Journal of Kidney Cancer and VHL最新文献

Wilms tumor, also denoted as nephroblastoma, an embryonal type of renal cancer, is the most common cancer that affects children in the first 5 years of life. Wilms tumor is very rarely seen in adults. Both adults and children showcase varied clinical symptoms. The metastasis of tumor in both adults and children are not uncommon. Though histological differences between children and adults are insignificant, the prognosis of adult Wilms tumors compared to children is abysmal. Despite remarkable advancements in oncology, no standard treatment protocol exists for Wilms tumor in adults. Children Wilms tumor treatment protocol is currently followed for adults with some changes. In this article, we reviewed the available treatment options for Wilms tumor in adults and protocols followed widely.

The widespread use of computed tomography (CT) has increased the incidence of small renal cell masses. We aimed to evaluate the usefulness of the angular interface sign (ice cream cone sign) to differentiate a broad spectrum of small renal masses using CT. The prospective study included CT images of patients with exophytic renal masses ≤ 4 cm in maximal dimension. The presence or absence of an angular interface of the renal parenchyma with the deep part of the renal mass was assessed. Correlation with the final pathological diagnosis was performed. The study included 116 patients with renal parenchymal masses of a mean (± SD) diameter of 28 (± 8.8) mm and a mean age of 47.7 (±12.8) years. The final diagnosis showed 101 neoplastic masses [66 renal cell carcinomas (RCC), 29 angiomyolipomas (AML), 3 lymphomas, and 3 oncocytomas] and 15 non-neoplastic masses [11 small abscesses, 2 complicated renal cysts, and 2 granulomas]. Angular interface sign was statistically comparable in neoplastic versus non-neoplastic lesions (37.6% versus 13.3%, respectively, P = 0.065). There was a statistically higher incidence of the sign when comparing benign versus malignant neoplastic masses (56.25 vs. 29%, respectively, P = 0.009). Also, comparing the sign in AML versus RCC was statistically significant (52% of AML versus 29% of RCC, P = 0.032). The angular interface sign seems beneficial in predicting the nature of small renal masses. The sign suggests benign rather than malignant small renal masses.

Von Hippel-Lindau disease (VHL) is a multisystem cancer syndrome caused by the inactivation of the VHL tumor suppressor gene and involves various organ systems including the central nervous system (CNS), endocrine system, and the kidneys. Tumors seen in patients with VHL disease can be benign or malignant and are usually multifocal, bilateral, and hypervascular in nature. As most lesions associated with VHL are asymptomatic initially, early diagnosis and the institution of an evidence-based surveillance protocol are of paramount importance. Screening, surveillance, and genetic counseling are key aspects in the management of patients diagnosed with VHL disease and often require a multidisciplinary approach and referral to specialized centers. This article will discuss the characteristic lesions seen with VHL disease, their diagnosis, screening protocols and management strategies, as well as an illustrative case to demonstrate the natural progression of the disease with classic imaging findings.

The most common congenital renal fusion anomaly is the horseshoe kidney (HSK) occurring in about 1 in 600-700 individuals in the Indian population. HSKs are associated with problems such as renal stones, obstruction of uretero-pelvic junction causing stasis, and infection due to ectopic location of the kidneys, malrotation of the kidneys, and vascular changes. In general, normally developed kidneys have more incidents of renal cell carcinoma (RCC) as compared to HSKs. The major issue arises during surgery of HSK due to their altered anatomy and aberrant blood supply. We present a case of HSK with RCC located in the isthmus of a 43-year-old woman.

Chromophobe renal cell carcinoma (chRCC) is one of the less common types of kidney cancer and generally portends a more favorable prognosis. RCC with sarcomatoid differentiation has a more aggressive clinical course with poor outcomes. Four cases of chRCC with varying degrees of sarcomatoid differentiation were retrospectively reviewed at our institution, and clinicopathologic data as well as clinical courses were reported. Patients with higher degrees of sarcomatoid differentiation and larger tumors at presentation generally had and worse overall survival. chRCC with sarcomatoid differentiation portends a poor prognosis with limited data on systemic treatment options for metastatic disease.

This study aimed to compare the antero-lateral and posterior localized renal masses in laparoscopic partial nephrectomy with the retroperitoneal approach in terms of operative, functional, and oncological outcomes. Patients who underwent retroperitoneal laparoscopic partial nephrectomy by a single surgeon between January 2013 and January 2021 were included in the study. A one-to-one propensity score matching (PSM) analysis was conducted to obtain two balanced groups. The patients were divided into two groups as posterior and antero-lateral according to the localization of the mass. A total of 239 patients were included in the PSM analysis, with 65 patients allocated to each group. The mean operative time was 79.2 ± 11.2 min in the posterior group, while it was 90.0 ± 11.6 min in the antero-lateral group (P < 0.001). Warm ischemia time was 15.9 ± 2.4 min in the posterior group and 18.6 ± 2.7 min in the antero-lateral group (P < 0.001). The median decrease in eGFR at 1 year was 4.8 (IQR, 2.9-6.9) mL/min in the posterior group and 5.0 (IQR, 2.8-11) mL/min in the antero-lateral group (P = 0.219). The warm ischemia time and clamping technique were found to be significant factors for predicting eGFR change after surgery (β:0.693, 95% CI: 0.39-0.99, P < 0.001; β:6.43, 95% CI: 1.1-11.7, P = 0.017, respectively). We report that retroperitoneal laparoscopic partial nephrectomy provided longer warm -ischemia and operative time for antero-lateral renal masses than posterior masses. However, long-term oncological and functional results were similar for both localizations.

Nephrectomy remains standard treatment for renal cell carcinoma (RCC). The Mayo Adhesive Probability (MAP) score is predictive of adherent perinephric fat and associated surgical complexity, and is determined by assessing perinephric fat and stranding. MAP has additionally predicted progression-free survival (PFS), though primarily reported in stage T1-T2 RCC. Here, we examine MAP's ability to predict overall survival (OS) and PFS in T3-T4 RCC. From our prospectively maintained RCC database, patients that underwent radical nephrectomy (2009-2016) with available abdominal imaging (<90 days preop) and T3/T4 RCC underwent MAP scoring. Survival analyses were conducted with MAP scores as individual (0-5) and dichotomized (0-3 vs 4-5) using Kaplan-Meier method. Multivariable Cox proportional hazard regression models for PFS and OS were built with backward elimination. 141 patients were included. 134 (95%) and 7 (5%) had pT3 and pT4 disease, respectively. 46.1% of patients had an inferior vena cava thrombus. Mean MAP score was 3.22±1.52, with 75 (53%) patients having a score between 0-3 and 66 (47%) having a score of 4-5. Both male gender (p=0.006) and clear cell histology (p=0.012) were associated with increased MAP scores. On Kaplan-Meier and multivariable analysis, no significant associations were identified between MAP and PFS (HR=1.01, 95% CI 0.85-1.20, p=0.93) or OS (HR=1.01, 95% CI 0.84-1.21, p=0.917). In this cohort of patients with locally advanced RCC, high MAP scores were not predictive of worse PFS or OS.