Context.— Familial adenomatous polyposis (FAP) is a rare genetic disorder with autosomal dominant inheritance, defined by numerous adenomatous polyps, which inevitably progress to colorectal carcinoma unless detected and managed early. Greater than 70% of patients with this syndrome also develop extraintestinal manifestations, such as multiple osteomas, dental abnormalities, and a variety of other lesions located throughout the body. These manifestations have historically been subcategorized as Gardner syndrome, Turcot syndrome, or gastric adenocarcinoma and proximal polyposis of the stomach. Recent studies, however, correlate the severity of gastrointestinal disease and the prominence of extraintestinal findings to specific mutations within the adenomatous polyposis coli gene ( APC), supporting a spectrum of disease as opposed to subcategorization. Advances in immunohistochemical and molecular techniques shed new light on the origin, classification, and progression risk of different entities associated with FAP. Objective.— To provide a comprehensive clinicopathologic review of neoplastic and nonneoplastic entities associated with FAP syndrome, with emphasis on recent developments in immunohistochemical and molecular profiles of extraintestinal manifestations in the thyroid, skin, soft tissue, bone, central nervous system, liver, and pancreas, and the subsequent changes in classification schemes and risk stratification. Data Sources.— This review will be based on peer-reviewed literature and the authors' experiences. Conclusions.— In this review we will provide an update on the clinicopathologic manifestations, immunohistochemical profiles, molecular features, and prognosis of entities seen in FAP, with a focus on routine recognition and appropriate workup of extraintestinal manifestations.

Background Urine culture, the gold standard for detecting and identifying bacteria in urine, is one of the highest volume tests in many microbiology laboratories. The inability to accurately predict which patients would benefit from culture leads not only to monopolization of laboratory resources, but also to unnecessary antimicrobial exposure as patients receive empirical treatment for suspected or presumed urinary tract infections (UTI) while awaiting culture results. A common approach to decrease unnecessary urine culture is screening samples using urinalysis (UA) parameters to determine those that should proceed to culture (reflex). In this study, we compared the performance of a novel uropathogen detection method to urinalysis for purposes of UTI screening. Methods Urine specimens submitted for culture (n = 194) were evaluated by urinalysis and a novel light scattering device (BacterioScan 216Dx UTI System) capable of detecting the presence of bacteria in urine. Sensitivity and specificity for prediction of a positive urine culture by UA and 216Dx were determined relative to urine culture results. A positive urine culture was defined as growth in culture of one or two uropathogens at concentrations of ≥50,000 CFU/mL. Results 194 urine samples were evaluated by UA, 216Dx, and urine culture. The 216Dx demonstrated a 100% [95%CI: 88.43%–100.0%] sensitivity and 81.71% [95%CI: 74.93%–87.30%] specificity for the detection of bacteriuria, vs UA with a sensitivity of 86.67% [95%CI 69.28%–96.24%] and specificity of 71.95% [95%CI: 64.41%–78.68%] when compared to urine culture (diagnostic reference method). Conclusions BacterioScan allows for an alternative method of screening with satisfactory sensitivity and improved specificity that may facilitate a reduction of unnecessary cultures. Additional studies are required to determine if a concomitant decrease in inappropriate antibiotic use can be realized with the 216Dx technology.

Anthracyclines are an effective chemotherapy agent. However, very few cases of idarubicin-induced cardiomyopathy exist. Herein, we describe a case of first-dose idarubicin-related acute heart failure in a woman with a history of myelodysplastic syndrome converted to acute myeloid leukaemia.

Adrenal-renal fusion with adrenal cortical adenoma is a rare anomaly with only a few cases described in the literature. Imaging-based identification of this anomaly remains a diagnostic challenge, making it difficult to differentiate upper pole renal malignancy from adrenal cortical adenoma. We describe a case of a 62-year-old woman with an upper pole cystic renal mass on imaging, who underwent robotic partial nephrectomy. Intraoperatively the renal mass was found to be an adrenal-renal fusion anomaly, with ectopic adrenal tissue. Adrenal-renal infusion of an adrenal cortical adenoma was confirmed on final pathology. Due to lack of imaging-based diagnosis, this condition should be considered in the differential for upper pole renal masses.