Cheng Na, Chen Jianning, Pan Yuhang, Jiang Ye, Zhou Jing, Shao Chunkui
Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China.
Diagn Pathol. 2018 Jan 22;13(1):8. doi: 10.1186/s13000-018-0687-y.
Splenic hamartoma is a rare benign vascular proliferative lesion composed of unorganized sinusoid-like channels lined with plump or flat endothelial cells and characterized by a CD8-positive immunophenotype of the lining cells. Scattered bizarre stromal cells can be found in some splenic hamartomas. The presence of splenic hamartoma with bizarre stromal cells is extremely rare and these bizarre cells make it possible to be regarded as a malignancy. Recognition of this rare histologic variant will help to avoid diagnostic confusion and overtreatment of this benign entity.
We report a case of a 40-year-old man with occasional left-sided waist back pain. A splenic space-occupying lesion was detected by ultrasound and magnetic resonance imaging. Microscopically bizarre large cells were scattered throughout the splenic hamartoma. The cells exhibited atypical nuclei, scarcely visible cytoplasm, and vesicular chromatin, and they did not form expansile clusters and lacked mitotic activity. An immunohistochemical panel was performed. The bizarre cells strongly expressed vimentin, and the Ki-67 index was very low. The lesion was diagnosed as a splenic hamartoma with bizarre stromal cells.
To the best of our knowledge, this is the first systematic review on a splenic hamartoma with bizarre stromal cells; only six cases have been described in the literature. Proper identification is important to secure adequate treatment.
脾错构瘤是一种罕见的良性血管增生性病变，由排列紊乱的类血窦样通道组成，内衬饱满或扁平的内皮细胞，其特征为内衬细胞呈CD8阳性免疫表型。在一些脾错构瘤中可发现散在的奇异间质细胞。伴有奇异间质细胞的脾错构瘤极为罕见，这些奇异细胞可能使其被视为恶性肿瘤。认识这种罕见的组织学变异有助于避免对这种良性病变的诊断混淆和过度治疗。
我们报告一例40岁男性，偶发左侧腰背痛。超声和磁共振成像检查发现脾脏占位性病变。显微镜下，奇异的大细胞散在于整个脾错构瘤中。这些细胞具有非典型核、几乎不可见的细胞质和泡状染色质，它们不形成膨胀性细胞团且缺乏有丝分裂活性。进行了免疫组织化学检测。奇异细胞强烈表达波形蛋白，Ki-67指数非常低。该病变被诊断为伴有奇异间质细胞的脾错构瘤。
据我们所知，这是关于伴有奇异间质细胞的脾错构瘤的首次系统综述；文献中仅描述了6例。正确识别对于确保适当治疗很重要。
