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Neurological Surgery No Shinkei Geka Volume 48, Issue 4 （April 2020）

Neurological Surgery 脳神経外科 48巻4号（2020年4月発行）

Japanese English

症例

外傷性脳挫傷との鑑別に窮した中枢神経原発悪性黒色腫の1例 A Case of Primary Intracranial Malignant Melanoma Mimicking a Traumatic Brain Contusion 田邉宜昭 ¹ , 豊岡輝繁 ¹ , 遠藤あるむ ¹ , 土井一真 ¹ , 美山真崇 ¹ , 竹内誠 ¹ , 富山新太 ¹ , 大谷直樹 ¹ , 戸村哲 ¹ , 和田孝次郎 ¹ , 森健太郎 ² Noriaki TANABE ¹ , Terushige TOYOOKA ¹ , Arumu ENDO ¹ , Kazuma DOI ¹ , Masataka MIYAMA ¹ , Satoru TAKEUCHI ¹ , Arata TOMIYAMA ¹ , Naoki OTANI ¹ , Satoshi TOMURA ¹ , Kojiro WADA ¹ , Kentaro MORI ² ¹防衛医科大学校病院脳神経外科 ²総合東京病院脳神経外科 ¹Department of Neurosurgery, National Defense Medical College Hospital ²Department of Neurosurgery, Tokyo General Hospital キーワード： central nervous system , malignant melanoma , brain contusion , gamma knife Keyword: central nervous system , malignant melanoma , brain contusion , gamma knife pp.341-347

発行日 2020年4月10日 Published Date 2020/4/10

DOI https://doi.org/10.11477/mf.1436204188

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参考文献 Reference

Ⅰ．はじめに

　悪性黒色腫は，脳実質，軟膜，粘膜組織および皮膚に存在するメラノサイトに由来する悪性腫瘍である．中枢神経原発悪性黒色腫（primary intracranial malignant melanoma：PIMM）は，悪性黒色腫の中でも約1%と極めて稀であり，原発性脳腫瘍の中でも0.1%とされる^3,6)．初期診断時に，頭蓋内圧亢進（43%），神経学的脱落徴候（35%），痙攣もしくはくも膜下出血（16%）を呈し発見されることが多い⁶⁾．発見されてからの生存期間中央値は12カ月とされ⁴⁾，極めて予後不良な疾患である．今回，頭部外傷時に痙攣発作を呈し，偶発的に指摘され当初脳挫傷が疑われていたが，病変の進展後に開頭術により診断されたPIMMの1例について，文献的考察を加えて報告する．

　Primary intracranial malignant melanoma（PIMM）is a rare neoplasm of the central nervous system, accounting for 1% of cases of malignant melanomas and 0.1% of cases of brain tumors. Here, we report a case of PIMM that was initially considered to be a traumatic brain contusion. A 44-year-old man was transferred to a local hospital because of general tonic convulsion after falling while riding a bike. CT showed an irregular high-density area in the left temporal pole, which was diagnosed as a traumatic contusion. MRI performed 3 months after the initial episode revealed an enlarged temporal lesion with surrounding edema, suggestive of a neoplasm. The MRI showed the lesion as mixed signal intensity, suggesting both solid and cystic components. Subtotal resection was performed, except for the tumor adhering to the peripheral middle cerebral arteries（MCAs). The definitive diagnosis was made based on pathological findings and no evidence of extracranial lesions. Gamma knife surgery was performed for the remnant tumor adjacent to MCAs. The radiologically positive tumor chronologically regressed, and the patient remained progression-free for 18 months. Radiological findings of PIMM vary but typically include high density on CT and hyperintensity on T1-weighted MRI. Close observation enabled early diagnosis based on the suspicion of a neoplasm according to atypical radiological findings. PIMM has a poor prognosis with an overall survival of 12.0 months without confirmative treatment. Gamma knife surgery might achieve suppression of this highly progressive tumor.