Leunen Karin, Amant Frederic, Debiec-Rychter Maria, Croes Romaric, Hagemeijer Anne, Schoenmakers Eric F P M, Vergote Ignace
Division of Gynecological Oncology, Department of Obstetrics & Gynecology, University Hospitals Leuven, Belgium.
Gynecol Oncol. 2003 Oct;91(1):265-71. doi: 10.1016/s0090-8258(03)00477-3.
Although the clinical picture of endometrial stromal sarcoma (ESS) is variable, it was never reported to present as a postpartum hemorrhage. In addition, ESS is a tumor type of which, due to its rarity, little is known regarding chemosensitivity and genetic changes.
A 28-year-old woman complaining of persistent postpartum bleeding was referred to our hospital, where she was diagnosed with ESS. At laparotomy, the invasion of nervous and vascular pelvic structures rendered her inoperable, and chemotherapy (doxorubicin 50 mg/m(2) for 15 min; ifosfamide 5 g/m(2)/24 h; mesna 5 g/m(2), every 3 weeks) was initiated. The ESS appeared to be chemosensitive because after three treatment cycles the tumor iliac metastase significantly decreased in volume and became surgically removable. Chemosensitivity was confirmed microscopically. Three additional courses of chemotherapy and pelvic irradiation were administered. Cytogenetic evaluation of both the primary as well as the metastatic lesions revealed a t(10;17)(q22;p13) as the sole cytogenetic abnormality.
Three interesting features of this particular case put ESS in a new perspective. First, the fundal ESS permitted normal conception and pregnancy but caused a postpartum haemorrhage. Second, the ESS was clearly chemosensitive. Third, we report a novel cytogenetic aberration in ESS, the molecular characterization of which might lead to the identification of the deregulated pathway(s) triggering tumor development in ESS.
尽管子宫内膜间质肉瘤（ESS）的临床表现多样，但从未有过产后出血的报道。此外，ESS是一种罕见的肿瘤类型，关于其化疗敏感性和基因变化知之甚少。
一名28岁的女性因产后持续出血被转诊至我院，在我院被诊断为ESS。剖腹探查时，盆腔神经和血管结构受侵使其无法手术，遂开始化疗（多柔比星50mg/m²，持续15分钟；异环磷酰胺5g/m²/24小时；美司钠5g/m²，每3周一次）。ESS似乎对化疗敏感，因为三个治疗周期后，肿瘤髂骨转移灶体积明显减小，变得可以手术切除。化疗敏感性经显微镜证实。又进行了三个疗程的化疗和盆腔放疗。对原发灶和转移灶的细胞遗传学评估显示，唯一的细胞遗传学异常为t(10;17)(q22;p13)。
该特殊病例的三个有趣特征使ESS有了新的视角。第一，子宫底部的ESS允许正常受孕和妊娠，但导致了产后出血。第二，ESS对化疗明显敏感。第三，我们报告了ESS中一种新的细胞遗传学畸变，对其进行分子特征分析可能会导致识别出触发ESS肿瘤发生的失调通路。
