Pathology Department, University Hospital of Saint-Etienne, France.
Gynecology and Obstetrics Department, University Hospital of Saint-Etienne, France.
Pathol Res Pract. 2020 Dec;216(12):153285. doi: 10.1016/j.prp.2020.153285. Epub 2020 Nov 9.
Overgrowth syndromes are characterized by a global or regional excess growth of various tissue types, especially of mesenchymal nature. The CLOVES (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal naevi, Scoliosis/Skeletal and spinal anomalies) syndrome is characterized by an asymmetric growth excess associated with PIK3CA mutations, found in mosaic, affecting the lesional, but not the normal tissues. Herein, we report the case of a patient affected by CLOVES syndrome, harboring a 13 cm leiomyoma of the uterine broad ligament. The leiomyoma tissue was examined by next-generation sequencing showing the absence of related mutations.
过度生长综合征的特征是各种组织类型的全身性或区域性过度生长,特别是间充质来源的组织。CLOVES(先天性脂肪性不对称性躯干过度生长伴淋巴管畸形、毛细血管畸形、静脉畸形和混合型血管畸形、表皮痣、脊柱侧凸/骨骼和脊柱畸形)综合征的特征是与 PIK3CA 突变相关的不对称生长过度,该突变呈镶嵌型,仅影响病变组织,而不影响正常组织。在此,我们报告了一例患有 CLOVES 综合征的患者,其阔韧带内有 13 厘米的平滑肌瘤。对平滑肌瘤组织进行下一代测序检查显示无相关突变。