Journal of Kidney Cancer and VHL最新文献

Pheochromocytoma, a rare neuroendocrine tumor of the adrenal glands, drives excessive catecholamine production, precipitating hypertension, cardiovascular crises, and systemic symptoms. Laparoscopic adrenalectomy has long been the surgical gold standard, but robotic adrenalectomy is increasingly recognized as a precise, minimally invasive alternative with potential advantages in recovery and operative precision. This narrative review critically evaluates the efficacy, safety, and cost-effectiveness of robotic adrenalectomy for pheochromocytoma, synthesizing evidence from clinical studies to compare perioperative outcomes, complications, and economic impacts against laparoscopic approaches. While robotic techniques demonstrate promising short-term results, including reduced blood loss and shorter hospital stays, the analysis identifies gaps in long-term outcome data and potential publication bias favoring newer technologies. This review underscores the necessity for rigorous prospective studies to validate these findings and refine patient selection criteria. By contextualizing robotic adrenalectomy within the evolving landscape of minimally invasive surgery, this work aims to guide clinical practice, optimize resource allocation, and improve patient-centered care.

Clear cell renal cell carcinoma (ccRCC) is marked by aberrant hypoxia-driven signaling and enhanced angiogenesis mediated by hypoxia-inducible factor 1-alpha (HIF-1α) and vascular endothelial growth factor alpha (VEGF-α). Zinc (Zn), an essential trace element with emerging anticancer potential, was evaluated for its ability to modulate angiogenesis in von Hippel-Lindau (VHL)-deficient 786-0 cells under normoxic and hypoxic conditions. Using quantitative real-time polymerase chain reaction (qRT-PCR), Western blotting, enzyme-linked immunosorbent assay (ELISA), and immunofluorescence, we observed that Zn treatment reduced HIF-1α expression and VEGF-α secretion across both oxygenation states. Notably, Zn inhibited the hypoxia-induced nuclear accumulation of HIF-1α and attenuated paracrine endothelial activation, as shown by reduced human umbilical vein endothelial cell (HUVEC) viability in conditioned media assays. These effects likely involve transcriptional repression, enhanced proteasomal degradation of HIF-1α, and interference with VEGF-α-dependent signaling. Overall, our findings suggest that zinc may function as a multifunctional modulator of tumor angiogenesis and holds potential as an adjuvant in antiangiogenic strategies, particularly under hypoxic conditions.

Renal 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.

Patients 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 presurgical imaging characteristic that could be used to inform treatment selection or modified to decrease postoperative AKI risk in RN patients.

Cutaneous 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.

The Meet-URO score provided a more accurate prognostication than the international metastatic RCC database consortium (IMDC) risk group classification for patients with metastatic renal cell carcinoma (mRCC) by incorporating the pretreatment neutrophil-to-lymphocyte ratio (NLR) and the presence of bone metastases in different settings of the disease. To additionally validate the Meet-URO score on overall survival (OS) in a cohort of mRCC patients treated with first-line pembrolizumab plus axitinib, a post hoc analysis of the observational prospective ProPAXI study was conducted. Progression-free survival (PFS) was also considered. Harrell's C-index was used to compare the discriminative ability on OS and PFS. Overall, the ProPAXI study included 170 patients. Both the five- and the three-risk group Meet-URO score were evaluated to account for the small sample size. The five Meet-URO risk group score showed a mOS of 27.1 months (p = 0.064) and 10.3 (p = 0.014) months for group 4 and group 5, respectively, while it was not reached for the other groups (p < 0.01). Although a worsening of PFS was observed with increasing the risk group, these differences were not statistically significant (p =0.19). Similar results were observed fot the three-risk group Meet-URO score. Both five and the three Meet-URO risk groups showed a better C-index for OS (0.69 and 0.66, respectively) compared to IMDC (0.62) and for PFS (0.60 and 0.59, respectively) compared to IMDC (0.56). These findings suggest that the Meet-URO score may provide more accurate prognostic stratification than IMDC alone in mRCC patients treated with first-line pembrolizumab and axitinib.

The kidney is enveloped by perirenal fat, which secretes various hormones and cytokines, known as adipokines. Adipokines have been demonstrated to influence the development and progression of tumors, including renal cell carcinoma (RCC). Visfatin, an adipokine secreted by the adipose tissue, has been implicated in RCC, but its precise role remains unclear. In this study, we investigated the expression of visfatin in perirenal fat from patients with RCC and its correlation with the RCC malignant phenotype, and we examined the role of visfatin in RCC cell lines in vitro. This study included adipose tissue samples from 57 Japanese patients with clear cell RCC who underwent partial or radical nephrectomy. We examined the mRNA expression level of visfatin using real-time PCR. In vitro MTT assay and western blot were performed using human RCC cell lines. The mRNA expression of visfatin in peri-tumor versus peri-normal fat was higher in Fuhrman grade ≥2 cases compared with Fuhrman grade 1 cases. Furthermore, the addition of visfatin to RCC cell lines promoted cell proliferation, which was accompanied by increased protein expression of HIF1α, p-Akt, and p-ERK. Conversely, the addition of FK866, a visfatin inhibitor, suppressed cell proliferation and reduced these proteins. Our findings suggest that visfatin from peri-tumor adipose tissue influences the malignancy of RCC and plays a role in promoting the growth of RCC. This indicates a potential mechanism by which adipose tissue contributes to the progression of RCC, providing a possible target for therapeutic intervention.

Von Hippel-Lindau (VHL) disease is a hereditary condition caused by mutations in the VHL-tumor suppressor gene leading to constitutive overproduction of HIF-1alpha and HIF-2alpha, two proangiogenic factors, involved in the development of highly vascular tumors. Published evidence has shown that FSH-receptor is expressed in endothelial cells of blood vessels (BV) in several types of tumors. Given that VHL-associated tumors are highly vascular, it is plausible that FSH-receptor could be expressed in their vasculature as well. This immunohistochemical study involved 71 patients diagnosed with VHL-associated tumors, who required surgical intervention. Tissue specimens from these patients included CNS-hemangioblastoma, pancreatic neuroendocrine tumors (panNET), and clear cell renal cell carcinoma (ccRCC). Immunohistochemical staining was performed using a highly specific monoclonal antibody against the human FSH-receptor to assess its expression in the endothelial cells and tumor cells. The distribution of FSH-receptor staining was analyzed using digital imaging techniques. FSHR-protein expression was detected in the BV endothelial cells in 100% of VHL-associated CNS-hemangioblastoma, panNET, and ccRCC cases. In CNS-hemangioblastoma, 96% of cases showed FSH-receptor positivity in tumor stromal cells. In panNET, 88% of the cases displayed FSH-receptor expression in tumor cells. No tumor cells showed FSH-receptor expression in ccRCC. This is the first study to demonstrate FSH-receptor expression by cells of VHL-associated tumors, with distinct expression patterns in different tumor types.

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant hereditary tumor syndrome caused by mutations in the VHL gene. It is characterized by the occurrence of tumors in multiple organs. Pancreatic involvement in VHL syndrome can present as pancreatic cysts or neuroendocrine tumors, which may interfere with both pancreatic exocrine and endocrine pancreatic functions. To our knowledge, no patients with VHL syndrome complicated by diabetes mellitus, pulmonary nodules, and thyroid nodules are reported in the literature. This study aims to explore the pathogenesis of diabetes, pulmonary nodules, and thyroid nodules in VHL syndrome through the analysis of a patient with VHL syndrome and to review relevant literature.

Ocular melanoma is a form of melanoma that rarely offers actionable mutations for treatment with systemic therapy and is relatively radioresistant. As such, surgery is the mainstay of treatment for localized disease and can be considered for oligometastatic disease. We present a case of ocular melanoma that recurred with a solitary renal metastasis 9 years after initial diagnosis and treatment with intraocular brachytherapy. After multidisciplinary discussion, the patient underwent a partial nephrectomy for her solitary renal metastasis. The patient continued in follow-up 3.5 years after partial nephrectomy. She was treated again surgically for a solitary metastasis to the breast before initiation of systemic therapy once multifocal disease was identified. We suggest interdisciplinary management of patients with metastatic involvement of target organs, given the rapidly changing treatment landscape for melanoma and other forms of cancer.