Neurothekeoma

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File:Neurothekeoma2.JPG
Neurothekeoma histology slide

Neurothekeoma is a benign cutaneous tumor first described by Gallager and Helwig, who proposed the term in order to reflect the presumed origin of the lesion from nerve sheath.[1] Microscopically, the lesions described closely resembled the tumor, “nerve sheath myxoma,” an entity first described by Harkin and Reed.[2] The latter had, through the years, been variously described as “Bizarre cutaneous neurofibroma,”[3] “Myxoma of nerve sheath,”[3] and “Pacinian neurofibroma.”[3]

Clinically, neurothekeomas present as a solitary nodule of the skin [4] The most common sites of occurrence are the head and neck and the extremities.[4] The lesions range in size from about 0.5 cm. to more than 3 cm.[4] The average patient age is about 25 years, but neurothkeomas may occur at any age.[4] Women are affected about more often; the male to female ratio is approximately 1:2.[4]

Microscopically, neurothekeoma consists of closely aggregated bundles or fascicles of spindle-shaped cells.[5] The fascicles may or may not have a myxoid background.[5]

Since the time of their first description, it has been reported that neurothekeomas are likely not of nerve sheath origin, as implied by the term.[4] Consequently, neurothekeoma and nerve sheath myxoma are likely not related histogenetically, although they are similar in appearance and in behavior.[4]

See also

References

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