Journal of Kidney Cancer and VHL最新文献

Double Trouble: Renal Collision Tumors with Unexpected Histology-A Case Report with Review of Literature. 双重困扰：组织学异常的肾碰撞瘤1例并文献复习。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-08-15 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i3.479

Puneeth Medapati, Harshdeep Singh, Thiyagarajan Kalaiselvi Aravind, Siddharth Yadav

{"title":"Double Trouble: Renal Collision Tumors with Unexpected Histology-A Case Report with Review of Literature.","authors":"Puneeth Medapati, Harshdeep Singh, Thiyagarajan Kalaiselvi Aravind, Siddharth Yadav","doi":"10.15586/jkc.v13i3.479","DOIUrl":"https://doi.org/10.15586/jkc.v13i3.479","url":null,"abstract":"Primary Non-Hodgkin's Lymphoma (NHL) involving the adrenal gland is rare, and its synchronous occurrence with renal cell carcinoma (RCC) is exceedingly uncommon. Although adrenal involvement is observed in approximately 20% of systemic NHL cases, primary adrenal lymphoma accounts for only a small proportion of these cases. RCC accounts for 2-3% of all malignancies and is the second most common genitourinary cancer. The coexistence of RCC with other primary malignancies has been described, often in the context of familial syndromes; however, its synchronous presentation with primary adrenal NHL is exceptionally rare. A 54-year-old male with diabetes presented with intermittent dull left flank pain for 18 months and a recent loss of appetite. Contrast-enhanced computed tomography revealed a large, heterogeneous, hyper-enhancing mass in the left suprarenal region replacing the adrenal gland, along with a separate enhancing exophytic lesion in the left kidney. Positron emission tomography showed high metabolic activity in both lesions and regional lymph nodes. The patient underwent open radical nephrectomy with adrenalectomy and regional lymph node dissection. Histopathology demonstrated diffuse large B-cell lymphoma involving the adrenal gland with extension into the kidney, perinephric tissue, and perihilar fat, along with a separate Grade II clear cell RCC in the lower pole of the kidney. Immunohistochemistry confirmed B-cell lineage lymphoma. Lymph nodes were negative for metastasis. The patient received nine cycles of CHOP (Cyclophosphamide, Doxorubicin [Hydroxydaunorubicin], Vincristine [Oncovin], and Prednisolone) chemotherapy and remains recurrence-free at the 6-month follow-up. This case highlights a rare synchronous occurrence of primary adrenal NHL and ipsilateral clear cell RCC. Shared genetic or immunological mechanisms may underlie this association, warranting further investigation.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 3","pages":"34-38"},"PeriodicalIF":1.3,"publicationDate":"2026-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482183/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148799204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Real-world Effectiveness of Single-Agent Tivozanib in Patients with Metastatic Clear Cell Renal Cell Carcinoma. 替沃扎尼单药治疗转移性透明细胞肾细胞癌的实际疗效。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-08-05 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i3.472

Varun Nandakumar, Yeonjung Jo, Georges Gebrael, Zeynep Irem Ozay, Laxmi Upadhyay, Krishnam Goel, Nicolas Sayegh, Ayana Srivastava, Micah Ostrowski, Tanner Hardy, Edwin Lin, Vinay Mathew Thomas, Benjamin Louis Maughan, Haoran Li, Neeraj Agarwal, Umang Swami

{"title":"Real-world Effectiveness of Single-Agent Tivozanib in Patients with Metastatic Clear Cell Renal Cell Carcinoma.","authors":"Varun Nandakumar, Yeonjung Jo, Georges Gebrael, Zeynep Irem Ozay, Laxmi Upadhyay, Krishnam Goel, Nicolas Sayegh, Ayana Srivastava, Micah Ostrowski, Tanner Hardy, Edwin Lin, Vinay Mathew Thomas, Benjamin Louis Maughan, Haoran Li, Neeraj Agarwal, Umang Swami","doi":"10.15586/jkc.v13i3.472","DOIUrl":"10.15586/jkc.v13i3.472","url":null,"abstract":"Tivozanib, a selective vascular endothelial growth factor receptor (VEGFR) tyrosine kinase inhibitor, is approved for relapsed or refractory metastatic clear cell renal cell carcinoma (mccRCC). However, real-world evidence of its effectiveness is limited. Herein, we sought to assess the real-world outcomes of single-agent tivozanib in patients with mccRCC. This retrospective study utilized the US-based Flatiron Health electronic health record-derived de-identified database. Patients with mccRCC who received single-agent tivozanib were included. Primary endpoints were real-world time to next treatment (rwTTNT) and real-world overall survival (rwOS). rwTTNT was defined as time from start of tivozanib to next therapy or death, and rwOS as time from start of tivozanib to death, with both censored at loss to follow-up. Median rwTTNT and rwOS with 95% confidence intervals (CIs) were estimated using Kaplan-Meier estimator, stratified by the line of therapy. Of the 13,909 patients with renal cell carcinoma in the dataset, 9,732 had clear cell histology. Among these, 145 patients treated with single-agent tivozanib were included in the analysis. The median age was 68 years (IQR, 61-73 years), and 67.6% were males. rwTTNT and rwOS in the third-line therapy setting were 6.9 (95% CI 3.4, 11) and 11.0 (95% CI 8, 21) months, respectively, and in the fourth-line setting were 4.7 (95% CI 3.6, 7.5) and 8.4 (95% CI 7.2, 20) months, respectively. In summary, single-agent tivozanib potentially provides clinically meaningful benefits in heavily pretreated patients with mccRCC. These findings support its role as a useful later-line therapeutic option in mccRCC management.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 3","pages":"27-33"},"PeriodicalIF":1.3,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13456933/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148708075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Primary Pediatric Primitive Neuroectodermal Tumor of Kidney Presenting as a Lumbar Abscess: A Rare Case Report. 小儿原发性肾脏原始神经外胚层肿瘤表现为腰椎脓肿：一罕见病例报告。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-07-15 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i3.483

Shatakshee Tewari, Varsha Kumar, Vatsala Misra

引用次数: 0

Expanding Role of Nephron-Sparing Surgery in Non-syndromic Unilateral Unifocal Wilm's Tumor-Our Experience and Review of Literature. 保留肾元手术在非综合征单侧单灶性肾母细胞瘤中的扩大作用——我们的经验和文献回顾。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-07-08 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i3.340

Sahil Kiran Pethe, Virender Sekhon, Atanu Kumar Pal, Rajesh Ahlawat

{"title":"Expanding Role of Nephron-Sparing Surgery in Non-syndromic Unilateral Unifocal Wilm's Tumor-Our Experience and Review of Literature.","authors":"Sahil Kiran Pethe, Virender Sekhon, Atanu Kumar Pal, Rajesh Ahlawat","doi":"10.15586/jkc.v13i3.340","DOIUrl":"10.15586/jkc.v13i3.340","url":null,"abstract":"Wilms' tumor is the most common renal malignancy in the pediatric age group. The unilateral type is the most prevalent, with a tenth of cases being a part of genetic malformation syndromes. The majority of Wilms' tumors are detected by chance detection of abdominal mass on ultrasound. Treatment protocols established by the Renal Tumor Committee of the Children's Oncology Group (COG) in North America and the International Society of Pediatric Oncology (SIOP) in Europe emphasize approaches such as surgical resection, neoadjuvant chemotherapy, and, in rare cases, irradiation. Radical nephrectomy with lymph node sampling forms the mainstay in the management of unilateral Wilms' tumor (uWT). Nephron-sparing surgery (NSS) is becoming increasingly popular for unilateral pediatric cases due to its ability to preserve functional renal tissue. This article reviews various recent studies that explored the role of NSS in uWT. Additionally, two case studies involving NSS for uWT and their outcomes are presented. This study found that factors such as the timing and duration of neoadjuvant chemotherapy, tumor mass size, presence of tumor margins, and intraoperative ischemia time significantly influenced the outcome of NSS.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 2","pages":"12-21"},"PeriodicalIF":1.3,"publicationDate":"2026-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13357643/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148438358","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Navigating Recurrence in Clear Cell Renal Cell Carcinoma after Adjuvant Immunotherapy. 透明细胞肾细胞癌辅助免疫治疗后复发的导向性。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-07-03 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i3.450

Goutham Sunny, Shrikanth Ravichandran, Abinash Patnaik, Rajan Yadav

{"title":"Navigating Recurrence in Clear Cell Renal Cell Carcinoma after Adjuvant Immunotherapy.","authors":"Goutham Sunny, Shrikanth Ravichandran, Abinash Patnaik, Rajan Yadav","doi":"10.15586/jkc.v13i3.450","DOIUrl":"10.15586/jkc.v13i3.450","url":null,"abstract":"Follow-up of clear cell renal cell carcinoma following adjuvant pembrolizumab is a relatively new clinical problem that is influenced by immune pressure and heterogeneous resistance biology. Adjuvant anti-PD-1 treatment has revolutioniz ed postoperative treatment by improving disease-free survival and the overall survival rate but a significant proportion of patients develop recurrence with unique timing, and clinical and molecular pattern, which has important implications. This review attempts to summariz e and explain the available data on recurrence phenotypes, including timing, disease burden, and molecular determinants and then elucidates how these factors influence the choice of subsequent treatment strategies. Early recurrence may suggest primary immune resistance, supporting a mechanism switch towards vascular endothelial growth factor (VEGF)-targeted or hypoxia-inducible factor-2α (HIF-2α)-targeted therapy, although this interpretat ion remains a hypothesis rather than an established mechanistic truth. Conversely, late recurrence may be associated with residual immunological sensitiv ity, potentially permitting immune checkpoint inhibitors-tyrosine kinase inhibitors combinations or immunotherapy rechallenge in carefully selected patients. The status of focal therapy in the oligometastatic relapse, emerging treatment trends in the real world, and recommendations provided by the modern global guidelines are addressed. In the setting where there are no prospective trials in the post-adjuvant setting, a biologically informed, mechanism-based approach is required to streamline sequencing strategies in this unique and more frequently observed population of patients.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 3","pages":"1-11"},"PeriodicalIF":1.3,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13343324/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148413348","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Prognostic Impact of Metastatic Pattern in Renal Cell Carcinoma: IMDC, IMDC-7, and Meet-URO Scores. 肾细胞癌转移模式对预后的影响：IMDC、IMDC-7和met - uro评分

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-06-22 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i2.456

Okan Cetin, Nihan Eren, Melin Aydan Ahmed, Meltem Ekenel, Mert Basaran

{"title":"Prognostic Impact of Metastatic Pattern in Renal Cell Carcinoma: IMDC, IMDC-7, and Meet-URO Scores.","authors":"Okan Cetin, Nihan Eren, Melin Aydan Ahmed, Meltem Ekenel, Mert Basaran","doi":"10.15586/jkc.v13i2.456","DOIUrl":"10.15586/jkc.v13i2.456","url":null,"abstract":"The International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) score is widely used for prognostic stratification in metastatic renal cell carcinoma (mRCC); however, the value of extended models incorporating metastatic site information remains uncertain in patients treated with first-line tyrosine kinase inhibitor (TKI) monotherapy. We retrospectively analyzed 183 patients with mRCC who received first-line TKI therapy (sunitinib, pazopanib, or cabozantinib) between 2013 and 2023. The overall survival (OS) and progression-free survival (PFS) were assessed using Kaplan-Meier analysis and Cox regression models, and the prognostic performance of IMDC, IMDC-7, and Meet-URO scores was compared using Harrell's concordance index. At baseline, 47% of patients had metastases to high-risk sites (bone, liver, or brain), and 36% had three or more metastatic sites. In univariate analyses, bone, liver, and brain metastases as well as the presence of ≥3 metastatic sites were associated with significantly shorter OS and PFS. On multivariable analysis, anemia, poor performance status, absence of prior nephrectomy, and ≥3 metastatic sites independently predicted worse OS, while anemia, poor performance status, and bone metastasis remained independently associated with inferior PFS. All three prognostic models effectively stratified survival outcomes; however, IMDC-7 demonstrated the highest discriminatory ability for OS, followed by IMDC and Meet-URO. These findings highlight the strong prognostic impact of both metastatic burden and distribution in mRCC and support the incorporation of metastatic site assessment into routine risk stratification, particularly in settings where access to immune-based combination therapies remains limited.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 2","pages":"38-49"},"PeriodicalIF":1.3,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13309919/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148346727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Adult Wilms' Tumor during Pregnancy: A Rare and Challenging Clinical Scenario with Literature Review. 妊娠期成人肾母细胞瘤：罕见且具有挑战性的临床情况及文献综述。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-06-15 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i2.484

Pushpita Roy, Syed Md Asadul Hoque, Wai Wai Mroy

{"title":"Adult Wilms' Tumor during Pregnancy: A Rare and Challenging Clinical Scenario with Literature Review.","authors":"Pushpita Roy, Syed Md Asadul Hoque, Wai Wai Mroy","doi":"10.15586/jkc.v13i2.484","DOIUrl":"10.15586/jkc.v13i2.484","url":null,"abstract":"Wilms' tumor (nephroblastoma) is a predominantly pediatric malignancy and is exceedingly rare in adults, particularly during pregnancy. We report a case of a 26-year-old South Asian woman diagnosed with a left-sided large retroperitoneal mass incidentally during the second trimester of pregnancy. The patient underwent radical nephrectomy during pregnancy, and histopathology and immunohistochemistry confirmed high-risk nephroblastoma. The patient was closely monitored throughout the pregnancy period and delivered a healthy preterm infant via cesarean section. Systemic chemotherapy was started after the delivery. Despite multimodal management, including surgery and systemic chemotherapy, the disease showed aggressive progression with widespread metastases. The patient required multiple lines of chemotherapy and palliative care, with partial symptomatic improvement. This case highlights the diagnostic and therapeutic challenges of managing adult Wilms' tumors during pregnancy, the importance of multidisciplinary care, and the need for timely intervention to optimize maternal and fetal outcomes.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 2","pages":"29-37"},"PeriodicalIF":1.3,"publicationDate":"2026-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13316835/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148362932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pathologic Complete Response After Preoperative Ipilimumab and Nivolumab in an HLRCC Patient with Stage III Renal Cell Carcinoma. Ipilimumab和Nivolumab在hrcc合并III期肾细胞癌患者术前的病理完全缓解。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-06-01 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i2.455

Neil Rakheja, Payal Kapur, John Zimmerman, Asim Afaq, James Brugarolas, Jeffrey Cadeddu, Hans Hammers

引用次数: 0

Concurrent Chronic Myeloid Leukemia and Metastatic Renal Cell Carcinoma in a 61-year-old Female: Successful Treatment with Low-Dose Immunotherapy and Combination Targeted Therapy. 1例61岁女性并发慢性髓系白血病和转移性肾细胞癌：低剂量免疫治疗和联合靶向治疗成功。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-05-29 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i2.437

Ajay Gupta, Shuaib Zaidi, Uma Ravishankar, Shefali Kalra, Sangeeta Taneja, Pankaj Baweja

引用次数: 0

Prognostic Significance of NOTCH1-ICD Expression in Renal Cell Carcinoma. NOTCH1-ICD表达在肾细胞癌中的预后意义。

IF 1.3

Journal of Kidney Cancer and VHL Pub Date : 2026-05-08 eCollection Date: 2026-01-01 DOI: 10.15586/jkc.v13i2.446

Milica Višić, Justine Paris, Manh Duc Hoang, Duško Dunđerović, Snežana Živković Perišić, Sanja Radojević Škodrić, Guilhem Bousquet, Jelena Filipović

{"title":"Prognostic Significance of NOTCH1-ICD Expression in Renal Cell Carcinoma.","authors":"Milica Višić, Justine Paris, Manh Duc Hoang, Duško Dunđerović, Snežana Živković Perišić, Sanja Radojević Škodrić, Guilhem Bousquet, Jelena Filipović","doi":"10.15586/jkc.v13i2.446","DOIUrl":"10.15586/jkc.v13i2.446","url":null,"abstract":"The NOTCH1 signaling pathway regulates proliferation, differentiation, and apoptosis, with its intracellular domain (NOTCH1-ICD) reflecting pathway activation. While NOTCH1 dysregulation has been linked to renal cell carcinoma (RCC), its prognostic significance across RCC subtypes remains unclear. In this study, we analyzed NOTCH1-ICD immunohistochemical expression in 101 RCC patients: 69 clear cell RCC (ccRCC), 15 papillary RCC (pRCC), and 17 chromophobe RCC (chRCC), and correlated results with clinicopathological features and survival. In ccRCC, high NOTCH1-ICD expression (>15% positive nuclei) identified a small subgroup of tumors with aggressive features and a trend toward poorer overall survival; however, in multivariate analysis, tumor grade emerged as the only independent prognostic factor (HR = 3.36, 95% CI: 1.07-10.49, p = 0.037), while NOTCH1 showed nonsignificant association with poorer survival (HR = 1.30, 95% CI: 0.87-1.93, p = 0.203). In contrast, chRCC and pRCC exhibited minimal NOTCH1-ICD expression, with no observable impact on survival. NOTCH1-ICD was also detected in tumor endothelial cells, suggesting potential vascular mimicry. These findings indicate that NOTCH1-ICD may reflect tumor aggressiveness in ccRCC and could have implications for targeted therapy, but its independent prognostic value requires validation in larger cohorts.","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"13 2","pages":"8-15"},"PeriodicalIF":1.3,"publicationDate":"2026-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161165/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147933814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0