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Sebaceous carcinoma arising within an ovarian mature cystic teratoma: A case report with discussion of clinical management and genetic evaluation

机译:卵巢成熟的囊性畸胎瘤内产生的皮脂腺癌:一例报告,并附有临床治疗和基因评估的讨论

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Sebaceous carcinomas are rare tumors, with the majority of described cases occurring within the eyelid. To date, there are nine documented reports of sebaceous carcinoma arising within a mature cystic teratoma of the ovary. Although the majority of cases originate from idiopathic mutations, there exists a strong association between this rare tumor and hereditary syndromes of DNA mismatch repair deficiency, such as Lynch syndrome and the lesser-known Muir-Torre syndrome. Here we present the case of a 67?year-old woman with a longstanding history of a small left ovarian cyst with sonographic features of an ovarian dermoid. After nine years, the left adnexal mass was noted to have enlarged, and she underwent a laparoscopic bilateral salpingo-oophorectomy. The final pathology was reported as sebaceous carcinoma arising within a mature cystic teratoma. The patient underwent subsequent surgical staging and has been followed for eight months without evidence of disease. This report includes a review of the current literature, as well as a brief discussion of the clinical management of women with sebaceous carcinoma arising within a mature teratoma. Additionally, we comment on the broader, hereditary significance of a diagnosis of sebaceous carcinoma, and use this case to demonstrate the thorough histologic and genetic evaluation that is recommended for patients diagnosed with this rare tumor.
机译:皮脂腺癌是罕见的肿瘤,大多数描述的病例都发生在眼睑内。迄今为止,已有九份文献报道了在成熟的卵巢囊性畸胎瘤内发生皮脂腺癌。尽管大多数病例来自特发性突变,但这种罕见的肿瘤与DNA失配修复缺陷的遗传综合征(例如Lynch综合征和鲜为人知的Muir-Torre综合征)之间存在很强的联系。在这里,我们介绍了一个67岁的妇女的案例,该妇女具有一个小的左卵巢囊肿的悠久历史,并具有卵巢皮样的超声特征。九年后,发现左附件包块增大,她接受了腹腔镜双侧输卵管卵巢切除术。据报道,最终病理是皮脂腺癌发生在成熟的囊性畸胎瘤内。患者接受了随后的手术分期,并已随访八个月而无疾病迹象。该报告包括对当前文献的回顾,以及对在成熟畸胎瘤内发生的皮脂腺癌女性的临床管理的简短讨论。此外,我们对皮脂腺癌的诊断具有更广泛的遗传意义进行了评论,并使用此案例来证明建议对诊断为这种罕见肿瘤的患者进行彻底的组织学和遗传学评估。

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