濃厚な遺伝を認めた小脳hemangioblastoma（いわゆるLindau腫瘍）の1治験例

A CASE OF CYSTIC HEMANGIOBLASTOMA OF THE CEREBELLUM WITH HEAVY HEREDITY IN THE FAMILY TREE

I．緒言

　1926年，Lindau^1）は小脳に原発する嚢包のうち，壁の一部に血管腫がmural noduleとして存在するものを見出し，かかる症例を多数集めて詳細な研究を行なつた。その際，彼はこの種の腫瘍をその組織学的特徴からangioplastische Tumoren （kapillare Angiome）と呼び，その発生を胎生期における間胚葉の発達異常に帰している。その後本腫瘍はhemangioblastoma （Cushing ＆ Bailey^2））あるいは，angioreticuloma （Bergstrand, Oliv—ecrona ＆ Tonnis^3））なる名称で呼ばれ，その頻度は比較的稀れであるにもかかわらず，綱膜のangioma，脊髄および脊髄神経根のhemangioblastoma，膵，肝，脾，腎，副睾丸などの嚢包を合併することがあり（von Hippel—Lindau病）またときに赤血球増多症を伴ったり，家族的発生をみるという特異性のために注目をひき，すでに欧米においては，かなりの数の症例が報告されている。しかし本邦においてはいまだその報告^4）9）は少なく，1家系に多数の患者の発症をみた例はないようである。

　われわれは最近濃厚な遺伝関係を認めた本症の1例に遭遇し，手術により腫瘍を全摘出し治癒せしめることができたのでここに報告する。

There are many reports of hernangioblastoma of the cerebellum in Japan.

Among them, however, few cases have demon-strated heredity in the family tree.

The patient, a school girl of 13 years, was admitted to the department of pediatrics of our hospital with the chief complaints of severe headache and ataxia on Feb. 17, 1965.

She had been in good health until 3 months prior to admission when she began to have headache at times. She had increasing headache, and on admis-sion time she could neither eat, walk, nor write, because of her vomitting, vertigo, and tremor of her both hands.

The retrograde brachial angiography revealed a round tumor stain in the right posterior fossa.

She was transferred to the department of neuro-surgery of our hospital.

Suboccipital craniectomy was performed on Mar. 1, 1965, and a cystic tumor (approximately 2 gr. of tumor weight and 50 cc of the content of cyst) was successfully removed from her right cerebellar hemisphere. The tumor specimen showed histolo-gically hemangioblastoma of the cerebellum.

Family history revealed 5 more patients with the similar signs and symptoms on her father's side.One of them had been operated and proved to have cerebellar hemangioblastoma, but all 5 cases already died probably by this hemangioblastoma of the cerebellum.

The patient was discharged home with the con-dition of complete self-sufficiency in ADL 40 days after operation.