Congenital Testicular Juvenile Granulosa Cell Tumor in a Neonate with X/XY Mosaicism

Raju, Usha; Fine, Gerald; Warrier, Rajasekharan; Kini, Ratnakar; Weiss, Lester

doi:10.1097/00000478-198608000-00008

Cited by 41 publications

(20 citation statements)

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“…Furthermore, the detection of cytokeratin subtypes 8 and 18 coincides with results on immunoblotting23 and those previously obtained with antibody CAM5p2 against cytokeratin subtypes 8, 18, and 1922 on ovarian granulosa cell tumours. According to our results, the reactivity is not due to cytokeratin subtype 19. The negativity for cytokeratin even after protein digestion of paraffin wax sections differs from the results reported by Benjamin et at22 and may be due to different epitopes which react with the antibodies KLI -and C uAM52 C -,g The main divergence from the results of Czernobilsky et al23 on ovarian granuloma cell tumours is the negativity for desmoplakin in our case, although the same antibody DP 1 & 2-Due, Dieckmann, Niedobitek, Bornhoft, Loy, Stein as paralleled by the variations in cytokeratin expressions on ovarian granulosa cell tumours,22 or whether this represents antigenic differences in testicular and ovarian granulosa cell tumours.…”

Section: Discussionsupporting

confidence: 66%

Testicular sex cord stromal tumour with granulosa cell differentiation: detection of steroid hormone receptors as a possible basis for tumour development and therapeutic management.

Düe

Dieckmann²,

Niedobitek³

et al. 1990

J Clin Pathol

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Section: Discussionsupporting

confidence: 66%

Testicular sex cord stromal tumour with granulosa cell differentiation: detection of steroid hormone receptors as a possible basis for tumour development and therapeutic management.

Düe

Dieckmann²,

Niedobitek³

et al. 1990

J Clin Pathol

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“…In case 1, a small paraovarian cystic tumor proved to be a JGCT. Although rare, this neoplasm has been described in testicles of infants and children with abnormal sexual development [27][28][29] and in some patients with no apparent sexual abnormalities. 12 Histological analysis of the tumors in cases 2-4 revealed typical characteristics of gonadoblastoma.…”

Section: Discussionmentioning

confidence: 99%

Distribution of Y-chromosome-bearing cells in gonadoblastoma and dysgenetic testis in 45,X/46,XY infants

et al. 2005

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Gonadoblastoma is an unusual mixed germ cell-sex cord-stromal tumor that has the potential for malignant transformation and 30% of all patients with gonadoblastoma develop germ cell tumors mainly dysgerminoma/ seminoma. An additional 10% gives rise to other malignant germ cell neoplasms. This tumor affects a subset of patients with intersex disorders. The age at diagnosis is variable ranging from birth to the fourth decade, but around 94% of cases are diagnosed during the first three decades of life and there are few cases with gonadoblastoma diagnosed in infants. In this paper, we present the histological and molecular findings of four patients with gonadal dysgenesis who developed gonadoblastoma in the first 2 years of life and one case with bilateral dysgerminoma diagnosed at 15 years of age. The sex chromosomes of mosaic patients do not distribute homogenously in dysgenetic gonads; however, statistical analysis of FISH results revealed significant differences between the XY cell line in the gonadoblastoma compared with the dysgenetic testis. Our cases demonstrate that tumors could be present at a very early age, so the prophylactic removal of the gonads is advised.

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“…When associated to external genitalia abnormalities, like ambiguous genitalia or hypospadia, JGCT usually presents abnormal karyotypes involving X/XY mosaicism or structural abnormalities of the chromosome Y 12 , 17 - 19. Chromosomal abnormality linked to the Y chromosome was reported to be found in 20% of the cases 18 , 19…”

Section: Discussionmentioning

confidence: 99%

“…Similarly, the method of dosage using Siemens kit accepts several interferences as causing false-positive results mainly in the range of 10–50 mUI/L. Therefore AFP and hCG determinations are considered useless tumor markers for this type of neoplasm 18 , 19. Imaging studies, mainly those obtained by ultrasound examination, show a complex cystic and solid mass.…”

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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Congenital Testicular Juvenile Granulosa Cell Tumor in a Neonate with X/XY Mosaicism

Cited by 41 publications

References 0 publications

Testicular sex cord stromal tumour with granulosa cell differentiation: detection of steroid hormone receptors as a possible basis for tumour development and therapeutic management.

Testicular sex cord stromal tumour with granulosa cell differentiation: detection of steroid hormone receptors as a possible basis for tumour development and therapeutic management.

Distribution of Y-chromosome-bearing cells in gonadoblastoma and dysgenetic testis in 45,X/46,XY infants

Granulosa cell tumor of the testis in a newborn

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