三種の原発性脳腫瘍を合併した1例

Triple Primary Intracranial Tumors of Different Cell Types:A case report

I．はじめに

　原発性脳腫瘍における異種多発性脳腫瘍の占める割合は0.1-0.4％とされ^6,8,9），比較的希なものである．特にvon Recklinghausen病（vR病）などの母斑症に合併することはよく知られているが，今回われわれが経験した症例は，多発性のglioblastoma multiformeに多発性髄膜腫と嫌色素性脳下垂体腺種を合併した極めて興味ある症例であり，母斑症も認められなかった．現在までに母斑症を伴わず三種の原発性脳腫瘍が認められた症例が2例報告されている^7,8）．異種多発性脳腫瘍の発生要因及びその治療方針につき文献的考察を含めて報告する．

We report a case of triple intracranial tumors of different cell types without phacomatosis. The patient was a 77-year-old female who was hospitalized with left hemiparesis and vomiting. Computed tomography (CT) scans revealed a large tumor mass in the right frontal lobe and relatively small tumor masses in the medial right frontal and parietal lobes. Other tumors were also detected in the sella turcica, left sphenoidal wing, left anterior clinoidal process and left cerebellar convexity. This case was considered to be one of metastatic brain tumors, and surgery was performed for the right frontal tumor because of its mass effect. The tumor was so highly vascular that it could not be totally removed. Postoperatively, the mass effect showed a gradual in-crease on CT scans because of intratumoral hemor-rhage and peritumoral edema. The patient's conscious-ness level gradually fell, and she died of pneumonia and cardiac insufficiency 1 month after the operation. The surgical specimen of the tumor was diagnosed as glioblastoma multiforme by histological examination. At autopsy, the small tumors in the medial frontal lobe and parietal lobe were found to be clearly separated from the large right frontal tumor and were diagnosed as multicentric glioblastoma multiforme. The sellar tumor revealed chromophobe pituitary adenoma and was diagnosed as a prolactinoma by immunohistoche-mical examination. The tumors in the left sphenoidal wing and left cerebellar convexity were diagnosed as transitional meningiomas. Multiple primary intracranial tumors of different cell types without phacomatosis are relatively rare, but almost 100 reported cases could be found in the literature. However, triple primary brain tumors with no associated phacomatosis are extremely rare. Only three cases including our own have been reported. Among these cases, ours is highly unique in having multicentric glioma, multiple meningiomas and pituitary adenoma. Von Recklinghausen's disease is a well-known factor for multiplicity of brain tumors. Pa-tients with neurofibromatosis type 2 as the subtype, and who are known to have multiple tumors without phacoma have been related to abnormality of chromo-some No. 22. Chromosome or gene analysis is thus very important in patients who have multiple primary brain tumors, although our patient could not be examined in this way. In the present case, the three different tumors were thought to have been incidental, not only because meningioma, pituitary adenoma and glioma have a high incidence among intracranial neoplasms and because they were separate. The etiology and therapy of multi-ple primary intracranial tumors are discussed.