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[Plurifocal testicular hamartomas and testicular feminization syndrome].
Ann Pathol. 2000 Dec; 20(6):605-8.AP

Abstract

We report the case of a 14-year-old girl with a testicular feminization syndrome. The inguinal cryptorchid testis contained plurifocal hamartomas ranging from 0.5 to 1 cm. They were composed of tubules lined by cylindrical Sertoli cells immunoreactive for alpha-inhibin and p30/32(MIC2). The stroma contained few Leydig cells. Ultrastructural study showed tubules with immature Sertoli cells. The testicular feminization syndrome is caused by mutations of the androgen receptor gene. Patients with male genotype 46, XY have a female morphotype with external sexual organs without ambiguity. They have neither uterus nor ovary but two cryptorchid testis in which sex-cord stromal tumors can develop. Their malignant transformation is rare but requires preventive bilateral orchidectomy.

Authors+Show Affiliations

Service d'anatomie pathologique, CHU Nord, Amiens.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

11148355

Citation

Chatelain, D, et al. "[Plurifocal Testicular Hamartomas and Testicular Feminization Syndrome]." Annales De Pathologie, vol. 20, no. 6, 2000, pp. 605-8.
Chatelain D, Ricard J, Ghighi C, et al. [Plurifocal testicular hamartomas and testicular feminization syndrome]. Ann Pathol. 2000;20(6):605-8.
Chatelain, D., Ricard, J., Ghighi, C., Colombat, M., Leclercq, F., Cordonnier, C., Pouzac, M., Sevestre, H., & Gontier, M. F. (2000). [Plurifocal testicular hamartomas and testicular feminization syndrome]. Annales De Pathologie, 20(6), 605-8.
Chatelain D, et al. [Plurifocal Testicular Hamartomas and Testicular Feminization Syndrome]. Ann Pathol. 2000;20(6):605-8. PubMed PMID: 11148355.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Plurifocal testicular hamartomas and testicular feminization syndrome]. AU - Chatelain,D, AU - Ricard,J, AU - Ghighi,C, AU - Colombat,M, AU - Leclercq,F, AU - Cordonnier,C, AU - Pouzac,M, AU - Sevestre,H, AU - Gontier,M F, PY - 2001/1/10/pubmed PY - 2001/3/3/medline PY - 2001/1/10/entrez SP - 605 EP - 8 JF - Annales de pathologie JO - Ann Pathol VL - 20 IS - 6 N2 - We report the case of a 14-year-old girl with a testicular feminization syndrome. The inguinal cryptorchid testis contained plurifocal hamartomas ranging from 0.5 to 1 cm. They were composed of tubules lined by cylindrical Sertoli cells immunoreactive for alpha-inhibin and p30/32(MIC2). The stroma contained few Leydig cells. Ultrastructural study showed tubules with immature Sertoli cells. The testicular feminization syndrome is caused by mutations of the androgen receptor gene. Patients with male genotype 46, XY have a female morphotype with external sexual organs without ambiguity. They have neither uterus nor ovary but two cryptorchid testis in which sex-cord stromal tumors can develop. Their malignant transformation is rare but requires preventive bilateral orchidectomy. SN - 0242-6498 UR - https://www.unboundmedicine.com/medline/citation/11148355/[Plurifocal_testicular_hamartomas_and_testicular_feminization_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/7031 DB - PRIME DP - Unbound Medicine ER -