Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Am J Surg Pathol. 2011 Oct;35(10):1592-9. doi: 10.1097/PAS.0b013e31822a24a6.
A woman with Carney complex presented at the age of 22 years with abdominal pain and hirsutism. As a baby, she had undergone excision of a right eyelid lesion, and at age 14 years she had undergone removal of a left lower eyelid nodule that subsequently recurred. Investigation revealed an elevated level of serum testosterone and a 2-cm left ovarian tumor. A left salpingo-oophorectomy was performed. Postoperatively, she experienced relief from abdominal pain, the serum level of testosterone normalized, and the hirsutism ameliorated. The tumor featured sheets of eosinophilic cells with lipochrome pigment, myeloid metaplasia, stromal metaplasia, and markedly abnormal blood vessels. Immunocytochemically, the tumor cells were positive for vimentin, synaptophysin, inhibin-A, and calrenin. Because of the clinical setting in which the neoplasm occurred, it is likely that is occurrence was related to Carney complex.
一位患有卡尼综合征的女性,22 岁时因腹痛和多毛症就诊。她还是婴儿时,右眼上睑病变已被切除,14 岁时左眼下眼睑结节已被切除,但后来复发。检查发现血清睾酮水平升高,左侧卵巢有 2 厘米大的肿瘤。行左侧输卵管卵巢切除术。术后,她的腹痛缓解,血清睾酮水平正常,多毛症改善。肿瘤表现为嗜酸性细胞片伴有脂褐素色素、骨髓化生、间质化生和明显异常的血管。免疫细胞化学显示肿瘤细胞阳性表达波形蛋白、突触素、抑制素-A 和钙网蛋白。由于肿瘤发生的临床背景,它很可能与卡尼综合征有关。