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I.緒言
著者らはvon Recklinghausen病の完全型を示し,脊髄の多発性Glioblastoma,大脳における大型異型膠細胞の集族,結腸のPolyposisなどを伴つている例を経験した。
本疾患の概念がvon Recklinghausenによつて樹立されて以来,組織発生を中心とする多くの見解が発表されてきたが,現在の趨勢は神経鞘腫と組織発生上同一視する傾向にあるように思われる。
A 26-year-old male beginning at the age of ten had small skin tumors increasing slowly in number and size. From the age of twenty the patient has been suffering from severe headaches, visual disturbances and weakness in the legs.
Pathological findings: The autopsy showed tumors of varying sizes the largest of which was the size of a man's fist. These had grown up in the cranial, spinal and sympathic nerves. Also there were many small tumors in the skin. On some tumors the showed tiny pigmentary chages which were blackisch ra-ther than milk-coffee-colored. The histological appearances of the tumors were summarized in two types of neurinoma; the fascicular. and the reticular. The reticular was noted more often in the larger tumors with dege-nerative changes. The fascicular with a slight meningiomatous nuance was seen locally in several tumors of the intradural spinal nerves, while other of the intradural spinal and cranial nerves had an angiomatous appearance.
In addition to these findings there were multiple glioblastomas of the spinal cord, malformation of the central canal of the spinal cord and polyposis of the colon.
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