Down症候群に合併した基底核Endodermal Sinus Tumorの1例

A Case of Endodermal Sinus Tumor in the Basal Ganglia Associated with Down's Syndrome

I．はじめに

　Down症候群に白血病が合併しやすいことはよく知られているが，他の悪性腫瘍が合併することは稀とされている．1970年のMillerらの統計では，56,199例のDown症候群の患者のうち5例に脳腫瘍，206例に白血病の合併を認めており，白血病と比較すると明らかに脳腫瘍の合併する頻度は少ない^6）．今回われわれは，Down症候群の患者で右片麻痺にて発症した基底核en—dodermal sinus tumorの1例を経験したので報告する．

A case of Down's syndrome accompanied by endo-dermal sinus tumor of, the left basal ganglia is reported. She was referred to us because of right hemiparesis.A CT scan showed a calcified mass in the left basal ganglia, which was well enhanced on contrast CT. Emergency removal of the tumor was performed because of its rapid growth, intratumoral hemorrhage, and acute hydrocephalus. The histological diagnosis estab-lished after surgery was endodermal sinus tumor. Alphafetoprotein-positive cells were seen in the resected tumor by light microscopy. Postoperative irradiation was very effective, and the high level of serum al-phafetoprotein decreased during the course of irradia-tion. The residual tumor, enhanced on contrast CT, was treated by administration of cisplatin and etoposide after irradiation.

Cases of malignant tumors other than leukemia, which are associated with Down's syndrome, are ex-tremely rare. To our knowledge, including three cases of germ cell tumor, there have been only nine cases of brain tumors associated with Down's syndrome re-ported previously.