TFE3 Translocation-Associated Renal Cell Carcinoma Presenting as Avascular Necrosis of the Femur in a 19-Year-Old Patient: Case Report and Review of the Literature.

In the United States, renal cell carcinoma (RCC) accounts for approximately 3% of adult malignancies and 90-95% of all neoplasms arising from the kidney. According to the National Cancer Institute, 58 240 new cases and 13 040 deaths from renal cancer will occur in 2010. RCC usually occurs in older adults between the ages of 50 and 70 and is rare in young adults and children. We describe a case of a TFE3 translocation-associated RCC in a 19-year-old patient presenting as avascular necrosis of the femur. Due to the rarity of this malignancy, we present this case including a review of the existing literature relative to diagnosis and treatment.

Trisomy 18 and hepatoblastoma.

A 4-month-old girl with trisomy 18 had a congenital heart defect and an hepatoblastoma.

Unusual histopathology of mucormycosis in acute myelogenous leukemia.

Mucormycosis is a mycotic disease caused by certain genera of the class Zygomycetes (order Mucorales). It is usually a rapidly progressive opportunistic infection characterized by vascular invasion, neutrophilic response, and intravascular thromboses. Entomophthoromycosis describes another disease caused by Zycomycetes, but of the order Entophthorales. In contrast to mucormycosis, entomophthoromycosis is not associated with underlying disease. Vascular invasion by the hyphae does not occur, and the hyphae are surrounded by eosinophilic sheathes. These sheathes are regarded as one form of the Splendore-Hoeppli phenomenon. The authors report an unusual case of mucormycosis in a severely pancytopenic patient dying of acute myelogenous leukemia. At autopsy, eosinophilic sheathes with radiating fibrils were seen about intravascular hyphae, simulating entomophthoromycosis. This pseudo-Splendore-Hoeppli phenomenon appears to be caused by adsorption of plasma and fibrin to the hyphal walls in the absence of both neutrophilic response and extensive fibrin clotting.

Insulin and glucagon production in experimental cirrhosis.

The release of insulin and glucagon in cirrhotic rats was examined. Rats were made cirrhotic by a combination treatment of carbon tetrachloride (CCl4) and phenobarbitone. Liver cirrhosis was verified by histologic findings. Both basal and stimulated release of insulin from isolated pancreatic islets, in vitro, were decreased significantly in cirrhotic rats, as compared with control rats. Basal, but not stimulated, levels of glucagon, in vitro, were reduced significantly in cirrhotic rats. Circulating levels of plasma insulin, glucagon, glucose, bilirubin, and amylase levels were unaffected in cirrhotic rats when compared with control rats. There were no signs of pancreatitis. The results indicated that the release of insulin and glucagon is depressed in cirrhotic rats and in rats treated with phenobarbitone and CCl4. Clearance of circulating insulin and glucagon by the liver was apparently reduced, since circulating levels of insulin and glucagon were unaltered in all treated rats.