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Pyloric Atresia
Joseph Junewick, MD FACR
over 7 years ago
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Diffuse Neurofibroma

Case Detail

Anatomy: Musculoskeletal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

Teenage female with neurofibromatosis type 1 and enlarging pigmented skin lesions.


Case Images


Diagnosis

Diffuse Neurofibroma

Findings

MR – Plaque like infiltrative T2 and postgadolinium hyperintensities of the skin and subcutaneous soft tissues of the left arm, axilla, shoulder, breast and ankle.

Discussion

Soft tissue neurofibromas can be divided into 3 groups: localized, plexiform and diffuse. The “localized” form is ovoid or fusiform in shape and the nerve can be traced to and from the lesion. The “plexiform” type involves a long segment of the nerve and its branches and resembles a mass with infiltrating margins. The “diffuse” form is characterized by infiltration subcutaneous tissues.

Diffuse neurofibroma is an uncommon form of neurofobroma that has a uniform matrix of fibrillary collagen and Schwann cells. It occurs primarily in children and young adults. Slow growth with acceleration in adolescence and pregnancy is typical.

Diffuse neurofibromas tend to involve the skin and subcutaneous soft tissues. The lesion creeps between subcutaneous structures without destruction. Occasionally, large ectatic vessels are present. Malignant transformation is rare.

Reference

Chen W, et al. Soft Tissue Diffuse Neurofibromas. J Ultrasound Med 2007; 26:513-518.

Hassel DS, et al. Imaging Appearance of Diffuse Neurofibroma. AJR 2008; 190:582-588.



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