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題 名 | Primary Intracranial Germ-Cell Tumor in Children=原發性兒童顱內胚芽母細胞瘤 |
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作 者 | 林英智; 徐山靜; 朱惠瑜; 遲景上; | 書刊名 | 中華醫學雜誌 |
卷 期 | 60:5 1997.11[民86.11] |
頁 次 | 頁259-264 |
分類號 | 417.5821 |
關鍵詞 | 胚芽母細胞瘤; 顱內; 混合型胚芽母細胞瘤; 胚芽瘤; 尿崩症; Germ-cell tumor; Intracranial; Mixed germ-cell tumor; Germinoma; Diabetes insipidus; DI; |
語 文 | 英文(English) |
中文摘要 | 背景:原發於顱內之胚芽母細胞瘤大約占所有臺灣小兒中樞神經系統原發惡性腫瘤的11.1%。因其好發於下視丘至腦下垂體區域,並且常分泌生化物質,因此顱內胚芽母細胞瘤經常表現神經及內分泌症狀。進一步說,常常在神經症狀出現前或者在放射線檢查可以發現異常的很久一段時間以前,病患即有內分泌方面症狀。本篇報告乃臺中榮民總醫院之經驗並且和相關文獻比較討論。 方法:自1983年1995年,共有12例診斷為原發性顱內胚芽母細胞瘤的病童。我們仔細地回顧其病歷,統計分析臨床症狀和腫瘤位置的關係;腫瘤組織分類,治療方法和其預後的相關性。 結果:12例病童中,男性有7例,女性有5例,年齡分布從5至15歲。最常發生的症狀為顱內壓增高的症狀(9/12),其次為尿崩症(8/12),視覺損傷(8/12)及男孩性早熟(3/12)。有11例發生於松果體到腦下垂體區域。其中兩側合併有松果體及腦下垂體的病灶。3例發生於松果體的兒童都有內壓增高的症狀,其中2例有視覺損傷的症狀;而6例發生腦下垂體區域的病童,最常發生的三個症狀為:尿崩症(6/6),顱內壓增高的症狀(5/6),及視覺損傷(5/6)。有6例組織分類為純胚芽瘤,此6例皆有接受放射線或外科手術治療,目前都存活。另6例為非純胚芽瘤,即使皆有接受積極治療其中包含放射線,外科手術和化學治療,目前有3例已經死亡,平均存活時間為3.3年。 結論:對於患有中樞性尿崩症的兒童,或是患有性早熟的男童,必須給予完整檢查以排除顱內胚芽母細胞瘤的可能性。即使檢查結果正常也必須長期追蹤。正確且完整的病理組織診斷,可在幫助我們給予正確治療計畫。對於非純胚芽瘤,研究新治療方法以改善其預後是目前切要課題。 |
英文摘要 | Background: Primary intracranial germ-cell tumors (GCTs) account for about 11.1% of all primary brain tumors of children in Taiwan. Because these tumors commonly involve the hypothalamus-pituitary gland regions and their biochemical secreting character, patients frequently display neuroendocrinological symptoms and signs. Endocrinopathy, if present, often occurs prior to other neurological or radiological manifestations. This article reviews experience here, with present results comparing them with previous reports. Methods: Twelve children who were diagnosed with primary intracranial germ-cell tumors between 1983 to 1995 were studied retrospectively. Their clinical presentations, laboratory results and treatment modalities as well as the current status were collected for presentation here. Results: There were seven boys and five girls. The age distribution was from 5 to 15 years old. The most common symptom was increased intracranial pressure (9/12), followed by diabetes insipidus (8/12), vision deficit (8/12) and sexual precocity in 3 boys. In 11 patients the tumors arose from the suprasellar or pineal regions. In two patients the tumors arose synchronously in the suprasellar and pineal reginos. Pure germinoma was found in six patients. Only one had elevated tumor marker. These six patients all received radiation with or without operation therapy, and all are still alive. Six patients, each wih a non-germinomatous malignant germ-cell tumor, had a poorer prognosis. Although they received aggressive treatment, including operation, radiation and chemotherapy, three patients died, with a mean survival period of 3.3 years. Conclusions: In cases of diabetes insipidus in children or sexual precocity in boys, a thorough investigation for intracranial germ-cell tumors is recommended. The treatment and outcome are different for germinomas and non-germinomatous malignant germ-cell tumors. A thorough pathological diagnosis is recommended for planning of treatment protocol in order to improve prognosis. |
本系統中英文摘要資訊取自各篇刊載內容。