Department of Urology, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China.
Department of Pathology, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China.
Int J Immunopathol Pharmacol. 2021 Jan-Dec;35:20587384211002966. doi: 10.1177/20587384211002966.
Tubulocystic renal cell carcinoma is a rare neoplasm of kidney with low metastatic tendency. There has only been a relatively small collection of literature dedicated to this subtype. Here we present two cases diagnosed in our center with detailed clinical information. Along with literature review, we aim to paint a comprehensive profile of TC-RCC. Hematuria and asthenia could be the chief complaints although most patients are asymptomatic. This lesion has a signature multilocular cystic form on radiology and enhancement of septa should reveal malignancy. Histologically, the cysts are lined by a single layer of flattened, cuboidal/columnar, and hobnail epithelium with enlarged nuclei and intermediate to large nucleoli. PAX8 and AMACR are most commonly positive while CD10 or CK7 could be focally stained in some cases. Overall, the diagnosis of TC-RCC should be based on comprehensive clinical and molecular results because early determination of the lesion could prelude a timely intervention and favorable prognosis.
管状囊性肾细胞癌是一种罕见的肾脏肿瘤,具有较低的转移倾向。只有相对较少的文献专门针对这种亚型进行了研究。在这里,我们报告了在我们中心诊断的两例具有详细临床信息的病例。通过文献复习,我们旨在全面描述 TC-RCC。血尿和乏力可能是主要症状,尽管大多数患者无症状。这种病变在影像学上具有特征性的多房囊性形态,分隔的强化应提示恶性。组织学上,囊肿由单层扁平、立方/柱状、鞋钉状上皮细胞排列而成,细胞核增大,核仁大小介于中等至大。PAX8 和 AMACR 通常阳性,而在某些病例中 CD10 或 CK7 可能局灶性染色。总之,TC-RCC 的诊断应基于全面的临床和分子结果,因为早期确定病变可以及时干预并获得良好的预后。