Department of Pathology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Pathology, Abu Ali Sina Hospital, Shiraz Transplant Center, Shiraz University of Medical Sciences, Shiraz, Iran.
J Med Case Rep. 2023 Sep 22;17(1):404. doi: 10.1186/s13256-023-04144-0.
Sclerosing angiomatoid nodular transformation of the spleen is a relatively rare benign vascular lesion in both adult and pediatric age groups with unclear etiopathogenesis and variable clinical presentations. Many benign and also malignant splenic masses could mimic sclerosing angiomatoid nodular transformation, both clinically and radiologically. Herein, we report our experience with a case of sclerosing angiomatoid nodular transformation in a 3-year-old girl.
A 3-year-old Iranian girl presented with abdominal pain, back pain, and constipation for 2 weeks. She was being followed up by a pediatrician due to her short stature and persistent anemia. Physical examination showed stable vital signs, short stature, pallor, and a puffy face. Laboratory evaluation showed normochromic normocytic anemia with a normal reticulocyte count, ferritin, and hemoglobin electrophoresis. Radiologic assessments revealed a hypoechoic lesion in the spleen with high vascularity, clinically suspected to be lymphoma. She was operated on, and after partial splenectomy, pathologic evaluation of the spleen showed a solitary, well-demarcated, and unencapsulated dark mass. Microscopic examination revealed micronodular appearance composed of irregular-shaped vascular spaces lined by plump endothelial cells and surrounded by concentric collagen fibers, features in keeping with sclerosing angiomatoid nodular transformation. The patient's anemia was resolved after surgery, and no clinical or radiologic deficits were noted during the 10-month follow-up visits.
Although sclerosing angiomatoid nodular transformation is exceedingly rare in children, it should be considered a differential diagnosis in pediatric splenic neoplasms with concurrent hematologic manifestations, such as anemia.
脾脏硬化性血管样结节性转化是一种在成人和儿童群体中都相对罕见的良性血管病变，其病因和发病机制尚不清楚，临床表现也各不相同。许多良性和恶性脾肿块在临床和影像学上都可能与硬化性血管样结节性转化相混淆。在此，我们报告了一例 3 岁女孩的硬化性血管样结节性转化病例。
一名 3 岁的伊朗女孩因腹痛、背痛和便秘持续 2 周就诊。她因身材矮小和持续性贫血而被儿科医生随访。体格检查显示生命体征稳定，身材矮小，面色苍白，面部肿胀。实验室评估显示正细胞正色素性贫血，网织红细胞计数、铁蛋白和血红蛋白电泳正常。影像学评估显示脾脏内有一个低回声病变，血管丰富，临床上怀疑为淋巴瘤。她接受了手术，部分脾切除后，脾脏的病理评估显示为单个、界限清楚、无包膜的暗褐色肿块。显微镜下观察显示，不规则形状的血管腔呈微结节状，由丰满的内皮细胞衬里，周围环绕着同心状的胶原纤维，这些特征符合硬化性血管样结节性转化。手术后，患者的贫血得到了纠正，在 10 个月的随访中没有发现临床或影像学缺陷。
尽管硬化性血管样结节性转化在儿童中极为罕见，但在伴有血液学表现（如贫血）的儿童脾肿瘤中，应将其作为鉴别诊断之一。
