petrilli²,

Macedo³

et al. 2012

J Pediatr (Rio J)

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Objective: To perform a comparative assessment of the clinical and epidemiological aspects of testicular tumors in childhood and adolescence.Methods: Retrospective analysis of medical records of patients with testicular or paratesticular neoplasms. Patients under 10 years were classified as children and patients between 10 and 20 were classified as adolescents. The obtained results were compared through the twosample test for proportions: non-parametric Mann-Whitney test and log-rank test.Results: 60 patients were admitted in the period from January 1992 to July 2009: 34 children and 26 adolescents with testicular or paratesticular neoplasms. The main manifestations were testicular tumor and scrotal pain. Pain complaints were more common in adolescents (p = 0.006), who presented a mean time from disease onset to diagnosis of 4.9 months, longer than children, who presented a period of 2.3 months from disease onset to diagnosis (p = 0.01). Histological types were as follows: germ cell tumors in 32/60 (53%), rhabdomyosarcomas (RMSs) in 23/60 (38.3%), and other in 5/60 (8.3%). Adolescents presented a higher incidence of RMSs, lymph node metastases (p = 0.003) and distant metastases (p = 0.035). Differences in survival rates among the studied patients were not statistically significant, the only indicative being that survival in RMS cases is longer for children (p = 0.072). Conclusion:Compared to children, adolescents with testicular tumor presented longer time from disease onset to diagnosis, aggressive histological type and advanced illness at diagnosis, despite the small sample analyzed. J Pediatr (Rio J) ResumoObjetivo: Avaliar comparativamente aspectos clínicos e epidemiológicos dos tumores de testículo na infância e adolescência.Métodos: Análise retrospectiva dos prontuários de pacientes com neoplasias de testículo ou paratesticulares. Foram classificados como crianças os menores de 10 anos e como adolescentes os pacientes entre 10 e 20 anos. Os resultados obtidos foram comparados por meio do teste para duas proporções: teste não paramétrico de Mann-Whitney e teste de log-rank. Resultados:No período de janeiro de 1992 a julho de 2009, foram admitidos 60 pacientes: 34 crianças e 26 adolescentes com neoplasias de testículo ou paratesticulares. As principais manifestações foram tumor e dor na bolsa escrotal. A queixa de dor foi mais comum em adolescentes (p = 0,006). Estes apresentaram tempo médio de história de 4,9 meses, mais prolongado do que crianças, com 2,3 meses (p = 0,01). Os tipos histológicos encontrados foram: tumores de células germinativas em 32/60 (53%), rabdomiossarcomas (RMSs) em 23/60 (38,3%) e outros em 5/60 (8,3%). Os adolescentes apresentaram maior frequência de RMSs, metástases em linfonodos (p = 0,003) e a distância (p = 0,035). As diferenças na sobrevida dos pacientes estudados não foram estatisticamente significantes, havendo apenas indicativo de que a sobrevida, nos casos de RMS, é maior nas crianças (p = 0,072). Conclusões:Os adolescentes com tumor testicular apresentaram maior t...

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“…Juvenil granüloza hücreli tümör (JGHT) 6 aydan kü-çük infantlarda en sık karşılaşılan testis tümörüdür (2,4) . En önemli klinik bulgusu tek taraflı, ağrısız skrotal şişliktir.…”

Section: Discussionunclassified

“…Ultrasonografi ayrıca tümöral lezyonun dışında kalan normal testiküler parankimin değerlendirilmesine de olanak sağ-lar (7) . Gerek klinik özellikleri gerekse ultrasonografik görünümünden dolayı testiküler teratom ve yolk kesesi tümörleri ayırıcı tanıda ilk sırada akılda bulundurulmalıdır (9,2) . Bu durumda HCG ve AFP gibi spesifik tümör belirteçleri yol gösterici olmakla birlikte, AFP değerleri özellikle ilk 6 ay içerisinde teratom tanısı-nın konulabilmesi veya dışlanabilmesi için yetersiz kalmaktadır (3) .…”

Section: Discussionunclassified

“…ilave tedavinin gerekmediği kabul edilmektedir (2,7) . Bununla birlikte, yapılan çalışmalarda AFP değer-leri normal olan, ultrasonografide küçük ve sınırları belirgin olan ve frozen incelmesinde selim olarak raporlanan lezyonlarda ister teratom isterse JGHT olsun testis koruyucu cerrahinin uygulanabileceği bildirilmektedir (15,8,10) .…”

Section: H Taşkınlar Ve Ark Sütçocuklarında Ender Bir Testiküler Tunclassified

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