Literature DB >> 23533290

Evaluation of retained testes in adolescent girls and women with complete androgen insensitivity syndrome.

Rola S Nakhal1, Margaret Hall-Craggs, Alex Freeman, Alex Kirkham, Gerard S Conway, Rupali Arora, Christopher R J Woodhouse, Dan N Wood, Sarah M Creighton.   

Abstract

PURPOSE: To evaluate the magnetic resonance (MR) imaging appearance of the testes in women with complete androgen insensitivity syndrome (CAIS), including any benign or malignant changes.
MATERIALS AND METHODS: This was a retrospective review of the testicular MR images and histologic reports from 25 patients with CAIS who chose to retain their testes beyond age 16 years and who were imaged between January 2004 and December 2010. Ethical approval was obtained, and informed consent was obtained from each subject to review the medical records, images, and histologic slides and reports. Imaging and histologic findings were compared.
RESULTS: Twelve patients (mean age, 24 years; age range, 18-39 years) retained their testes and 13 (mean age, 22 years; age range, 17-37 years) eventually underwent gonadectomy. Review of the MR images showed that testicular parenchyma was heterogeneous in 30 of 46 testes (65%). The most common changes on MR images included simple-looking paratesticular cysts (34 of 46 testes, 74%) and low-signal-intensity, well-defined Sertoli cell adenomas (26 of 46 testes, 56%). Correlation of the histologic and MR imaging findings showed that MR imaging could correctly depict the presence or absence of Sertoli cell adenomas in 19 of 23 testes (83%). Paratesticular cysts were correctly detected in 22 of 23 testes (96%). Microscopic examination showed that the testes were composed of atrophic seminiferous tubules, whereas germ cells were found in 13 of 26 testes (50%). All paratesticular cysts were confirmed to be benign; however, a focus of intratubular germ cell neoplasia was found in a Sertoli cell adenoma. Premalignant foci were detected in three patients, two with intratubular germ cell neoplasia and one with sex cord tumor with annular tubules. No invasive cancers were found.
CONCLUSION: MR imaging is accurate in the detection of testicular changes, including paratesticular cysts and Sertoli cell adenomas. Although these changes are usually benign, Sertoli adenomas can sometimes harbor premalignant lesions. MR imaging cannot depict premalignant changes; therefore, the standard of care for patients with CAIS should remain gonadectomy after puberty.

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Year:  2013        PMID: 23533290     DOI: 10.1148/radiol.13121068

Source DB:  PubMed          Journal:  Radiology        ISSN: 0033-8419            Impact factor:   11.105


  15 in total

Review 1.  A Management Protocol for Gonad Preservation in Patients with Androgen Insensitivity Syndrome.

Authors:  Erica M Weidler; Maria E Linnaus; Arlene B Baratz; Luis F Goncalves; Smita Bailey; S Janett Hernandez; Veronica Gomez-Lobo; Kathleen van Leeuwen
Journal:  J Pediatr Adolesc Gynecol       Date:  2019-06-21       Impact factor: 1.814

Review 2.  Gender identity, gender assignment and reassignment in individuals with disorders of sex development: a major of dilemma.

Authors:  A D Fisher; J Ristori; E Fanni; G Castellini; G Forti; M Maggi
Journal:  J Endocrinol Invest       Date:  2016-06-10       Impact factor: 4.256

3.  Presence of Germ Cells in Disorders of Sex Development: Implications for Fertility Potential and Preservation.

Authors:  Courtney Finlayson; Michael K Fritsch; Emilie K Johnson; Ilina Rosoklija; Yasmin Gosiengfiao; Elizabeth Yerkes; Mary Beth Madonna; Teresa K Woodruff; Earl Cheng
Journal:  J Urol       Date:  2016-11-10       Impact factor: 7.450

Review 4.  A practical guide for evaluating gonadal germ cell tumor predisposition in differences of sex development.

Authors:  Louise C Pyle; Katherine L Nathanson
Journal:  Am J Med Genet C Semin Med Genet       Date:  2017-05-25       Impact factor: 3.908

5.  Gender Incongruity in a Person with 46,XY and Complete Androgen Insensitivity Syndrome Raised as a Female.

Authors:  Diego Jesús Del Can Sánchez; Suset Dueñas Disotuar; Ana Piñar Gutiérrez; Miguel Ángel Japón Rodríguez; Isabel Olea Comas; Alejandro Déniz García; Alfonso Soto Moreno; Miguel Ángel Mangas Cruz
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Review 6.  Clinical management in mixed gonadal dysgenesis with chromosomal mosaicism: Considerations in newborns and adolescents.

Authors:  Erica M Weidler; Margaret Pearson; Kathleen van Leeuwen; Erin Garvey
Journal:  Semin Pediatr Surg       Date:  2019-09-19       Impact factor: 2.754

Review 7.  [Progress on evaluation, diagnosis and management of disorders of sex development].

Authors:  Guangjie Chen; Xiaohao Wang; Daxing Tang
Journal:  Zhejiang Da Xue Xue Bao Yi Xue Ban       Date:  2019-06-25

8.  Society for Endocrinology UK guidance on the initial evaluation of an infant or an adolescent with a suspected disorder of sex development (Revised 2015).

Authors:  S Faisal Ahmed; John C Achermann; Wiebke Arlt; Adam Balen; Gerry Conway; Zoe Edwards; Sue Elford; Ieuan A Hughes; Louise Izatt; Nils Krone; Harriet Miles; Stuart O'Toole; Les Perry; Caroline Sanders; Margaret Simmonds; Andrew Watt; Debbie Willis
Journal:  Clin Endocrinol (Oxf)       Date:  2015-08-13       Impact factor: 3.478

9.  Usefulness and role of magnetic resonance imaging in a case of complete androgen insensitivity syndrome.

Authors:  Daniela Grasso; Camela Borreggine; Caterina Campanale; Antonio Longo; Gianpaolo Grilli; Luca Macarini
Journal:  Radiol Case Rep       Date:  2016-02-17

10.  Malignant Germ Cell Tumors and Their Precursor Gonadal Lesions in Patients with XY-DSD: A Case Series and Review of the Literature.

Authors:  Sahra Steinmacher; Sara Y Brucker; Andrina Kölle; Bernhard Krämer; Dorit Schöller; Katharina Rall
Journal:  Int J Environ Res Public Health       Date:  2021-05-25       Impact factor: 3.390

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