Laboratory Studies
- Laboratory studies generally are not beneficial.
Imaging Studies
- Plain radiograph findings generally are not specific. The rare intraosseous lesion presents as a benign-appearing, well-circumscribed lesion. Differential diagnoses for these lesions include giant cell tumors, chordomas (when involving the spine), and chondroblastomas. Massive bony destruction may be present, especially when the lesion involves the sacrum.
- Special studies to consider are those employing computed tomography (CT) scanning or magnetic resonance imaging (MRI). MRI is particularly useful; it shows a usually round or oval mass with a moderately bright signal on T1-weighted images and a bright, heterogeneous signal on T2-weighted images.[8] The mass is usually less than 2.5 cm in size. The lesion enhances uniformly with gadolinium contrast.
Diagnostic Procedures
- Biopsy may be needed to clearly define the tissue type. For bone lesions and for particularly large soft-tissue lesions, biopsy is prudent to ensure correct diagnosis and management. Whenever a biopsy is considered, the strict biopsy guideline principles must be followed.
Histologic Findings
Lesions of the spinal cord often have a dumbbell shape; otherwise, they are fusiform in shape. They have an epineurium encapsulation, frequently with overlying vessels. The cut surface is pink or white. In very large masses, degenerative cysts, hemorrhage, or dystrophic calcification may be present.
Neurilemmomas have a well-defined, fibrous capsule. Histologically, there are 2 distinct regions, known as Antoni A and B.
Antoni A areas are cellular regions with predominantly benign spindle cells in many intersecting bundles. They may palisade around eosinophilic regions that are called Verocay bodies. Antoni A cells are positive for S100 staining.
Antoni B areas are much less cellular and have a background of loose connective tissue that is myxomatous in appearance.
Occasionally, a more aggressive histologic appearance may predominate, but these forms usually lack mitotic figures.
Staging
Neurilemmomas are commonly graded using the Enneking system—consisting of grades 1, 2, and 3—for benign lesions. Grade 1 lesions are inactive, grade 2 lesions deform the surrounding tissues but are not destructive or locally aggressive, and grade 3 lesions are locally aggressive and may invade local tissues but do not have a metastatic potential. Generally, neurilemmomas are grade 2 or 3.
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