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Characteristics of Diffuse Neurofibroma

May 5, 2009
Written by: , Filed in: Musculoskeletal Radiology
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Characteristics of Diffuse Neurofibroma

Neurofibromas are common soft tissue tumors representing approximately 5% of benign soft tissue neoplasms. About 90% are localized and the remainders are essentially plexiform neurofibromas.

Diffuse neurofibromas are quite rare and demonstrate a poorly defined, infiltrative growth pattern. Malignant transformation is rare.

A retrospective review was made of 339 patients, 10 of which had imaging studies to describe the appearance of diffuse neurofibroma and to assess demographic data.

It discovered that a plaque-like growth pattern, prominent vascularity, and marked enhancement are characteristic of diffuse neurofibroma.

MDCT and MR Update: Body and Musculoskeletal Imaging

University of California San Francisco, Department of Radiology and Biomedical Imaging
MDCT and MR Update: Body and Musculoskeletal Imaging program features helical multi-detector row CT (MDCT) and MRI as it applies to diseases in the chest, abdomen and pelvis, and musculoskeletal system. Newer and established cross-sectional methods are discussed. Click here to read more or order:

MDCT and MR Update: Body and Musculoskeletal Imaging

Results
There were 8 MRIs, 5 CTs, and 1 ultrasound (US). Seven patients had neurofibromatosis (NF) I and 1 patient had NF II.

Six lesions were on the trunk, 3 on the head and neck, and 1 on the extremities.

Five lesions were >15 cm, 4 lesions ranged between 5 and 15 cm, and 1 lesions was <5 cm. Half of the lesions had a plaque-like growth pattern; the mean thickness was 2.5 cm.

Of those, all involved the skin and subcutaneous tissue. Two of those lesions had extension to the fascia.

Three lesions had an infiltrative growth pattern, 2 of which were in the subcutaneous adipose tissue, neither extending to the skin or fascia. The 3rd lesion was intramuscular in the lower extremity.

All lesions contained macroscopic fat.

One lesion had a mass-like growth pattern and one was mixed infiltrative and plaque-like.

Overall, 9 of the 10 lesions had skin and subcutaneous involvement, 6 had fascial extension, with 2 demonstrating soft tissue involvement deep to the fascia. Half of the lesions had prominent vascularity.

MRI signal intensity was nonspecific. Half of the patients had received contrast and all of those had intense enhancement.

On CT, lesions were either similar to muscle or similar to fat and demonstrated enhancement after contrast.

Ultrasound was available in 1 patient with a mixed growth pattern.

The plaque-like portion was hyperechoic and the deep infiltrative portion hypoechoic. Demographic data in 339 patients demonstrated an equal gender distribution.

The mean age was 35.1 +/- 18.2 years and 349 diffuse neurofibromas were found; 9% were in deep structures and the remainder was similar in distribution between the head and neck, extremities and trunk.

Conclusions
Diffuse neurofibroma can have a plaque-like growth pattern, which is characteristic. The infiltrative growth pattern is nonspecific. It involves the skin or subcutaneous tissues, has prominent vascularity, and demonstrates marked contrast enhancement.

Reviewer’s Comments
This study differs from prior reports, demonstrating a wide age range and even distribution pattern between head and neck, extremities, and trunk. NF I has an increased incidence in patients with diffuse neurofibroma and is likely higher than previously reported.

Author: Cornelia Wenokor, MD

Reference:
Hassell DS, Bancroft LW, et al. Imaging Appearance of Diffuse Neurofibroma. AJR; 2008; 190 (March): 582-588

MDCT and MR Update: Body and Musculoskeletal Imaging

University of California San Francisco, Department of Radiology and Biomedical Imaging
MDCT and MR Update: Body and Musculoskeletal Imaging program features helical multi-detector row CT (MDCT) and MRI as it applies to diseases in the chest, abdomen and pelvis, and musculoskeletal system. Newer and established cross-sectional methods are discussed. Click here to read more or order:

MDCT and MR Update: Body and Musculoskeletal Imaging

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