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題名 | Cellular Neurothekeoma with Atypical Features in a Child=一位小孩之非典型的細胞性神經鞘瘤 |
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作者 | 何承懋; 曾勝弘; 蔡建誠; 陳芸; | 書刊名 | 慈濟醫學 |
卷期 | 17:1 民94.02 |
頁次 | 頁43-45+59 |
分類號 | 415.78 |
關鍵詞 | 細胞性神經鞘瘤; 非典型表現; 腿部腫瘤; Cellular neurothekeoma; Atypical features; Leg tumor; |
語文 | 英文(English) |
中文摘要 | 在皮膚的良性腫瘤中,細胞性神經鞘瘤並不常見,它是神經鞘瘤的一種變形。細胞性神經鞘瘤的組織病理形態通常表現輕微的細胞分裂,而有非典型表現的細胞性神經鞘瘤則非常少(包括腫瘤較大、深部穿透性、明顯的細胞多性形、高細胞分裂、廣泛的邊緣浸潤性、與血管侵犯等非典型表現),這些非典型表現可能讓它很難與軟組織肉瘤做區分而誤判。我們報告一位9歲的小女孩在左大腿皮慮表面長了一個在6個月內可逐漸長大成1.5×1.3×1公分的腫瘤,在腫瘤除後,病理檢查發現是一個細胞性神經鞘瘤但有很明顯的非典型表現:包括深部穿透到皮下脂肪組織、明顯的細胞多形性、高細胞分裂比率(5/10高倍視野)、及廣泛邊緣浸潤。免疫組織化學染色劑顯示這些細胞的α-smooth muscle actin是陽性,但cytokeratin和S-100是陰性。她術後恢復良好,在一年半的追蹤期間,腫瘤沒有復發。 |
英文摘要 | Cellular neurothekeoma (CNT) is an uncommon benign skin neoplasm and a variant of neurothekeoma (NT). Histopathological examination of cellular neurothekeoma often reveals mild cellular atypia and occasional mitoses. CNTs with atypical features (including large size, deep penetration, marked cytologic plecomorphism, high mitotic rate, diffusely infiltrative borders, vascular invasion) are very rare. The rare presence of atypical features may sometimes make CNT difficult to distinguish from soft tissue sarcoma. We describe a case of a 9-year-old girl with a progressively enlarging left thigh tumor (1.5×1.3×1 cm) over 6 months. After total resection of the tumor, examination revealed histological findings consistent with prominent atypical features, including deep penetration into the subcutaneous adipose tissue, marked cytological pleomorphism, high mitotic rate (5/10HPF), and diffusely infiltrative border. Immunohistochemically, these cells were positive for α-smooth muscle actin, but negative for cytokeratin and S-100. Her clinical course was uneventful without recurrence after one and a half years of follow-up. |
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