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Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare disease of unknown etiology that typically presents as nodal disease in young children. However, it also can present in various extranodal sites and can be difficult to recognize if not considered in the differential diagnosis. We describe a 55-year-old man who was discovered to have extranodal Rosai-Dorfman disease that presented as a cardiac mass involving the left atrium and ventricle during evaluation for atypical chest pain, and discuss the clinical, radiologic, and pathologic findings as well as treatment approach and consideration of a possible relationship of this entity to IgG4-related sclerosing lesions., (Published by Elsevier Inc.)

GLUT-1, an erythrocyte-type glucose transporter protein expressed in juvenile hemangiomas, has recently been shown to be a sensitive marker of perineurial cells and their tumors in a small number of cases. However, GLUT-1 expression has not been systematically examined in other mesenchymal neoplasms. Prompted by a recent report of GLUT-1 expression in epithelioid sarcoma, a tumor not generally felt to show perineurial differentiation, we examined GLUT-1 expression in a wide variety of mesenchymal tumors. Sections from 247 mesenchymal tumors of a variety of histologic subtypes were retrieved from our archives and immunostained for GLUT-1 using heat-induced epitope retrieval and the DAKO ADVANCE detection system (DAKO, Carpinteria, CA). Scoring was as follows: negative (<5% of cells), 1+ (5%-25% of cells), 2+ (25%-50% of cells), and 3+ (>50% of cells). All benign nerve sheath tumors showed a peripheral rim of positive normal perineurial cells, with 2 neurofibromas and 3 schwannomas showing more extensive staining. Three of 4 perineuriomas showed strong GLUT-1 expression. All juvenile hemangiomas were GLUT-1 positive. GLUT-1 expression was also seen in a wide variety of benign and malignant mesenchymal tumors. However, GLUT-1 expression was absent in nonjuvenile hemangioma endothelial tumors and in almost all low-grade lesions that enter the histologic differential diagnosis of perineurial tumors, including low-grade fibromyxoid sarcoma, dermatofibrosarcoma protuberans, and myxofibrosarcoma. We conclude that GLUT-1 expression in mesenchymal tumors is by no means specific for perineurial differentiation, but may instead represent upregulation of this protein within hypoxic zones, secondary to upstream activation of proteins such as hypoxia-inducible factor 1-alpha.

Only 8 cases of hamartomas of mature cardiac myocytes have been reported. The aim of the study was to describe 7 new cases and provide clinicopathologic correlation. Our anatomical pathology database was searched for all cases of cardiac hamartoma, of which 7 represented mature myocyte type. Medical records were reviewed for clinical information, and microscopic slides were evaluated for extent of characteristics. Five males and 2 females ranged in age from 6 months to 74 years (mean, 23 years). There were 11 ventricular hamartomas (8 left free wall, 2 right free wall, 1 septum). Death in 3 infants was unrelated to incidental hamartomas discovered at autopsy. A 10- and 16-year-old were asymptomatic but had abnormal electrocardiogram (ECG) results, which led to detection of cardiac masses by imaging studies. Two adult males had only mild coronary disease angiographically. The 57-year-old, who died suddenly, had a 7-year history of abnormal ECG results. The 74-year-old, who died after aortic surgery, had a 3-year history of chest discomfort. Their hamartomas were identified at autopsy and contributed to sudden death in 1. Microscopically, all hamartomas were involved by myocyte hypertrophy and disarray, without inflammation or calcification. Myocyte vacuolization and venular dilatation occurred only in the pediatric cases, and interstitial adipose tissue only affected 1 adult. In conclusion, hamartomas of mature cardiac myocytes may be detected at any age. They primarily affect males, arise predominantly in the left ventricle, are asymptomatic, may have nonspecific ECG findings, and rarely may be associated with sudden death. Microscopic findings in infants differ from older patients.

Infected aortic aneurysms are uncommon, and only rarely have their surgical pathological features been described. Clinical and histopathologic features were evaluated in patients undergoing surgical repair of infected aneurysms of the descending thoracic or abdominal aorta over a 24-year period. Findings were compared with observations (primarily from autopsy studies) from the previous 25-year period (1950 to 1975) and other more recent reports. Of the 29 patients in our study, 79% were men, 90% had risk factors for atherosclerosis, and 72% had an identifiable risk or source of infection. Fever was present in 76%, and abdominal or back pain was seen in 66%. Among the 20 cases with an identifiable causative organism, staphylococcus accounted for 30%, streptococcus for 20%, salmonella for 20%, Escherichia coli for 15%, and other organisms for 15%. Aneurysms were saccular in 59% and infrarenal in 42%, and had a mean diameter of 5.6 cm. Microscopically, 6 patterns were recognized: acute inflammation superimposed on severe chronic atherosclerosis (55%), atherosclerosis with chronic inflammation (20%), acute inflammation without atherosclerosis (7%), chronic adventitial inflammation (7%), pseudoaneurysm formation (7%), and necrotizing granulomatous inflammation (4%). Special stains for organisms were positive in only 38% of the cases. Pathological findings of this series of surgical specimens spanning the fourth quarter of the twentieth century were not appreciably different from those described in autopsy series from the preceding years, although the causative microorganisms and agents used to treat them, preoperative diagnostic modalities, and surgical approaches have evolved.