Department of Pediatric Hematology and Oncology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Avenue Hippocrate 10, Brussels, Belgium.
Pediatr Nephrol. 2010 May;25(5):983-6. doi: 10.1007/s00467-009-1393-5. Epub 2009 Dec 9.
Infantile myofibromatosis is a rare disorder characterized by the formation of tumors in the skin, soft tissues, bone, and viscera. We report the case of a 3-week-old girl who presented with severe hypertension due to generalized infantile myofibromatosis including renal involvement. The infant was treated by chemotherapy and showed progressive regression of the tumors. However, her evolution was marked by the development of aneurismal dilations of the renal and iliac arteries as observed in fibromuscular dysplasia. We discuss the possibility of a link between these two mesenchymal disorders.
婴儿肌纤维瘤病是一种罕见的疾病,其特征是皮肤、软组织、骨骼和内脏形成肿瘤。我们报告了一例 3 周大的女婴,因包括肾脏受累在内的全身性婴儿肌纤维瘤病导致严重高血压。该婴儿接受了化疗治疗,并显示出肿瘤的逐渐消退。然而,她的病情发展伴有肾动脉和髂动脉的瘤样扩张,这在纤维肌性发育不良中也有观察到。我们讨论了这两种间充质疾病之间可能存在的联系。