Abstract
Pathogmonic signs of von Recklinghausen's disease include cafe au lait spots and multiple neurofibroma. However, the correlation with bleeding is not well known.A 21-year-old man was referred to our hospital because of a swelling in the left cheek region. He had cafe au lait spots on the skin, and the clinical diagnosis was von Recklinghausen's disease. Magnetic resonance imaging showed enhancement of a mass extending from the left infratemporal fossa to the cheek region. Subsequently, the swelling increased rapidly because of hemorrhage in the tumor mass. To confirm the source of hemorrhage, an open biopsy of the temporal region and cheek was performed. The tumor mass included a large hematoma. Histological examination revealed neurofibroma with hematoma. Spontaneous hemorrhage may have been caused by invasion of neurofibroma tissue into a vessel wall or by tumor infection. Rebleeding has not occurred during 3 years of follow-up.
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