A Case of von Recklinghausen's Disease with Giant Hematoma into a Pachydermatocele.

Ogura Chika, Natsuaki Masaru, Miyata Akiko, Lin Yoshiaki, Minami Shoichiro, Ito Takaaki

A case of Recklinghausen’s disease with a giant hematoma inside a pachydermatocele was reported. A 37 year-old male patient was diagnosed with Recklinghausen’s disease at the age of 4 years. From the age of 5,many nodules appeared over the whole body and they gradually increased in number. A pachydermatocele on the right side of his waist increased in size gradually. The tumor had rapidly increased in size over a three-day period when the patient presented himself to our outpatient clinic, whereupon he was immediately hospitalized. We diagnosed a giant hematoma in the pachydermatocele based on findings from Computer Tomography and progressive anemia. Thus we perfomed an emergency operation under general anesthesia to resect the tumor. The weight of the resected tumor was 2.2 kg and the volume of the hematoma was 4,010 ml ; he received a transfusion of 800 ml. It is well known that a pachydermatocele of Recklinghausen’s disease has a tendency to bleed during resection, such operations are generally avoided. But a resection at an early stage of the pachydermatocele should be considered when the tumor has a tendency to grow rapidly.

A Case of von Recklinghausen's Disease with Giant Hematoma into a Pachydermatocele.

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