Verma Saurav, Dhamija Ekta, Barwad Adarsh, Kumar Venkatesan S, Rastogi Sameer
Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Department of Radiodiagnosis, Dr. B.R.A Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Clin Sarcoma Res. 2020 Jul 30;10:12. doi: 10.1186/s13569-020-00134-8. eCollection 2020.
Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids.
A 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence of Kasabach-Merritt phenomenon. After no improvement with several attempts at debridement and anti-tubercular treatment; a diagnosis of Kaposiform Haemangioendothelioma was reached on the basis of overall clinical picture and histology. The child was treated with propranolol and steroids and had an excellent clinical response and a near complete resolution on imaging at 5 months.
These cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma.
卡波西样血管内皮瘤是一种罕见的血管肿瘤，可累及皮肤、深部软组织或骨骼。它是一种通常见于婴儿的局部侵袭性肿瘤。在此，我们报告一例卡波西样血管内皮瘤患儿，其对普萘洛尔和类固醇治疗有反应。
一名3岁男童右膝下方出现肿胀，并伴有特征性的紫色皮肤病变。没有卡萨巴赫-梅里特现象的证据。在多次清创和抗结核治疗均无改善后；根据整体临床表现和组织学诊断为卡波西样血管内皮瘤。该患儿接受了普萘洛尔和类固醇治疗，临床反应良好，5个月时影像学检查显示几乎完全消退。
这些病例常被误诊，尽管诊断延迟，但通过适当的多模式管理仍有良好的预后。本病例突出了卡波西样血管内皮瘤的独特和典型特征。
