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Neurological Surgery No Shinkei Geka Volume 23, Issue 7 （July 1995）

Neurological Surgery 脳神経外科 23巻7号（1995年7月発行）

Japanese English

症例

下肢原発Alveolar Soft Part Sarcomaの脳転移の2例 Two Cases with Metastatic Intracerebral Alveolar Soft Part Sarcoma 影治照喜 ¹ , 関貫聖二 ¹ , 本藤秀樹 ¹ , 松本圭蔵 ¹ , 中村宗夫 ² , 山下茂 ³ Teruyoshi KAGEJI ¹ , Seiji KANNUKI ¹ , Hideki HONDO ¹ , Keizo MATSUMOTO ¹ , Muneo NAKAMURA ² , Shigeru YAMASHITA ³ ¹徳島大学脳神経外科 ²徳島大学第2病理 ³高知赤十字病院脳神経外科 ¹Department of Neurological Surgery, School of Medicine, The University of Tokushima ²The Second Department of Pathology, School of Medicine, The University of Tokushima ³Department of Neurological Surgery, Kohchi Red Cross Hospital キーワード： Alveolar Soft part sarcoma , Metastatic brain tumor , Surgical treatment Keyword: Alveolar Soft part sarcoma , Metastatic brain tumor , Surgical treatment pp.627-632

発行日 1995年7月10日 Published Date 1995/7/10

DOI https://doi.org/10.11477/mf.1436901054

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Abstract 文献概要
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I．はじめに

　Alveonar soft part sarcomaは非常に稀な腫瘍で若い女性に多く下肢に好発し，増殖は緩慢であるが再発しやすく肺や脳転移が多いといわれている．今回，われわれは肺と脳に転移した下肢原発のalveolar soft part sarco—maの2症例を経験した．本腫瘍の脳転移の場合，絶対的予後は不良といわれているが，1例は脳転移後より18年が経過している現在でも再発を認めていないことから非常に稀な症例と考えられた．本腫瘍の特徴，治療法，組織起源について文献的考察を加えて報告する．

The alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor which usually occurs in the lower extrem-ity of young girls. The incidence of metastasis is said to be highest in the lung (38%), and second highest in the bone and brain (33%). This report describes two cases of metastatic intracerebral alveolar soft part sarcoma, originating in the lower extremity.

A female patient noticed a painless swelling in her right leg at the age of 11, and 10 years later she under-went total removal of the tumor. The diagnosis was alveolar soft part sarcoma. At 37 she was admitted to our service with a diagnosis of cerebral metastasis in the left frontal lobe. Since then she has undergone sur-gical removal 4 times for recurrent cerebral metastasis and twice for lung metastasis. Now she is 55 years old and doing well except for mild left hemiparesis. She survives without cerebral or general metastasis 44 years following the onset of the sarcoma in her right leg and 18 years following the onset of the metastatic brain tumor.

A 30-year-old man, who noticed a painless swelling in his left thigh in January 1991, underwent total re-moval of the tumor and the diagnosis was alveolar soft part sarcoma. He was admitted to our service with no neurological deficits in October 1992, but a CT scan showed a metastatic brain tumor in the left parieto-occipital region. Total removal of this metastatic brain tumor was successfully performed in November 1992. However, he died because of multiple brain and lung metastasis in February, 1994. Only 18 cases of metastatic intracerebral ASPS have been reported in Japan. ASPS shows very slow growth in spite of the sarcoma, but metastatic lesions of the in-tracerebral region have demonstrated rapid growth. We recommend radical removal of the tumor as the best management. Histogenesis of ASPS is controversial, but its origin from muscle is supported by recent im-munohistochemical findings.