Negussie Dereje
Department of Gynecology, Jimma University, Jimma.
Ethiop Med J. 2007 Jul;45(3):307-12.
This report refers to a rare case of complete androgen insensitivity syndrome that had presented at the age of 35 years with complaint of abdominal mass, primary amenorrhea and infertility to Jimma University Hospital. A well-developed breast with absence of axillary and pubic hair was seen on examination. There was also an abdominal mass arising from the pelvis occupying the hypogastric and right iliac region. Additionally, there was a reducible mass of 5 by 4 c.m at the left inguinal region. She had a normal female external genitalia with blindly ending vagina of about 4 c.m long. Laporotomy was done and a grayish white solid mass of 20 by 10 c.m. was found and removed. The reducible mass in the left inguinal canal was also removed surgically which was followed by herinorraphy. There were no ovaries or any other internal female genital organs identifiable. Histopathology of the tumor showed features of seminoma while the mass in the inguinal canal was consistent with testis. Literature review on diagnosis, differential diagnosis and management of androgen insensitivity syndrome was made and discussed. This is the first case report of androgen insensitivity syndrome (AIS) in an Ethiopian patient.
本报告讲述了一例罕见的完全性雄激素不敏感综合征病例,该患者35岁时因腹部肿块、原发性闭经和不孕前往吉姆马大学医院就诊。检查发现乳房发育良好,但腋窝和耻骨区无毛发。盆腔出现腹部肿块,占据下腹和右髂区。此外,左腹股沟区有一个大小为5×4厘米、可回纳的肿块。患者女性外生殖器正常,阴道盲端约4厘米长。进行了剖腹探查,发现并切除了一个大小为20×10厘米的灰白色实性肿块。左腹股沟管内可回纳的肿块也通过手术切除,随后进行了疝修补术。未发现卵巢或任何其他女性内生殖器。肿瘤的组织病理学显示为精原细胞瘤特征,而腹股沟管内的肿块与睾丸一致。对雄激素不敏感综合征的诊断、鉴别诊断和治疗进行了文献综述并展开讨论。这是埃塞俄比亚患者中首例雄激素不敏感综合征(AIS)的病例报告。