Soft tissue gliomatosis. Morphologic unity and histogenetic diversity

Area of Science:

• Pediatric Pathology
• Developmental Biology
• Surgical Oncology

Background:

• Ectopic tissues, known as heterotopias, can manifest in various non-native locations.
• Mature glial tissue in soft tissue is a rare form of heterotopia, particularly in pediatric cases.

Purpose of the Study:

• To describe the clinical, histological, and immunohistochemical features of six pediatric cases with mature glial tissue in soft tissue.
• To explore the potential origins and histogenesis of these unusual glial heterotopias.

Main Methods:

• Histological examination of solitary soft tissue masses in six children.
• Immunohistochemical analysis using antibodies for glial fibrillary acidic protein, vimentin, and epithelial membrane antigen.
• Review of clinical presentations and patient histories.

Main Results:

• Six pediatric patients (4.5 months to 2 years) presented with solitary masses on the chest wall, scalp, or gluteal region.
• All lesions showed pale-staining fibrillary foci of mature neuroglia, immunoreactive for glial fibrillary acidic protein.
• One scalp lesion included cartilage and meningothelial tissue; gluteal and scalp lesions were linked to teratoma recurrence and sequestered encephaloceles, respectively.

Conclusions:

• The histogenesis of soft tissue glial heterotopias is varied, including teratoma recurrence, sequestered encephaloceles, and unknown origins.
• Soft tissue gliomatosis, especially on the chest wall, presents diagnostic challenges due to unclear origins.
• Understanding these rare entities is crucial for accurate diagnosis and management in pediatric pathology.