Journal of Kidney Cancer and VHL (JKCVHL)
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Primary Renal Fibrosarcoma with Massive Tumor Size and Inferior Vena Cava Extension Mimicking Renal Cell Carcinoma: A Rare Case Report
Full text linkPrimary 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
Partial Nephrectomy of Renal Cell Carcinoma in Horseshoe Kidney with Assistance of Intraoperative Retrograde Pyelogram: A Case Report and Literature Review
Full text linkHorseshoe 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
Rosy Cells with a Rocky Core: Low-Grade Oncocytic Tumor with Osseous Metaplasia
Full text linkOsseous 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
Proceedings of IKCS North America 2025 - Abstracts
No full textWe 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
Body Composition May Be a Novel Presurgical Risk Factor for Acute Kidney Injury among Clear Cell Renal Cell Cancer Patients Undergoing Radical Nephrectomy
Full text linkPatients with renal cell carcinoma (RCC) undergoing nephrectomy are at risk for acute kidney injury (AKI). Prior studies have focused predominantly on nonmodifiable surgical AKI risk factors. We conducted the first investigation of body composition features and AKI to identify factors that could improve presurgical risk stratification and be targeted in future interventions. We analyzed data from 1199 patients with stages I–III, clear cell RCC undergoing radical (RN) or partial nephrectomy (PN) from 2000 to 2020. AKI was defined as a serum creatinine (sCr) increase by 0.3 mg/dL within 48 h or a 1.5-fold increase in sCr within 7 days. Preoperative computed tomography (CT) scans were segmented to determine quantities and radiodensities of adipose tissue and skeletal muscle using Automatica software. Multivariable generalized linear models estimated 7-day risk differences (RD) and 95% confidence intervals (CI) within surgical subgroups. AKI was more frequent among patients undergoing RN (66%) than PN (26%). For RN, only higher visceral adipose tissue (VAT) quantity was significantly associated with greater AKI risk (RD per 40-unit increase 5.2 [95% CI: 1.3, 9.2]). We initially detected a similar association in PN, but after multivariable adjustment for all body composition features, associations were attenuated and became nonsignificant. Associations between presurgical body composition and AKI risk vary by surgery type. Higher VAT quantity increased AKI risk only among RN patients. If confirmed, CT-derived VAT quantity may be a novel pre-surgical imaging characteristic that could be used to inform treatment selection or modified to decrease postoperative AKI risk in RN patients
A Decade of Surgical Management of Renal Cell Carcinoma with IVC Thrombus and Bilateral Renal Tumors: Emphasis on Auto-transplantation
Full text linkTo assess the surgical outcomes and techniques in managing renal cell carcinoma (RCC) with inferior vena cava (IVC) thrombus and bilateral renal tumors with a focus on the role of autotransplantation in complex cases, this retrospective study analyzed 58 patients treated at our center between 2013 and 2023 for RCC with tumor thrombus extending into the IVC and, in some cases, the right atrium (RA). Surgical management included radical nephrectomy and thrombectomy with techniques adapted to thrombus level. For level I and II thrombi, innovative occluding maneuvers were used to control the contralateral renal vein. For level IV thrombi, a beating heart technique combined with cardiopulmonary bypass (CPB) was employed. Of the 10 patients with bilateral renal tumors, 2 underwent autotransplantation and 8 underwent bilateral partial nephrectomy. In this 10-year retrospective study of 58 patients with either RCC with venous tumor extension or bilateral RCC, 40 males and 18 females, with a mean age of 66 ± 8 years. Tumor involvement was predominantly right-sided (72.4%). Thrombus levels included 53.44% Level I, 25.9% Level II, and 3.4% Level IV. Intraoperative and postoperative complications were minimal, affecting 10 patients; patients with Level I thrombus had a better survival rate; and one patient with Level IV thrombus died postoperatively. The mean blood loss was 360 mL and the mean operative time was 195 minutes. Histopathology revealed clear cell carcinoma in 65.5% of cases. Among the 10 patients with bilateral renal tumors, autotransplantation and partial nephrectomies resulted in excellent renal preservation and favorable outcomes. This study demonstrates the effectiveness of radical nephrectomy and thrombectomy for RCC with venous tumor extension. Tailored surgical techniques, including autotransplantation for bilateral tumors, resulted in excellent outcomes with minimal complications. Personalized surgical strategies were key to preserving renal function and improving survival in complex RCC cases
VHL Syndrome with Diabetes Mellitus, and Pulmonary and ThyroidNodules
Full text linkVon Hippel–Lindau (VHL) syndrome is an autosomal dominant hereditary tumor syndrome caused by mutations in the VHL gene. It is characterizedby the occurrence of tumors in multiple organs. Pancreatic involvement in VHL syndrome can present as pancreatic cysts or neuroendocrinetumors, which may interfere with both pancreatic exocrine and endocrine pancreatic functions. To our knowledge, no patients with VHLsyndrome complicated by diabetes mellitus, pulmonary nodules, and thyroid nodules are reported in the literature. This study aims to explore thepathogenesis of diabetes, pulmonary nodules, and thyroid nodules in VHL syndrome through the analysis of a patient with VHL syndrome andto review relevant literature
Reno-Gonadal Venous Anastomosis for Right Renal Venous Drainage Following Juxtarenal Cavectomy for Renal Cell Carcinoma
Full text linkThis article is a case report of a 62-year-old male with a left-sided renal cell carcinoma (RCC) with a level II inferior vena cava (IVC) thrombus and caval occlusion. He was managed with open left radical nephrectomy and juxtarenal cavectomy. To preserve right renal venous drainage, the right renal vein was anastomosed to the right gonadal vein. He has not had any renal functional decline or disease recurrence with 3 years of follow-up. The focus of this article is to discuss this distinctive method for vascular reconstruction as an option for right renal venous drainage following left nephrectomy and juxtarenal cavectomy
Recurrent Metastatic Renal Cell Carcinoma Manifesting as Cutaneous Small Vessel Vasculitis: A Case Report
Full text linkCutaneous small vessel vasculitis (CSVV) is an immune-mediated inflammatory disorder affecting the small dermal vessels and is often linked to autoimmune diseases, infections, or malignancies. Renal cell carcinoma (RCC), a common urologic malignancy, rarely presents with CSVV as a paraneoplastic manifestation. This case describes a 68-year-old woman with a history of left radical nephrectomy for clear cell RCC who presented with persistent flank pain and progressive purpuric, painful lesions on her lower limbs. Imaging revealed recurrent metastatic disease in the left retroperitoneum with inferior vena cava (IVC) involvement and a hypermetabolic right supraclavicular lymph node. Skin biopsy demonstrated neutrophilic infiltration and features of leukocytoclastic vasculitis, while immunohistochemistry confirmed the paraneoplastic nature of the vasculitis with positivity for IgM, vimentin, and cytokeratin. Initiation of sunitinib therapy resulted in significant improvement of the cutaneous lesions and tumor regression, followed by successful tumor debulking and IVC reconstruction. This case underscores the importance of considering paraneoplastic vasculitis in the differential diagnosis of new or atypical skin manifestations in patients with a history of RCC. Timely recognition and targeted therapy can improve outcomes and provide valuable insights into the broader systemic impact of malignancies like RCC
Imaging Characteristics of Pediatric Renal Cell Carcinoma and Wilms Tumor and Its Impact on Their Managementand Outcomes: A Case Report and Review of Literature
Full text linkRenal cell carcinoma (RCC) in children is rare, comprising only 1.4–6.3% of pediatric renal tumors. Differentiating RCC from Wilms tumor, the most common pediatric renal tumor is crucial due to differing management and prognosis. Imaging characteristics, such as the presence of calcifications and cystic components, guided the decision to perform surgery without pretreatment biopsy, reducing the risk of needle tract seeding. Translocation-associated RCC is the most common subtype in children, and surgical resection remains the cornerstone of treatment. Long-term follow-up is essential due to the potential for late recurrences. We are reporting the details of a 4-year-old boy, who presented with a 15-day history of fever and abdominal distension, accompanied by a ballotable mass in the right lumbar region. Imaging studies, including a CT scan, revealed a large complex cystic mass in the right kidney, consistent with Bosniak category IV, and enlarged paraaortic nodes. Further evaluation with an FDG-PET scan confirmed the uptake only in the right kidney. The child underwent a right radical nephrectomy with retroperitoneal lymph node dissection. Histopathology revealed translocation-associated RCC features, characterized by slender papillae, psammoma bodies, necrosis, and uniform epithelial cells with hyperchromatic nuclei. Immunohistochemistry showed positivity for markers including TEF3, keratin, and vimentin, with a KI-67 proliferation index of 10–20%. The final stage was pT2aN0, and the patient had an uneventful recovery, with no recurrence at 36 months of follow-up