Nuchal fibromas
Nuchal type fibromas are rare benign fibrous tumors arising from the connective tissues of the neck.
Terminology
Nuchal fibroma is another acceptable term, whereas the term collagenosis nuchae is now discouraged.
Epidemiology
Nuchal type fibromas are rare tumors and mainly found in men in their 30s and 40s.
Clinical presentation
Nuchal type fibromas usually present as a slowly growing indolent masses .
Associations
Approximately half of the patients with the condition also have diabetes mellitus .
Pathology
Nuchal type fibromas are paucicellular collagenous tumors made of spindle cells with entrapped small nerve bundles similar to traumatic neuromas .
Etiology
The etiology of nuchal type fibroma is unknown .
Location
The typical location is the subcutaneous tissues of the posterior neck. They can also arise from the upper back but have also been seen in the shoulder face and the extremities .
Macroscopic appearance
Nuchal type fibromas are ill-defined tumors of firm consistency and usually white color .
Microscopic appearance
Microscopically nuchal fibromas show the following features :
- thick unorganized composition of collagen fibers with more delicate elastic fibers
- few bland spindle fibroblasts
- entrapped small peripheral nerves and adipose tissue
- extension into the underlying skeletal muscle
Immunohistochemistry
Immunohistochemistry stains usually express CD34 in are negative for β-catenin .
Radiographic features
Ultrasound
Ultrasound findings have been described as hypoechoic .
MRI
Nuchal type fibromas can be well or ill-defined lesions mostly found in the superficial soft tissues .
Signal characteristics
- T1: usually low signal intensity (rarely mixed or high signal)
- T2: low to mixed-signal intensity
- STIR: usually low signal intensity
- T1 C+ (Gd): avid enhancement
Radiology report
The radiological report should include a description of the following:
- location and size of the tumor
- relation to the muscular fascia
- relation to the spinous processes
Treatment and prognosis
Nuchal type fibromas are benign tumors. Local recurrence can happen after excision, but they do not metastasize .
History and etymology
The first description of nuchal fibroma was made by Enzinger and Weiss in 1988 .
Differential diagnosis
The differential diagnosis of nuchal type fibroma includes the following :
Siehe auch:
Assoziationen und Differentialdiagnosen zu Nuchal fibromas:
