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Rudimentary meningocele of the skin. Clinicopathologic features and differential diagnosis
A J Marrogi1, P E Swanson, M Kyriakos
1Department of Pathology, Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University School of Medicine, St. Louis, Missouri.
Abstract:
Although "rudimentary meningocele" (RM) or "meningothelial hamartoma" of the skin is a seemingly recently described entity, it has been included in past reports as a variant of primary cutaneous meningioma. We document our experience with four such lesions and compare the histologic and immunohistochemical features of these cases with those of seven classic meningoceles (CM) and four giant cell fibroblastomas (GCF). Although all of these entities share significant points of microscopic similarity, RM and CM are lesions composed of meningothelial cells, whereas GCF is probably of myofibroblastic origin. Rudimentary and classic meningoceles demonstrate cellular immunoreactivity for vimentin and epithelial membrane antigen, whereas the cells of GCF lack the latter determinant and may express muscle-specific actin. Our observations suggest that RM and CM represent closely related developmental malformations; however, RM becomes clinically apparent in a somewhat older patient population than CM and is not associated with major skeletal anomalies as may be found with CM. The distinction between various cutaneous meningothelial proliferations has prognostic importance, as does their separation from GCF. RM and CM are adequately treated by simple excision, whereas GCF, a probable form of fibromatosis, has the potential for local recurrence.
Insights
Rudimentary meningocele (RM) and classic meningoceles (CM) are skin lesions of meningothelial cells, distinct from giant cell fibroblastomas (GCF). Differentiating these cutaneous proliferations is crucial for prognosis and treatment.
Area of Science:
- Dermatopathology
- Surgical Pathology
- Developmental Biology
Background:
- Rudimentary meningocele (RM), also known as meningothelial hamartoma, is considered a variant of primary cutaneous meningioma.
- Previous reports have described RM, but its distinction from similar cutaneous lesions requires further clarification.
Purpose of the Study:
- To compare the histologic and immunohistochemical features of rudimentary meningoceles (RM) with classic meningoceles (CM) and giant cell fibroblastomas (GCF).
- To elucidate the relationship between RM, CM, and GCF and establish their distinct characteristics for accurate diagnosis and patient management.
Main Methods:
- Histologic examination of four RM lesions, seven CM lesions, and four GCF lesions.
- Immunohistochemical analysis using markers such as vimentin, epithelial membrane antigen, and muscle-specific actin.
Main Results:
- RM and CM are composed of meningothelial cells, showing immunoreactivity for vimentin and epithelial membrane antigen.
- GCF is likely of myofibroblastic origin, lacking epithelial membrane antigen and potentially expressing muscle-specific actin.
- RM presents in older patients than CM and is not associated with skeletal anomalies.
Conclusions:
- RM and CM are closely related developmental malformations, distinct from GCF.
- Accurate differentiation of these cutaneous meningothelial proliferations is important for prognosis.
- RM and CM are treated by excision, while GCF may recur locally.