Illescas Molina Tamara, Montalvo Montes Joaquín, Contreras Cecilia Esther, Muñoz Muñiz María del Mar, González González Antonio, Herraiz Martínez Miguel Angel
Servicio de Ginecología y Obstetricia, Hospital Clínico San Carlos, Madrid, España.
Ginecol Obstet Mex. 2009 Aug;77(8):380-6.
Renal angiomyolipomas are uncommon tumours during pregnancy, but it's important to consider them in the differential diagnosis, for the increased risk of complications such as bleeding, specially during pregnancy. Their characteristics are different depending on if the tumour is associated or not with facomatosis. To show both possibilities, we describe two cases of pregnant women with angiomyolipomas: the first one presented with bleeding of the tumour (Wünderlich's syndrome), but surveillance without treatment was possible until 37th week of gestation, and embolization of the tumour was performed afterwards. The second patient had multiple well known angiomyolipomas, associated to tuberous sclerosis; even though she had required two previous embolizations, she had an uneventful pregnancy, without any episodes of bleeding. Both pregnancies ended successfully by means of a cesarean section. Since there are few papers available in the literature, there is not an agreement about therapeutic management when pregnant women present with bleeding angiomyolipomas (Wünderlich's syndrome). Treatment options include partial or total nephrectomy (with cesarean section simultaneously in patients at 28 weeks of gestation or later), transcateter selective arterial embolization (which can also be preventive), and sometimes, conservative management. If this last option is possible, there's still discussion about the risk of vaginal delivery compared with that of cesarean section, in terms of bleeding of the tumour.
肾血管平滑肌脂肪瘤在孕期并不常见,但在鉴别诊断时考虑到它们很重要,因为其存在出血等并发症风险增加,尤其是在孕期。根据肿瘤是否与错构瘤病相关,其特征有所不同。为展示这两种可能性,我们描述了两例患有血管平滑肌脂肪瘤的孕妇病例:第一例出现肿瘤出血(肾出血性梗死综合征),但在妊娠37周前可行观察等待而不进行治疗,之后对肿瘤进行了栓塞治疗。第二例患者有多个已知的血管平滑肌脂肪瘤,与结节性硬化症相关;尽管她之前已接受过两次栓塞治疗,但孕期顺利,未发生任何出血事件。两次妊娠均通过剖宫产成功结束。由于文献中可用的论文较少,对于患有出血性血管平滑肌脂肪瘤(肾出血性梗死综合征)的孕妇,在治疗管理方面尚无共识。治疗选择包括部分或全肾切除术(妊娠28周及以后的患者同时行剖宫产)、经导管选择性动脉栓塞(也可作为预防性措施),有时也采用保守治疗。如果可以选择最后一种方案,在肿瘤出血方面,关于阴道分娩与剖宫产的风险仍存在争议。