关键词: DICER1 Granulosa cell tumor Sertoli–Leydig cell tumor case report gynandroblastoma sex cord–gonadal stromal tumor

Mesh : Male Female Humans Adolescent Granulosa Cell Tumor / surgery Sex Cord-Gonadal Stromal Tumors / diagnosis surgery Ovarian Neoplasms / diagnosis surgery pathology Biomarkers, Tumor

来  源:   DOI:10.1177/03000605231187796   PDF(Pubmed)

Abstract:
Mixed sex cord-stromal tumors, which consist of poorly differentiated Sertoli cells and Leydig cells and juvenile granulosa cell tumor tissue, are extremely rare. Most of these tumors are unilateral and stage I at the time of diagnosis; nonetheless, according to the available relevant English-language literature, these tumors maintain a malignant potential. We herein report a case involving a 15-year-old girl diagnosed with a mixed sex cord-stromal tumor (gynandroblastoma with juvenile granulosa cell tumor component). Left salpingo-oophorectomy was initially performed, and the diagnosis of a juvenile granulosa cell tumor was established. Right salpingo-oophorectomy was performed 1 year later, at which time the specimen showed a different growth pattern involving epithelioid cells and tubules, resembling a Sertoli-Leydig cell tumor. Immunohistochemical staining was performed and the specimen was compared with that obtained 1 year earlier. We concluded that the tumors were linked and most likely constituted a gynandroblastoma (mixed form of sex cord-stromal tumor). Although this is an extremely uncommon ovarian tumor, it should be considered when diverse tumor morphology is identified. Bilateral metachronous involvement of the ovaries is possible. The grade of the Sertoli-Leydig cell component may influence the prognosis of such a tumor.
摘要:
混合性性索间质肿瘤,由低分化的支持细胞和Leydig细胞和幼年颗粒细胞肿瘤组织组成,极为罕见。这些肿瘤大多数是单侧的,在诊断时是I期;尽管如此,根据现有的相关英语文献,这些肿瘤具有恶性潜能。我们在此报告了一例涉及一名15岁女孩的病例,该女孩被诊断患有混合性性索间质肿瘤(具有幼年颗粒细胞肿瘤成分的妇科母细胞瘤)。最初进行了左输卵管卵巢切除术,并确定了幼年颗粒细胞瘤的诊断。1年后行右侧输卵管卵巢切除术,此时标本显示出涉及上皮样细胞和小管的不同生长模式,类似于Sertoli-Leydig细胞肿瘤。进行免疫组织化学染色,并将样本与1年前获得的样本进行比较。我们得出的结论是,这些肿瘤是相互关联的,最有可能构成妇科母细胞瘤(性索间质肿瘤的混合形式)。虽然这是一种极为罕见的卵巢肿瘤,当确定不同的肿瘤形态时,应该考虑。双侧卵巢异时受累是可能的。Sertoli-Leydig细胞成分的等级可能会影响此类肿瘤的预后。
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