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Plexiform granular cell tumor. A report of two cases
1Dermatopathology Section, Boston University School of Medicine, Massachusetts 02118.
The American Journal of Dermatopathology
|October 1, 1994
Summary
A rare plexiform granular cell tumor variant was identified in two young girls, presenting as painful intradermal plaques. Histopathology and immunohistochemistry suggest a neural origin for this granular cell tumor.
Area of Science:
- Dermatopathology
- Pediatric Oncology
- Skeletal Muscle Pathology
Background:
- Granular cell tumors (GCTs) are rare neoplasms of uncertain origin, typically presenting as solitary lesions.
- While commonly found in the head and neck, GCTs can occur in various locations, including the skin.
Observation:
- A novel variant of granular cell tumor exhibiting a plexiform pattern was observed in two pediatric patients.
- The tumors presented clinically as intradermal plaques on the lower leg and back, associated with pain or tenderness.
- Histopathological examination revealed fascicles of granular cells arranged in a plexiform manner, particularly around nerve bundles.
Findings:
- Cytoplasmic granules within the tumor cells were periodic acid-Schiff (PAS) positive.
- Immunohistochemical analysis demonstrated strong positivity for S-100 protein and weak positivity for alpha-1-antichymotrypsin and vimentin.
- The characteristic S-100 positivity supports the proposed neural differentiation of granular cell tumors.
Implications:
- This finding expands the known histopathological spectrum of granular cell tumors.
- The plexiform pattern and neural association provide further evidence for a Schwann cell origin of GCTs.
- Understanding this variant aids in accurate diagnosis and classification of cutaneous neoplasms in children.