Shabtaie Samuel A, Wang Bo, Owyong Mark, Ruiz-Mesa Catalina, Corrales-Medina Fernando F, Rojas Claudia P, Infante Juan C, Neville Holly L, Perez Eduardo A, Sola Juan E, Hogan Anthony R
Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, FL.
Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Miami Miller School of Medicine, Miami, FL.
J Pediatr Surg. 2016 Jun;51(6):1047-50. doi: 10.1016/j.jpedsurg.2016.03.014. Epub 2016 Apr 9.
Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that usually manifests during early childhood. Typically the lesion presents with skin, soft tissue and bone involvement and is characterized histologically by ill-defined nodularity and the presence of spindle cells with resemblance to Kaposi's sarcoma. We report a rare neonatal case of a splenic kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon that was diagnosed with radiographic imaging. Because of the rapid onset of thrombocytopenia and anemia, the patient required urgent splenectomy with subsequent resolution of the blood dyscrasias.
卡波西样血管内皮瘤是一种罕见的具有局部侵袭性的血管肿瘤，通常在儿童早期出现。典型的病变表现为皮肤、软组织和骨骼受累，组织学特征为边界不清的结节状以及存在类似于卡波西肉瘤的梭形细胞。我们报告了一例罕见的新生儿脾卡波西样血管内皮瘤合并卡萨巴赫-梅里特现象，通过影像学检查得以诊断。由于血小板减少症和贫血迅速出现，该患者需要紧急行脾切除术，随后血液系统异常得以缓解。
