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KMID : 1103720170760030229
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Journal of the Korean Society of Radiology
2017 Volume.76 No. 3 p.229 ~ p.232
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Complete Androgen Insensitivity Syndrome with Paratesticular Leiomyoma: A Case Report
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Lee Ji-Hoon
Oh Hyung-Woo
Lee Mi-Ja
Lim Dong-Hoon
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Abstract
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Complete androgen insensitivity syndrome (AIS) is a rare, X-linked recessive disorder. Patients with AIS may develop primary amenorrhea due to androgen receptor resistance, resulting in a normal female phenotype and male (XY) karyotype. We report a case of a 30-year-old woman who was diagnosed with complete AIS. Ultrasonography and magnetic resonance imaging revealed bilateral inguinal cryptorchidism and no ovaries and uterus. After gonadectomy, the inguinal mass was confirmed as testicular atrophy with hamartomatous proliferation of Leydig cells and paratesticular leiomyoma. Although these tumors have been reported in association with AIS, this is the first case of paratesticular leiomyoma with hamartomatous proliferation of Leydig cells in atrophic testes being reported in Korea.
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KEYWORD
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Complete Androgen Insensitivity Syndrome, Paratesticular Leiomyoma, Cryptorchidism, Magnetic Resonance Imaging
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