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.
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.
We report a rare case of a 61-year-old female diagnosed with concurrent Chronic Myeloid Leukemia (CML) and Renal Cell Carcinoma (RCC). The patient had a history of CML treated with imatinib for 4 years, with loss of complete hematological response for 3 months before being diagnosed with RCC and lung metastases. Due to a T315I mutation in the BCR-ABL1 gene, the treatment regimen included a novel combination of Axitinib, Dasatinib, and low-dose nivolumab. The patient showed a remarkable therapeutic response with a complete metabolic response accompanied by a highly significant reduction in the size of the tumor and complete resolution of the metastatic lung lesions, as well as a major molecular response in terms of CML disease control.
We are pleased to present the abstracts accepted for the 2025 IKCS North America meeting! The research detailed here will be presented on stage and exhibited during our meeting, representing the best of what our scientific community has to offer. The ideas you will hear are both a review of what we understand so far about kidney cancer and an exciting look towards the future of how we can change the landscape for fellow researchers, practicing clinicians, and the patients and families who are relying on us to givethem hope as they face challenging diagnoses. Special thanks to our Scientific Planning Committee for their dedication in reviewing and selecting the research with the highest impact, the most creative ideas, and the most relevancy. I encourage you to engage with the science you hear as well as the people bringing these ideas forward! Together, we have the challenge and the privilege of shaping the future of kidney cancer.
We are proud to share the abstracts selected for presentation at the 2026 IKCS: Europe meeting! This featured research will be presented on stage in oral abstracts and exhibited as posters, representing the best of the kidney cancer research community. Contributions by these researchers reflect not only how far we have come in understanding kidney cancer but also the promising directions ahead. I hope you will consider the new perspectives and innovations that have the potential to shape the future for researchers, clinicians, and, most importantly, the patients and families looking to us for hope. We’re grateful to the Scientific Planning Committee for their thoughtful work in choosing research with the most impact and relevancy. As you explore the science and connect with your peers, I encourage you to engage, ask questions, and exchange ideas. We share the responsibility and the opportunity to advance the future of kidney cancer!
Horseshoe kidney (HSK), characterized by the fusion of two kidneys forming a U-shape, presents intricate challenges in renal anatomy and poses a unique landscape for the development of renal cell carcinoma (RCC). This abstract delves into the case of a 74-year-old male with HSK who also developed RCC, where the employment of intraoperative retrograde pyelogram (RP) played a pivotal role in enhancing surgical preci-sion. The patient’s complex tumor was successfully resected through meticulous identification and dissection. A comprehensive literature review reveals the significance of laparoscopic and robotic surgeries in treating RCC within HSKs, with 3D-reconstruction aiding in surgical planning. While advancements in imaging technologies have improved surgical outcomes, the underexplored utility of intraoperative RP stands out. RP provided real-time insights into the renal pelvis anatomy, guiding the surgical team in navigating intricate structures and ensuring optimal recon-struction post-tumor excision. The discussion underscores the challenges posed by RCC in HSKs, importance of preoperative 3D-reconstruction and angiography in surgical planning, and the critical role of intraoperative RP in mapping renal pelvis anatomy. Unlike conventional imaging methods, RP offers dynamic visualization of the renal drainage system, safeguarding against inadvertent closures and enhancing surgical precision. The successful utilization of RP in this case not only facilitated safe tumor resection but also highlighted its potential in managing unclear renal structures. In conclusion, the integration of intraoperative RP in surgical interventions for RCC within HSKs proves instrumental in enhancing surgical precision and navigating complex anatomical variations. By emphasizing the importance of real-time imaging guidance, surgeons can optimize treatment outcomes for individuals with RCC in the challenging context of HSK anomalies.
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.
Renal sarcomas are rare, accounting for less than 1% of all renal malignancies. Ewing sarcoma/PNET of the kidney is an aggressive and extremely rare neoplasm with only 120 cases reported so far. It is seen in young adults, and only few pediatric cases have been reported so far. We report an 8-year-old boy presenting with progressive left lumbar swelling for 1 year and a prior history of antitubercular therapy. Examination revealed a tender subcutaneous abscess measuring 7×5 cm in the left lumbar region. Fine-needle aspiration cytology suggested a small round blue cell tumor. Histopathological examination, supported by immunohistochemistry, confirmed the diagnosis of renal PNET, which is highly aggressive as compared to PNET arising from other sites. It needs to be distinguished from other primary renal tumors owing to its poor prognosis and aggressive nature. Clinical and radiographic features are nonspecific leading to diagnostic challenges. Definitive diagnosis requires histopathological examination and IHC.
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 ultra-sound. 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.
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 highrisk 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.
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.
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is a rare, aggressive hereditary cancer syndrome caused by germline mutations in the fumarate hydratase (FH) gene. Affected patients typically present with renal cell carcinoma (RCC) at a young age and often experience rapidly progressive disease and poor outcomes. Mean survival is significantly shorter for stages III and IV than for stages I and II(15.8 vs 80.7 months), underscoring the need for more effective therapeutic strategies. Here, we report an HLRCC patient with stage III RCC who achieved a pathologic complete response following one cycle of dual immune checkpoint blockade with nivolumab and ipilimumab and remains disease-free 15 months later. This case extends findings from previous reports and suggests that dual checkpoint blockade may result in clinically meaningful activity in a subset of patients.
We report a rare case of a 61-year-old female diagnosed with concurrent Chronic Myeloid Leukemia (CML) and Renal Cell Carcinoma (RCC). The patient had a history of CML treated with imatinib for 4 years, with loss of complete hematological response for 3 months before being diagnosed with RCC and lung metastases. Due to a T315I mutation in the BCR-ABL1 gene, the treatment regimen included a novel combination of Axitinib, Dasatinib, and low-dose nivolumab. The patient showed a remarkable therapeutic response with a complete metabolic response accompanied by a highly significant reduction in the size of the tumor and complete resolution of the metastatic lung lesions, as well as a major molecular response in terms of CML disease control.
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.
Advances in systemic therapies have improved survival in metastatic renal cell carcinoma (mRCC), leading to a growing population of long-term survivors who may receive both radiotherapy (RT) and tyrosine kinase inhibitors (TKIs) during their disease course. Both treatments induce vascular and mucosal toxicity, and their biological effects may overlap, increasing the risk of rare but life-threatening complications such as aorto-esophageal fistula (AEF). Herein, we present the case of a 47-year-old man with mRCC treated with nephrectomy and repeated pulmonary metastasectomies, followed by sunitinib, mediastinal stereotactic body radiotherapy (SBRT), and later cabozantinib for hepatic progression. Five years after thoracic RT and shortly after initiating cabozantinib, the patient developed massive hematemesis due to an AEF. Management included thoracic endovascular aortic repair (TEVAR), esophageal stenting, and prolonged antimicrobial therapy. Despite initial stabilization, recurrent fistulization and infections led to progressive deterioration and death 7 months later. This case underscores the catastrophic potential of RT-TKI interaction in long-term survivor patients. Sequential exposure can transform subclinical vascular injury into fatal outcomes. Risk stratification, nonconcurrent scheduling of RT and anti-VEGF therapy, and vigilant long-term monitoring are essential. Integration of multidisciplinary and palliative approaches is necessary to balance treatment efficacy with safety.
To evaluate whether the length of positive surgical margin carries a risk for recurrence, data of patients that underwent partial nephrectomy (PN) from six centers were evaluated. Fifty-three patints with positive surgical margins (PSMs) (the PSM group) and 438 patients with negative surgical margin (the NSM group) were included in the present study. Pathologic reevaluations were performed, and surgical margins were measured in micrometers. The number of positive margin areas, and the length of the maximum and total positive margins were evaluated. Data were analyzed using SPSS 27 package program. A p-value less than 0.001 was considered statistically significant. Local recurrence occured in 16.98% of patients in the PSM group and 4.24% of patients in the NSM group. (p<0.001). Patients with PSM were at fourfold increased risk for recurrence. Age, gender, tumor location, tumor side and size, and fuhrman grade were not associated with local recurrence of the tumor (p>0.01). However, positive surgical margin was an important risk factor for local recurrence (p<0.01). No relationship was found between pos-itive margin length and local recurrence (p=0.044). Logistic regression analysis did not identify any parameters associated with local recurrence. The presence of a PSM was significantly associated with an increased risk of local recurrence following PN. The number of positive margin foci and total or maximum length of margin involvement were not associated with recurrence. These findings suggest that it is the presence of PSM, rather than its extent, that may be the primary factor influencing oncological risk.
von Hippel-Lindau (VHL) disease, an autosomal dominant inherited disorder resulting from mutations in the VHL gene, is known to be associated with the development of neuroendocrine tumors (NET) in various organs, including the adrenal gland, pancreas, and paraganglion. However, the development of gastric NET (gNET) in VHL disease has not been reported. Limited studies have suggested that clear cell change is a distinctive feature of VHL-associated tumors. Herein, we first report a case of a patient with VHL syndrome who presented with a gNET showing clear cell change, an unusual morphological characteristic of gNET, and harbored a pathogenic germline VHL mutation (c.351 G>T). The patient underwent surgical treatment for retinal hemangioblastoma, gNET, and renal cell carcinoma, in addition to receiving endocrine therapy and antiangiogenic drugs. The patient survived for 17 years. Our case highlights the possibility of VHL-associated NET development in uncommon locations. Further studies are required to elucidate the correlation between VHL mutation sites and the clinical manifestation of the disease.
Horseshoe kidney (HSK), characterized by the fusion of two kidneys forming a U-shape, presents intricate challenges in renal anatomy and poses a unique landscape for the development of renal cell carcinoma (RCC). This abstract delves into the case of a 74-year-old male with HSK who also developed RCC, where the employment of intraoperative retrograde pyelogram (RP) played a pivotal role in enhancing surgical precision. The patient's complex tumor was successfully resected through meticulous identification and dissection. A comprehensive literature review reveals the significance of laparoscopic and robotic surgeries in treating RCC within HSKs, with 3D-reconstruction aiding in surgical planning. While advancements in imaging technologies have improved surgical outcomes, the underexplored utility of intraoperative RP stands out. RP provided real-time insights into the renal pelvis anatomy, guiding the surgical team in navigating intricate structures and ensuring optimal reconstruction post-tumor excision. The discussion underscores the challenges posed by RCC in HSKs, importance of preoperative 3D-reconstruction and angiography in surgical planning, and the critical role of intraoperative RP in mapping renal pelvis anatomy. Unlike conventional imaging methods, RP offers dynamic visualization of the renal drainage system, safeguarding against inadvertent closures and enhancing surgical precision. The successful utilization of RP in this case not only facilitated safe tumor resection but also highlighted its potential in managing unclear renal structures. In conclusion, the integration of intraoperative RP in surgical interventions for RCC within HSKs proves instrumental in enhancing surgical precision and navigating complex anatomical variations. By emphasizing the importance of real-time imaging guidance, surgeons can optimize treatment outcomes for individuals with RCC in the challenging context of HSK anomalies.
Primary renal fibrosarcoma is an exceedingly rare malignant mesenchymal tumor that accounts for only 1-3% of adult renal malignancies, often mistaken for sarcomatoid renal cell carcinoma (RCC) or leiomyosarcoma due to overlapping morphology. Thus, accurate disease diagnosis is crucial for its management. We report a case of a 49-year-old female, a chronic smoker, who presented with right flank pain and progressive abdominal swelling. Clinical examination revealed a firm mass in the right abdomen, and contrast-enhanced computed tomography demonstrated a large exophytic right renal mass with areas of necrosis, infiltration of the psoas muscle, and extension of tumor thrombus into the inferior vena cava (IVC). The patient underwent right radical nephrectomy with IVC thrombectomy, and a 20 × 15 cm irregular renal mass with 2 cm IVC thrombus was excised intraoperatively. Histopathology revealed a high-grade spindle cell neoplasm with interlacing fascicles and herringbone patterns, brisk mitoses (10-12 mitotic figures per 10HPF), and 20% necrosis. Immunohistochemistry showed diffuse vimentin positivity with negative staining for epithelial, myogenic, neural, and renal lineage markers, confirming the diagnosis of high-grade primary renal fibrosarcoma. This case is notable for being the largest renal fibrosarcoma reported to date, with rare IVC extension, features typically associated with advanced RCC rather than fibrosarcoma. Despite aggressive pathology, no metastases were identified, and the patient remained recurrence-free at 6 months postoperatively, with chemotherapy reserved for recurrence or metastasis. This report emphasizes the diagnostic challenges, surgical complexity, and clinical significance of primary renal fibrosarcoma and highlights the importance of including it in the differential diagnosis of large renal masses with vascular involvement.
Osseous metaplasia is rarely reported in renal neoplasms and is predominantly associated with a favorable prognosis. Low-grade oncocytic tumor (LOT) represents a novel diagnostic classification of renal neoplasms that exhibit overlapping features of oncocytomas and chromophobe renal cell carcinoma. To the best of our knowledge, there are two definitive cases of low-grade oncocytic tumors with osseous metaplasia in the English literature. We present the third case of a 52-year-old female diagnosed with a low-grade oncocytic tumor exhibiting osseous metaplasia.
