Neuromuscular choristoma: characteristic magnetic resonance imaging findings and association with post-biopsy fibromatosis

Objective To describe imaging characteristics of neuromuscular choristomas (NMC) and to differentiate them from fibrolipomatous hamartomas (FLH). Materials and methods Clinical and imaging characteristics of six patients with biopsy-proven NMC and six patients with FLH were reviewed by musculoskelet...

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Bibliographic Details
Published in:Skeletal radiology 2013-04, Vol.42 (4), p.567-577
Main Authors: Niederhauser, Blake D., Spinner, Robert J., Jentoft, Mark E., Everist, Brian M., Matsumoto, Jane M., Amrami, Kimberly K.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biopsy
Child
Choristoma - pathology
Diagnosis, Differential
Female
Fibroma - pathology
Fibromatosis, Aggressive - pathology
Humans
Imaging
Infant
Magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
Medicine
Medicine & Public Health
Middle Aged
Neuromuscular Diseases - pathology
Nuclear Medicine
Observer Variation
Orthopedics
Pathology
Radiology
Scientific Article
Sensitivity and Specificity
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Summary:Objective To describe imaging characteristics of neuromuscular choristomas (NMC) and to differentiate them from fibrolipomatous hamartomas (FLH). Materials and methods Clinical and imaging characteristics of six patients with biopsy-proven NMC and six patients with FLH were reviewed by musculoskeletal, a pediatric, and two in-training radiologists with a literature review to define typical magnetic resonance imaging features by consensus. Five radiology trainees blinded to cases and naive to the diagnosis of NMC and a musculoskeletal-trained radiologist rated each lesion as having more than or less than 50 % intralesional fat, as well as an overall impression using axial T1 images. Sensitivity, specificity, accuracy, and interobserver agreement kappa were determined. Results Typical features of NMC include smoothly tapering, fusiform enlargement of the sciatic nerve or brachial plexus elements with T1 and T2 signal characteristics closely following those of muscle. Longitudinal bands of intervening low T1 and T2 signal were often present and likely corresponded to fibrous tissue by pathology. Four of five patients with long-term follow-up (80 %) developed aggressive fibromatosis after percutaneous or surgical biopsy. Nerve fascicle thickening often resulted in a “coaxial cable” appearance similar to classic FLH, however, using a cutoff of
ISSN:0364-2348
1432-2161
DOI:10.1007/s00256-012-1546-7