■具有反极性的乳头状肾肿瘤(PRNRP)是一种具有独特临床病理特征的新型实体,仅描述了少数PRNRP患者。
■我们回顾性分析了9例PRNRP患者的数据,并评估了临床差异,组织形态学,免疫组织化学,和分子特征;预后;PRNRP与其他具有乳头状结构的肾脏肿瘤的鉴别诊断。
■有6名男性和3名女性,年龄在36至74岁之间(平均:62.33岁;中位数:68岁)。所有肿瘤均为单发,1~3.7cm(平均2.17cm,中位数2cm),在左右肾道出现了三个和六个肿瘤,分别。病理上,PRNRP很小,界限清楚的肿瘤,具有主要的乳头状形成。衬里上皮由单层的长方体至低柱状细胞组成,其低级核排列在肿瘤细胞的顶极上。水肿,粘液变性,在纤维血管核中发现透明变性。泡沫巨噬细胞,沙玛尸体,含铁血黄素沉积,一些患者存在浸润性肿瘤边界。免疫组织化学,所有肿瘤均显示GATA3弥漫性阳性染色。Sanger测序证实7例患者存在KRAS突变。所有患者术后预后良好,无复发。GATA3阳性染色和波形蛋白阴性染色是区分PRNRP与其他具有类似结构的肾肿瘤的最重要标记。
■这些研究结果表明,PRNRP是一种独特的肾肿瘤亚型,具有特定的病理特征和惰性行为,应与其他肾肿瘤区分开来。尤其是乳头状肾细胞癌。具有倒置核模式的肿瘤细胞单层,GATA3阳性染色和KRAS突变对病理诊断至关重要。由于其令人满意的预后,应另外制定PRNRP患者的监测和随访.
UNASSIGNED: Papillary renal neoplasm with reverse polarity (PRNRP) is a novel entity with unique clinicopathological characteristics, and only a small number of patients with PRNRP have been described.
UNASSIGNED: We retrospectively analyzed the data for nine patients with PRNRP and evaluated differences in the clinical, histomorphological, immunohistochemical, and molecular features; prognosis; and differential diagnosis of PRNRP from other renal tumors with papillary structure.
UNASSIGNED: There were six males and three females aged 36 to 74 years (mean: 62.33 years; median: 68 years). All the tumors were solitary and ranged from 1 to 3.7 cm (mean: 2.17 cm; median: 2 cm), with three and six tumors arose in the left and right renal tract, respectively. Pathologically, PRNRP is a small, well-circumscribed neoplasm with predominant papillary formations. The lining epithelium is composed of a monolayer of cuboidal to low-columnar cells with low-grade nuclei arranged against the apical pole of the tumor cells. Edema, mucinous degeneration, and hyaline degeneration are found in the fibrovascular cores. Foamy macrophages, psammoma bodies, hemosiderin deposition, and infiltrative tumor boundaries were present in some patients. Immunohistochemically, all tumors showed diffuse positive staining for
GATA3. Sanger sequencing confirmed the presence of KRAS mutation in seven patients. All patients had a good prognosis after surgery and were relapse free. Positive staining for
GATA3 and negative staining for vimentin were the most significant markers for differentiating PRNRP from other renal tumors with analogous structure.
UNASSIGNED: These findings suggested that PRNRP is a distinctive subtype of renal tumor with specific pathological features and indolent behaviors that should be distinguished from other renal tumors, especially papillary renal cell carcinoma. A monolayer of tumor cells with an inverted nuclear pattern, positive staining for
GATA3, and KRAS mutation are essential for pathological diagnosis. Owing to its satisfactory prognosis, the surveillance and follow-up of patients with PRNRP should be additionally formulated.