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題 名 | Clinicopathologic Analysis of Renal and Extrarenal Angiomyolipomas: Report of 44 Cases=44例腎臟及腎外血管肌肉脂肪瘤之臨床病理表現 |
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作 者 | 黃博琪; 陳榮達; 何霖; | 書刊名 | 中華醫學雜誌 |
卷 期 | 63:1 2000.01[民89.01] |
頁 次 | 頁37-44 |
分類號 | 415.53 |
關鍵詞 | 血管肌肉脂肪瘤; 組織化學; 免疫組織化學; 結節性硬化; Angiomyolipoma; Histochemistry; Immunohistochemistry; Tuberous sclerosis; |
語 文 | 英文(English) |
中文摘要 | 背景 血管肌肉脂肪瘤是一種罕見的長在腎臟、肝臟及其它內臟的肉瘤,它主要 是由平滑肌、脂肪細胞及小至中型併有玻璃樣病變的血管組成。本文的目的是探討不同部位 的血管肌肉脂肪瘤其各自獨特的臨床病理表現。 方法 我們收集了 37 個腎臟、4 個肝臟、 1 個淋巴節、1 個皮下組織及 1 個子宮頸的血 管肌肉脂肪瘤樣本,並分析其在臨床、組織、組織化學和免疫組織化學上的差異。 結果 臨床上的腎臟及肝臟血管肌肉脂肪瘤可合併結節性硬化,且好發於女性病患。病理上 的差異有以下幾點:(1) 除了皮下的血管肌肉脂肪瘤有偽包膜以外,其它的都無包膜;(2) 類上皮狀的平滑肌細胞常常出現在肝臟、偶爾在腎臟,但不存在於皮下組織及子宮頸的血管 肌肉脂肪瘤中。(3) 肝臟的血管肌肉脂肪瘤可獨特地表現出髓外造血和玻璃樣嗜伊紅染色性 顆粒。(4) 只有腎臟、淋巴節及肝臟的血管肌肉脂肪瘤細胞可染上 PAS 與 PAS-D 的顆粒及 表現出 HMB-45 的免疫組織化學反應。 結論 合併臨床、組織、組織化學和免疫組織化學的特徵,可顯示出腎臟及腎外血管肌肉脂 肪瘤的差異, 特別是與結節性硬化的相關性、 病患的性別、 平滑肌細胞的形態、 PAS 與 PAS-D 的顆粒之存在及 HMB-45 的免疫組織化學反應之表現。髓外造血和玻璃樣顆粒為肝臟 的血管肌肉脂肪瘤獨有的特徵。這些特別的病理表現不只暗示了不同的血管肌肉脂肪瘤的組 織來源,也可幫助我們作出正確的診斷。 |
英文摘要 | Background. Angiomyolipoma (AML) is a rare soft tissue tumor involving the kidneys, liver and other visceral organs. It is composed of smooth muscle cells, adipocytes and small- to medium-sized hyalinized vessels. The purpose of this study was to investigate the characteristic clinicopathologic manifestations of AML in different tissues. Methods. We collected 37 renal, four hepatic, one lymph node, one subcutaneous and one uterine cervical AML to analyze the clinical, histologic, histochemical and immunohistochemical variations of AML in different tissues. Results. Clinically, renal and hepatic AML were associated with tuberous sclerosis complex and predominate in women. There were several prominent pathologic differences between renal and extrarenal AML. First, none of the tumors were encapsulated except for the subcutaneous AML, which had a pseudocapsule. Secondly, the smooth muscle cells were usually epithelioid shaped in hepatic AML and occasionally in renal AML, but not in subcutaneous or uterine AML. Furthermore, hepatic AML characteristically showed extramedullary hematopoiesis and eosinophilic hyaline granules. Finally, only the tumor cells of renal, lymph node and hepatic AML had periodic acid-Schiff (PAS), PAS-D (after diastase digestion) positive granules and monoclonal mouse anti-human melanosome clone (HMB-45) immunoreactivity. Conclusions. By combining the clinical, histologic, histochemical and immunohistochemical features, some discrepancies between the renal and extrarenal AML were observed, such as female predominance, association with tuberous sclerosis complex, morphology of the smooth muscle cells, presence of PAS, PAS-D positive granules and HMB-45 immunoreactivity in tumor cells. Extramedullary hematopoiesis and hyaline globules are unique to hepatic AMLs. These specific pathologic manifestations not only implicate the histogenesis of AML from different organs, but also assist in making a correct diagnosis. |
本系統中英文摘要資訊取自各篇刊載內容。