The literature data base has been updated with two papers:
Yang and Chiang present the case of a 38 year old woman who presented with chest tightness and dyspnea. The patient had three previous episodes of spontaneous pneumothorax, and her father and sister had also had spontaneous pneumothoraces. Medical examination found numerous pinpoint papules on the patient’s back, chest X-ray showed a pneumothorax and several X-ray, and a chest tube was inserted. Subsequent thoracic CT revealed multiple pneumothoraces and lung cysts. Genetic testing confirmed a diagnosis of BHD.
The International Society of Urological Pathology Tumour Panel reports recommended additions and updates to the World Health Organisation classification of renal tumours. The Panel recommends that as hybrid oncocytic/chromophobe tumour only occurs in three settings, a diagnosis of BHD should be considered when this tumour is observed. They also recommend that HLRCC-associated RCC should be considered as a distinct epithelial subtype within the classification system, due to its aggressive behaviour.
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