Rodgers Brian, Zeim Sam, Crawford Byron, Neitzschman Harold, Daroca Philip, Scher Charles D
Department of Radiology , Tulane School of Medicine, New Orleans, LA, USA.
J Pediatr Hematol Oncol. 2007 Dec;29(12):808-10. doi: 10.1097/MPH.0b013e31815814b1.
Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Subsequent surgery and pathologic assessment revealed a papillary angioendothelioma (Dabska tumor) within lymphatic spaces. The child has no evidence of recurrence or metastases 1 year postoperatively.
乳头状血管内皮瘤是一种罕见的、低级别淋巴管肿瘤，通常发生于真皮内。我们报告了一例6岁女孩，她有孤立性脾肿大，伴有早饱及体重减轻症状，被发现患有脾乳头状血管内皮瘤。术前腹部计算机断层扫描显示为不规则的不均匀肿块；标记红细胞扫描排除了血管瘤，而正电子发射断层扫描显示摄取轻度增加。随后的手术及病理评估显示淋巴管间隙内有乳头状血管内皮瘤（达布斯卡瘤）。该患儿术后1年无复发或转移迹象。
