A case of intramural coronary amyloidosis associated with hemodialysis.

Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years. The patient was admitted with the working diagnosis of pneumonia. An echocardiography showed markedly reduced biventricular function manifested by low blood pressure with systolic in the 70s and elevated pulmonary artery pressure of 45 mmHg, which did not respond to therapy. Following her demise, the autopsy revealed bilateral pulmonary edema and pleural effusions. There was cardiac amyloid deposition exclusively in the coronary arteries but not in the perimyocytic interstitium. Amyloids were also found in pulmonary and intrarenal arteries and the colon wall. Previous case reports showed that beta 2-microglobulin amyloid deposits in various visceral organs but less frequently in the atrial and/or the ventricular myocardium. In the present case, amyloids in the heart were present in the intramural coronary arteries causing myocardial ischemia and infarction, which was the immediate cause of death.

A case of intramural coronary amyloidosis associated with hemodialysis

Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years. The patient was admitted with the working diagnosis of pneumonia. An echocardiography showed markedly reduced biventricular function manifested by low blood pressure with systolic in the 70s and elevated pulmonary artery pressure of 45 mmHg, which did not respond to therapy. Following her demise, the autopsy revealed bilateral pulmonary edema and pleural effusions. There was cardiac amyloid deposition exclusively in the coronary arteries but not in the perimyocytic interstitium. Amyloids were also found in pulmonary and intrarenal arteries and the colon wall. Previous case reports showed that beta 2-microglobulin amyloid deposits in various visceral organs but less frequently in the atrial and/or the ventricular myocardium. In the present case, amyloids in the heart were present in the intramural coronary arteries causing myocardial ischemia and infarction, which was the immediate cause of death.

Glomerular sparing pattern in primary kidney neoplasms: clinical, morphological and immunohistochemical study.

Glomerular sparing (GS) is defined as a unique growth pattern in which tumor cells replace the majority of renal tubes and overrun intact glomeruli. This phenomenon has been well recognized by pathologists as a typical infiltrative pattern and some studies suggested it was an independent risk factor. Here, we study the clinical, pathological, and immunohistochemical features of primary kidney neoplasms with glomerular sparing pattern. We searched the archives of our pathology department for nephrectomy specimens and reviewed all pathology reports from 2009-2013. We selected cases with tumor and collected clinicopathological information, focusing on re-evaluation of cases with glomerular sparing pattern. To facilitate our study we performed immunohistochemical stains of PAX-8, p63, and InI-1 on selected cases. We selected a total of 204 nephrectomy cases in this study, including 163 cases of renal cell carcinoma; 37 cases of urothelial carcinoma; 4 cases from other categories (Wilms tumor, primary diffuse large B-cell lymphoma, angiolipoma, rhabdoid tumor). Finally, we identified 7 cases of primary kidney tumors with glomerular sparing pattern: 2 cases of clear cell renal cell carcinomas (ccRCC), 1 case of collecting duct carcinoma, 2 cases of urothelial carcinoma (UC), 1 case of diffuse large B-cell lymphoma and 1 case of malignant rhabdoid tumor. The primary kidney tumors with glomerular sparing pattern are rare and incidence in our study is <4% (7/204). There is no specificity for any tumor type, but more commonly seen in high grade UC rather than RCC. It can also be seen in rare neoplasms such as collecting duct carcinoma, lymphoma and malignant rhabdoid tumor. These GS cases need to be recognized as they are often associated with high grade, high stage, large tumor size, and worse prognosis.