Von Recklinghausen´s disease is a relatively rare heredofamilial disease, characterized by cafe-au-lait spot and multiple cutaneous tumors along the nerve course.
The incidence is only one case of the every 3000 birth
A 36 year old house wife was admitted to the 3rd Army Hospital on 12th Oct, 1967 with chief complaints of a large nontender pedunculated tumor mass on the posterior aspect of the right thigh, multiple small sumors and cafe-au-lait spots on the whole body surface. About 16 years prior to admission, she had noticed brownish pigmentation on the face and trunk. 8 years prior to admission, she also noticed hen egg sized tumor mass on the posterior aspect of right thigh which was slowly grown up for 3 years.
Thereafter, during her pregnancy, the hen egg sized mass on the posterior aspect of right thigh was grown rapidly and multiple small sized masses were appeared on the whole body surface.
Two ulcers, many cafe-au-lait spots and elephantiasis were developed on the tumor surface of the posterior aspect of right thigh. So tumor was excised under the general anesthesia, and resected specimen was measured 38 × 27.5 × 72 cm in length, bredth and ciroumference, and 8.5 kg in weight.
Microscopic examination revealed a neurofibroma with mucoid degeneration. The etiology, incidence, clinical symptoms, prognosis and treatment of this disease were discussed and the literature reviewed.
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