Pathology, Chacha Nehru Children's Hospital, New Delhi, India.
Paediatric Surgery, Chacha Nehru Children's Hospital, New Delhi, India.
BMJ Case Rep. 2021 Feb 19;14(2):e239527. doi: 10.1136/bcr-2020-239527.
Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.
卡波西样血管内皮细胞瘤是一种边界性的内皮肿瘤，主要发生在儿童时期，累及四肢。它常与消耗性凝血病状态、卡-梅现象（KMP）密切相关。皮肤外受累并不常见。肠道受累也很少见，可能表现为急性腹痛。一个 24 天大的新生儿因胆汁性呕吐和发热 5 天就诊。切除的坏疽性十二指肠的切片包含一个黏膜下肿瘤，由浸润性小结节组成，呈裂隙状或新月形，CD34 阳性的梭形至扁平内皮衬里的血管腔，伴有纤维化区域。未发现核异型性或坏死。这些发现是卡波西样血管内皮细胞瘤的典型例子，没有任何紊乱的凝血谱。该病例引起了人们的兴趣，因为它以一种罕见的部位（如十二指肠）和没有共存的 KMP 为特征，表现为先天性偶然出现。
