Elastofibroma is an ill-defined fibroelastic tumor-like condition that affects the neck and upper shoulders. There is a genetic predisposition, with multifocality suggesting an enzymatic defect that results from abnormal elastogenesis. Patients usually present in the 60- to 70- year range, with females affected much more often than males (5:1). Although perhaps due to reporting bias, there is an increased incidence in Japanese patients.
Most elastofibromas are detected as slowly growing, deeply seated, firm masses between the shoulder blades and lower posterior neck. Imaging findings show a poorly circumscribed, heterogenous soft-tissue mass that has contrast enhancement, quite frequently bilateral. Simple excision is used in symptomatic patients.
The lesions range up to 20 cm, showing a histologic mixture of heavy, dense bands of collagenous tissue dissected by fat and abnormal elastic fibers (figure 1). The fat is entrapped within the large number of elastic fibers. The elastic fibers are fragmented into globules, simulating beads on string or pipe cleaners (figure 2). These globules show a serrated edge, highlighted by van Gieson elastic stain. The histologic differential diagnosis includes spindle cell lipoma, nuchal-type fibroma, and fibromatosis.
Lococo F, Cesario A, Mattei F, et al. Elastofibroma dorsi: Clinicopathological analysis of 71 cases. Thorac Cardiovasc Surg 2013;61(3):215-22.
Ochsner JE, Sewall SA, Brooks GN, Agni R. Best cases from the AFIP: Elastofibroma dorsi. Radiographics 2006;26(6):1873-6.
Lester D.R. Thompson, MD
From the Department of Pathology, Southern California Permanente Medical Group, Woodland Hills Medical Center, Woodland Hills, Calif.
Caption: Figure 1. Bands of irregular elastic fibers and fibrosis dissect between adipose tissue.
Caption: Figure 2. The elastic fibers are fragmented into globules, simulating a pipe cleaner (left). The globules are highlighted by a van Gieson elastic stain (right).
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|Title Annotation:||PATHOLOGY CLINIC|
|Author:||Thompson, Lester D.R.|
|Publication:||Ear, Nose and Throat Journal|
|Date:||Apr 1, 2017|
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