A rare case of primary hepatic solitary fibrous tumor associated with pregnancy

Gabriel Acosta-Gonzalez, M. Cho, R. Rogers, F. Mariz, Leon Pachter, A. G. Neto
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Abstract

Purpose: To describe a case of histologically and immunohistochemically confirmed primary hepatic solitary fibrous tumor (SFT) associated with pregnancy. Case Report: A 40-year-old Caucasian woman G3P1021 with history of oral contraceptive use and no other known significant past medical history delivered via C-section in November of 2012. Two months post delivery, she noted that her abdomen did not decrease in size and sought medical attention. As part of the work-up, an abdominal MRI revealed a 15.9 cm mass centered in segment 4b of the liver with extension into segments 5 and 8 within the right lobe. In addition, an exophytic component extending inferiorly from the liver into the right mid abdomen was noted. The patient underwent an uncomplicated hepatic segmentectomy with cholecystectomy. Grossly, the tumor consisted of a firm tan-white well-circumscribed and partially encapsulated mass. Histologically, the tumor was composed of cytologically bland spindle cells with a patternless architecture with hypocellular and hypercellular areas embedded within a collagenous fibrous stroma with occasional dilated branching thin-walled blood vessels. The tumor showed no infiltrative margins or necrosis and a mitotic count of 1/10HPF. Tumor cells were strongly and diffusely positive for CD34, BCL-2, and vimentin; weakly positive for STAT6 (nuclear distribution); and focally positive for CD99 and β-Catenin. In addition, estrogen and progesterone receptors (ER and PR) were also performed and showed positive staining. The diagnosis of SFT was confirmed. To date, 36 months post-resection, our patient has been followed with imaging, showing no evidence of residual or recurrent disease. Conclusions: Primary hepatic SFT is exceedingly rare and even more so in association with pregnancy. Positive immunohistochemical staining of tumor cells for progesterone and estrogen receptors may indicate hormonal stimulation as a driver of neoplastic cell proliferation.
妊娠合并原发性肝孤立性纤维性肿瘤1例
目的:报告一例经组织学和免疫组织化学证实的原发性肝孤立性纤维性肿瘤(SFT)与妊娠相关。病例报告:一名40岁白人女性,G3P1021,有口服避孕药史,无其他已知重大既往病史,于2012年11月剖腹产分娩。分娩两个月后,她注意到她的腹部没有变小,于是去看了医生。作为检查的一部分,腹部MRI显示一个15.9 cm的肿块,以肝脏4b节段为中心,扩展到右叶的5节段和8节段。此外,还发现一种外生成分,从肝脏向下延伸至右中腹部。患者接受了简单的肝段切除术和胆囊切除术。肉眼可见,肿瘤呈坚固的棕白色,边界清楚,部分包被。组织学上,肿瘤由细胞学上平淡的梭形细胞组成,结构无模式,胶原纤维间质内嵌有低细胞区和高细胞区,偶尔有扩张的分支薄壁血管。肿瘤无浸润边缘或坏死,有丝分裂计数为1/10HPF。肿瘤细胞CD34、BCL-2和vimentin呈强烈和弥漫性阳性;STAT6弱阳性(核分布);CD99和β-Catenin局部阳性。此外,雌激素和孕激素受体(ER和PR)也进行了检测,均呈阳性染色。确诊为SFT。到目前为止,切除后36个月,我们的患者进行了影像学随访,没有显示残留或复发疾病的证据。结论:原发性肝脏SFT极为罕见,与妊娠相关的情况更为罕见。肿瘤细胞孕激素和雌激素受体免疫组化染色阳性可能表明激素刺激是肿瘤细胞增殖的驱动因素。
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