Elephantiasis neuromatosa is a rare complication of neurofibromatosis. The condition was named by Virchow.
Clinical and pathologic features:
(1) It occurs in patients with neurofibromatosis type 1 (NF-1).
(2) There is hypertrophic enlargement of a limb, usually a lower extremity.
(3) A biopsy shows massive soft tissue neurofibroma.
(4) Severe cases may be associated with disability and/or disfigurement.
The enlarged limb may show:
(1) hypertrichosis
(2) hyperpigmentation
Other causes of elephantiasis should be excluded.
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Specialty: Hematology Oncology, Surgery, general, Surgery, orthopedic
