Department of Pathology, Habib Bourguiba University Hospital, Sfax, Tunisia.
Department of Pediatric Surgery, Hedi Chaker University Hospital, Sfax, Tunisia.
Pediatr Dev Pathol. 2021 Mar-Apr;24(2):137-141. doi: 10.1177/1093526620980614. Epub 2021 Jan 12.
Ovarian adrenal rest tumors (OART) are tumors that develop in females with congenital adrenal hyperplasia (CAH). In contrast to their counterpart in testicles, they are exceptional and few cases have been reported in the literature. In this report, we present clinicopathological findings of a female patient with CAH due to 21-hydroxylase deficiency who was incidentally diagnosed with OART with a review of the literature. The 14-year-old patient, who was raised as a boy, developed a virilizing syndrome with high testosterone levels that were attributed to non adherence to her replacement corticosteroid therapy. She consulted for sex reassignment surgery. Pelvic ultrasound was normal. She underwent hysterectomy and bilateral adnexectomy. No abnormalities were noticed during the operation. Grossly, both ovaries were variegated with well circumscribed and lobulated, brownish-yellow nodules. Histologically, the nodules were composed of nests of large polygonal cells with centrally located nuclei and prominent nucleoli. There was mild atypia and no crystals of Reinke. Thus, the findings of the histopathological examination were consistent with bilateral OART. Histological differential diagnosis of OART can be challenging particularly with leydig cell tumor, stromal luteoma and steroid cell tumors, not otherwise specified. OART must be considered in women with CAH and persistent virilizing symptoms despite negative imaging results.
卵巢肾上腺残株瘤（OART）是一种在先天性肾上腺皮质增生症（CAH）女性中发生的肿瘤。与睾丸中的对应物不同，它们是罕见的，文献中报道的病例很少。在本报告中，我们介绍了一名因 21-羟化酶缺乏导致 CAH 的女性患者的临床病理发现，该患者偶然被诊断为 OART，并对文献进行了回顾。这名 14 岁的患者被当作男孩抚养长大，出现了男性化综合征，伴有高睾酮水平，这归因于她未遵医嘱服用替代皮质激素治疗。她来咨询进行性别重置手术。盆腔超声检查正常。她接受了子宫切除术和双侧附件切除术。手术过程中未发现异常。大体上，双侧卵巢呈斑驳状，有界限清楚的分叶状、棕黄色小结节。组织学上，这些结节由大的多边形细胞巢组成，中央有核，核仁明显。有轻度异型性，没有 Reinke 晶体。因此，组织病理学检查结果与双侧 OART 一致。OART 的组织学鉴别诊断具有挑战性，特别是与 Leydig 细胞瘤、间质黄体瘤和类固醇细胞瘤，非特指型。在 CAH 女性中，尽管影像学检查结果为阴性，但仍存在持续性男性化症状时，必须考虑 OART 的可能性。
