Faculty Bibliography

Infantile myofibroma is a rare fibromatous tumor that is variable in presentation and is frequently mistaken for hemangioma or rhabdomyosarcoma. We describe a 14-month-old male who presented with multiple, enlarging, firm lesions on the shoulder. Biopsy revealed a proliferation of small spindle cells with myxoid and hyalinized stroma infiltrating into the superficial adipose tissue. We provide a brief review of the clinical presentation, histopathologic features, management and recent advances in our understanding of this rare condition.

OBJECTIVES: We hypothesized that knowing the regulations regarding emergency research without consent would increase public support for this type of research. METHODS: Randomized controlled trial. Consecutive patients presenting during eight 24-h periods received one of two interviews; the intervention interview included an educational paragraph. RESULTS: There were 473 (74% of eligible) patients who participated: 51% (95% confidence interval [CI] 46-55) were willing to be enrolled in a study using exception to informed consent; 84% (95% CI 80-87) believed that current therapy for cardiac arrest offers >/= 50% chance of full recovery, and these patients were less willing to enroll (odds ratio [OR] 0.5, 95% CI 0.3-0.9). The educational intervention increased willingness to enroll (OR 1.3, 95% CI 1.0-1.6, p = 0.03). CONCLUSIONS: A brief educational intervention had only a modest effect on willingness to participate in emergency research without consent. It may be more important to educate patients on the shortcomings of current therapy than on the ethical and regulatory justifications for such research.

BACKGROUND: Alpha-methylacyl-CoA racemase (AMACR), also known as P504S, is a protein that plays an important role in mitochondrial and peroxisomal beta-oxidation of branched-chain fatty acid and bile acid intermediates. AMACR has been established as a valuable diagnostic marker for prostate cancer and has recently been shown to be useful in the diagnosis of colorectal carcinoma. Despite the importance of lipid metabolism in sebum production by sebaceous glands of the skin, there are no studies evaluating the expression of AMACR in sebaceous neoplasms. METHODS: Five samples of normal sebaceous glands as well as five cases each of sebaceous hyperplasia (SH), sebaceous adenoma (SA), basal cell carcinoma (BCC) with sebaceous differentiation and extraocular sebaceous carcinoma (SC) were evaluated for immunohistochemical (IHC) expression of AMACR. Each case was reviewed by a single dermatopathologist and graded using a semi-objective grading schema. RESULTS: Normal sebaceous glands showed strong (4+) expression of AMACR. Among sebaceous neoplasms, SH showed the highest expression (4+), SA and BCC with sebaceous differentiation showed varied expression (2+ and 1+, respectively), and extraocular SC showed no expression of AMACR. CONCLUSIONS: The expression of AMACR is increased in benign sebaceous glands and SH; with decreasing AMACR expression in tumors with less sebaceous differentiation (i.e. SA and SC). These findings provide insight into the potential pathogenesis of sebaceous neoplasms while assisting in the microscopic distinction of SA from SC.