Wang Chung-Chieh, Wang Shuo-Meng, Liau Jau-Yu
National Taiwan University Hospital, Taipei, Taiwan.
Int J Surg Pathol. 2012 Oct;20(5):519-22. doi: 10.1177/1066896911434548. Epub 2012 Jan 23.
Hemangioblastoma of the kidney is a rare, newly recognized tumor with morphological features similar to its cerebellar counterpart. There have been only 4 cases reported in the literature in English, all of them occurring in middle-aged to elderly patients. Here, we report a case of renal hemangioblastoma in a young adult without clinical evidence of von Hippel-Lindau disease. The tumor was composed of polygonal cells with mildly eosinophilic to clear cytoplasm and a rich vascular network. Immunohistochemical staining revealed a typical profile (positivity for α-inhibin, neuron-specific enolase and S100; negative results for epithelial membrane antigen, HMB-45, and Melan-A), which confirmed the diagnosis. Despite the similarity to renal-cell carcinoma in morphology, hemangioblastoma of the kidney is clinically indolent. Correct recognition of this pathological entity is important to avoid overdiagnosis and unnecessary clinical treatment.
肾血管母细胞瘤是一种罕见的、新发现的肿瘤，其形态学特征与其小脑对应物相似。英文文献中仅报道过4例，均发生于中老年患者。在此，我们报告1例年轻成人肾血管母细胞瘤，该患者无冯·希佩尔-林道病的临床证据。肿瘤由多角形细胞组成，细胞质呈轻度嗜酸性至透明，血管网络丰富。免疫组织化学染色显示出典型特征（α-抑制素、神经元特异性烯醇化酶和S100呈阳性；上皮膜抗原、HMB-45和Melan-A呈阴性），这证实了诊断。尽管肾血管母细胞瘤在形态学上与肾细胞癌相似，但其临床过程呈惰性。正确认识这一病理实体对于避免过度诊断和不必要的临床治疗很重要。
