No abstract

Von Hippel-Lindau syndrome (VHLS) is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal disease, renal involvement typically begins closer to the end of the second decade of life and can present in different ways ranging from simple cystic lesions to solid tumors. Mutations in vhl are most often associated with clear cell renal carcinoma, the most common type of renal cancer, and also play a major role in sporadic cases of clear cell renal carcinoma. The recurrent, multifocal nature of this disease presents difficult challenges in the long-term management of patients with VHLS. Optimization of renal function warrants the use of several different approaches common to the management of renal carcinoma such as nephron sparing surgery, enucleation, ablation, and targeted therapies. In VHLS, renal lesions of 3 cm or bigger are considered to have metastatic potential and even small lesions often harbor malignancy. Many of the aspects of management revolve around optimizing both oncologic outcome and long-term renal function. As new surgical strategies and targeted therapies develop, the management of this complex disease evolves. This review will discuss the key aspects of the current management of VHLS.

In patients treated with RN and TT, CKD is an independent predictor of perioperative morbidity. This clinical variable should be considered when selecting patients and subsequent efforts should be made to optimize other competing risk factors in order to reduce the incidence of perioperative adverse events in this patient population.

No abstract

Pneumomediastinum is a clinical and radiographic finding that is associated with a wide range of acute processes that allow gas to disseminate through the tissues of mediastinum that often necessitates prompt consideration for thoracic intervention. In some circumstances, pneumomediastinum may arise from processes extrinsic to the thoracic cavity and is a secondary process arising from a primary retropharyngeal or retroperitoneal etiology. Acute emphysematous pyelonephritis is a rare and severe infectious disease of the kidney associated with a very high mortality rate. We report a case of advanced emphysematous pyelonephritis presenting with pneumomediastinum successfully treated with simple nephrectomy in the absence of any direct mediastinal intervention.