Cellular neurothekeoma

Cellular Neurothekeoma is a rare, benign tumor that primarily affects the skin. It was first described in the medical literature by Gallager and Helwig in 1980. The term "neurothekeoma" is derived from the Greek words "neuro" meaning nerve and "theke" meaning sheath, suggesting a neural origin. However, the exact origin of cellular neurothekeoma remains uncertain.

History

The term "neurothekeoma" was first used by Gallager and Helwig in 1980 to describe a benign cutaneous tumor of presumed neural origin. In 2005, Fetsch et al. proposed a classification system that divided neurothekeomas into two types: myxoid and cellular. The cellular type is more common and is characterized by a proliferation of spindle and epithelioid cells in a nested and fascicular pattern.

Clinical Presentation

Cellular neurothekeomas most commonly present as solitary, asymptomatic, skin-colored to reddish-brown papules or nodules. They are typically less than 2 cm in diameter and are most commonly found on the head, neck, and upper extremities. The lesions may be present for months to years before diagnosis.

Diagnosis

The diagnosis of cellular neurothekeoma is primarily based on histopathological examination. The tumor is composed of nests and fascicles of spindle and epithelioid cells with eosinophilic cytoplasm. Immunohistochemical staining is typically positive for NKI/C3, CD10, and P75, and negative for S100, HMB45, and Melan A, helping to distinguish cellular neurothekeoma from other cutaneous tumors.

Treatment and Prognosis

The treatment of choice for cellular neurothekeoma is complete surgical excision. The prognosis is excellent, with a low rate of local recurrence and no reported cases of metastasis.

See Also

• Dermatology
• Histopathology
• Immunohistochemistry