Renal cell carcinoma

Complete surgical resection of renal cell cancer confined to the kidney offers a hopeful prognosis of long-term remission or cure. Metastatic renal cell cancer is not effectively managed through the traditional modalities of surgery, chemotherapy, hormonal therapy, or radiation therapy. Quantum leaps in the understanding of immunobiology and molecular genetics, as well as the elucidation and application of biologic response modifiers, have created a climate of renewed enthusiasm for defining more active regimens for the management of metastatic renal cell cancer. As long-term remission and cure are highly unlikely for the majority of individuals presenting with advanced disease, attention to quality of life issues such as symptom control, cost containment, and honesty is appropriate. In diseases such as metastatic renal cell cancer where no effective standard of therapy has been demonstrated, participation in well-designed, carefully executed clinical trials with adequate reimbursement is encouraged for all eligible candidates. ²³ Individuals challenged with living with metastatic kidney cancer are aware of the gradual or precipitous nature of their declining process and generally do not harbor unrealistic hope for longterm survival. However, pain relief and comfort are reasonable hopes, and striving for and attaining an optimal quality of life will sustain both the individual and caregiver.     

Renal cell carcinoma: treatment with interferon

All IFN types--alpha, beta, and gamma--appear to have some antitumor activity against RCC. IFNa has been extensively studied and has demonstrated objective response rates between 15% and 20% when administered in a variety of doses, routes, and schedules. Intermediate dose levels may be associated with greater response rates than low dose levels, and high dose levels are poorly tolerated and usually require dose reduction because of toxicity. Among the means of administration, intramuscular or subcutaneous routes are favored because of logistic advantages; in the low- and intermediate-dose ranges chronic sequential administration (daily, three times a week, or five days per week) is tolerable and may ameliorate toxicity; none of these therapeutic recommendations can be proven to be superior, with respect to response, to several other alternatives. No survival advantage can yet be proven to result from IFN therapy for patients with RCC. Studies evaluating combinations of IFNa and other IFNs or cytotoxic agents have demonstrated increased toxicity. Although responses have been seen in the limited number of studies performed to date, these studies do not appear to support in vivo suggestions of dramatic synergism between these agents. Knowledge of the therapeutic use of IFN is in its infancy. Although the response rates described in this review are unimpressive, they are commensurate with the best available conventional therapy for RCC. As clinical strategies for the use of IFN improve, so too, might the therapeutic efficacy in RCC improve.     

Renal cell carcinoma with erythrocytosis and elevated erythropoietic stimulatory activity

A case study is presented of a 55-year-old man who had clear cell renal carcinoma with pulmonary metastases and erythrocytosis. The increase in red blood cell mass was associated with an elevation in erythropoietic stimulatory activity in serum, pleural fluid, and tumor-cyst fluid as determined by the exhypoxic polycythemic mouse assay. It is postulated that the increased erythropoietic stimulatory activity represents autonomous tumor secretion of erythropoietin or an erythropoietin-like material. Electron microscopic studies confirmed the proximal tubular origin of this tumor.     

Renal cell carcinoma containing abundant non-calcified fat

Renal masses found to contain macroscopic fatty elements on CT or MRI imaging can generally be classified as benign angiomyolipomas. Rarely, renal cell carcinomas may also contain evidence of macroscopic fat. When true adipocytic elements are present, this is generally due to a process of osseous metaplasia in which both fat cells and calcification are co-localized within the mass. We present a patient with a large papillary renal cell carcinoma containing abundant fat with sparse, punctate calcification remote from the fatty elements on imaging. This report highlights the need for radiologists to maintain caution when diagnosing renal angiomyolipomas on the basis of macroscopic fat and reviews the current literature on fat-containing renal masses.     

Renal cell carcinoma: staging and surveillance

Renal cell carcinoma is a common malignancy with many histologic subtypes. Appropriate treatment depends not only upon the specific subtype but also the size of the tumor and extent of spread at time of presentation. Approximately 5% of RCCs are part of a hereditary syndrome which must also be considered in the therapeutic decisions. Although some RCCs are detected with ultrasound, CT or MR is required for staging. CT is used most commonly as it is most readily available and relatively less expensive than MR imaging. The TNM classification of the American Joint Committee on Cancer has largely replaced the Robson classification. Early detection, accurate staging, and improved treatment options have resulted in improved 5-year survival of patients with renal carcinoma.     

15例Xp11.2易位/TFE3基因融合相关性肾癌的临床病理特点分析

目的探讨Xp11.2易位/TFE3基因融合相关性肾癌的临床病理学特征、诊断要点及鉴别诊断。方法回顾性分析15例Xp11.2易位/TFE3基因融合相关性肾癌患者的临床特征、组织学特点、免疫组化及荧光原位杂交,并结合相关文献复习讨论。结果15例患者中,男性6例,女性9例,平均(31.3±9.3)岁。12例为体检偶然发现,临床主要症状表现为血尿和腰腹部疼痛。肿瘤直径1.5~15 cm,平均5.4 cm,3例诊断时已发生转移。10例标本大体呈实性,切面灰白多见,其次为杂色、灰褐,5例切面伴囊性变,5例可见出血及坏死区域。镜下观察可见肿瘤由嗜酸性细胞或透明细胞构成的乳头状、巢状及腺泡状结构,肿瘤细胞界限清楚,核仁较明显,间质主要为纤细的纤维血管间隔,4例可以见到砂砾体。免疫组织化学结果显示肿瘤细胞均弥漫表达TFE3、PAX-8及CD10,不同程度表达AE1/AE3、Vimentin、HMB45及AMACR/P504S,CAIX、CK7、CD117均阴性表达。所有病例TFE3荧光原位杂交检测存在TFE3信号分离。结论Xp11.2易位/TFE3基因融合相关性肾癌是一种罕见的肾细胞癌类型,女性高发于男性,其诊断主要依靠组织病理学、免疫组织化学标记TFE3荧光原位杂交来确诊。     

肾嫌色细胞癌伴多发性血管平滑肌脂肪瘤1例报道

肾嫌色细胞癌是肾细胞癌比较少见的一种类型,约占肾细胞癌的5%~7%[1]。血管平滑肌脂肪瘤是肾脏常见的间叶性肿瘤,两种肿瘤发生在同侧肾脏,可形成复合瘤或碰撞瘤。丁彬等[2]统计了1969—2012年国外报道的58例肾血管平滑肌脂肪瘤伴恶性肿瘤,其中只有4例伴嫌色细胞癌,1例伴透明细胞癌和嫌色细胞癌,其余最常见的是伴透明细胞癌。     

Renal cell carcinoma: Evolving and emerging subtypes

Our knowledge of renal cell carcinoma (RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and defined, and our understanding of the biology and clinical correlates of these tumors is changing. Evolving concepts in Xp11 translocation carcinoma, mucinous tubular and spindle cell carcinoma, multilocular cystic clear cell RCC, and carcinoma associated with neuroblastoma are addressed within this review. Tubulocystic carcinoma, thyroid-like follicular carcinoma of kidney, acquired cystic disease-associated RCC, and clear cell papillary RCC are also described. Finally, candidate entities, including RCC with t(6;11) translocation, hybrid oncocytoma/chromophobe RCC, hereditary leiomyomatosis and RCC syndrome, and renal angiomyoadenomatous tumor are reviewed. Knowledge of these new entities is important for diagnosis, treatment and subsequent prognosis. This review provides a targeted summary of new developments in RCC.     

Renal cell carcinoma: real-time contrast-enhanced ultrasound findings

Objective  

To investigate the findings of renal cell carcinomas (RCCs) on contrast-enhanced ultrasound (CEUS).