我们先前描述了一系列病例,这些病例以一组不同的原发性卵巢胎盘部位滋养细胞肿瘤(PSTT)和上皮样滋养细胞肿瘤(ETT)为非妊娠组,与生殖细胞类型/起源一致。在这里,我们报告了一个新的卵巢非妊娠性PSTT病例。患者是一名13岁的年轻女性,因左肺自发性气胸入院。肺楔形切除标本的病理显示为转移性PSTT,卵巢活检显示非典型中间滋养细胞增殖,在随后的输卵管卵巢切除术标本中发现为PSTT。在卵巢里,肿瘤由单个分散的或小簇的主要是单个核细胞和罕见的多核细胞组成,广泛浸润卵巢实质,输卵管粘膜,和卵巢旁/输卵管旁软组织。存在成熟的囊性畸胎瘤的次要成分(小于总肿瘤体积的5%)。免疫组织化学,主要肿瘤的肿瘤细胞对hPL有弥漫性免疫反应,Gata3和AE1/AE3,只有罕见的hCG阳性或p63阳性细胞。形态学和免疫组织化学结果支持PSTT。分子基因分型显示正常肺组织和转移性PSTT之间的基因型相同,表明其生殖细胞类型/起源的非妊娠性质。该病例代表了这种具有远处(肺)转移的肿瘤的首例病例。该病例还提供了进一步的证据来支持我们的建议,即生殖细胞类型/起源的原发性卵巢非妊娠中间滋养细胞肿瘤,包括PSTT和ETT,应该在分类系统中得到正式承认。
We previously described a series of cases which characterize a distinct group of primary ovarian placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) as a non-gestational set consistent with germ cell type/origin. Here we report a new case of ovarian non-gestational PSTT. The patient was a 13 year-old young female admitted for a spontaneous pneumothorax of the left lung. The pathology of lung wedge excision specimen demonstrated metastatic PSTT and ovarian biopsy showed atypical intermediate trophoblastic proliferation which was found to be PSTT in the subsequent salpingo-oophorectomy specimen. In the ovary, the tumor was composed of singly dispersed or small clusters of predominantly mononuclear cells and rare multinucleated cells extensively infiltrating the ovarian parenchyma, tubal mucosa, and paraovarian/paratubal soft tissue. A minor component of mature cystic teratoma (less than 5% of total tumor volume) was present. Immunohistochemically, the neoplastic cells of main tumor were diffusely immunoreactive for hPL, Gata3 and AE1/AE3, and had only rare hCG-positive or p63-positive cells. The morphology and immunohistochemical results support a PSTT. Molecular genotyping revealed an identical genotype pattern between the normal lung tissue and the metastatic PSTT, indicating its non-gestational nature of germ cell type/origin. This case represents the first case of such tumor with distant (lung) metastasis. This case also provides further evidence to support our recommendation that primary ovarian non-gestational intermediate trophoblastic tumors of germ cell type/origin, including PSTT and ETT, should be formally recognized in classification systems.