Department of Ophthalmology, the First Affiliated Hospital of General Hospital of People's Liberation Army, Beijing, China.
Clin Exp Ophthalmol. 2014 Mar;42(2):182-9. doi: 10.1111/ceo.12139. Epub 2013 Aug 4.
To assess the activities of heparanase-1 and elements of the hedgehog signalling pathway in alveolar orbital rhabdomyosarcoma.
Specimens (n = 23) were divided into two groups, those from patients with preoperative chemoradiotherapy or untreated patients; six samples of normal extraocular muscle were used as a normal muscle group. The presence of heparanase-1, patched, smoothened and glioma-associated oncogene homologue-1 protein expression was determined in 23 cases of archival paraffin-embedded alveolar orbital rhabdomyosarcoma after immunohistochemistry. RNA was extracted from three groups of paraffin-embedded specimens and messenger RNA expressions of heparanase-1, smoothened and glioma-associated oncogene homologue-1 compared using nested reverse transcriptase polymerase chain reaction and a limiting dilution analysis.
The heparanase-1, patched, smoothened and glioma-associated oncogene homologue-1 protein was expressed in 91.3%, 87.0%, 91.3% and 78.3%, respectively, of the alveolar orbital rhabdomyosarcoma specimens. Untreated rhabdomyosarcoma samples tended to stain intensely, but staining was relatively weak in tissue obtained from the chemoradiotherapy group. The expression levels of heparanase-1, smoothened and glioma-associated oncogene homologue-1 messenger RNA in untreated and chemoradiotherapy groups paralleled that seen with immunology, and there were no significant differences in heparanase-1, smoothened and glioma-associated oncogene homologue-1 messenger RNA levels between the chemoradiotherapy group and the normal muscle group (P > 0.05). However, the messenger RNA in the untreated group were all significantly higher than those in the chemoradiotherapy and normal muscle groups (P < 0.01).
Both heparanase-1 and hedgehog signalling pathway are involved in the pathogenesis of alveolar orbital rhabdomyosarcoma; however, chemotherapy and/or radiotherapy appears to significantly inhibit their upregulation.
评估肝素酶-1 及刺猬信号通路在肺泡眼眶横纹肌肉瘤中的作用。
将 23 例标本分为术前放化疗组和未治疗组，6 例正常眼外肌标本作为正常肌肉组。采用免疫组化方法检测 23 例存档石蜡包埋肺泡眼眶横纹肌肉瘤中肝素酶-1、 patched 、 smoothened 和 glioma-associated oncogene homologue-1 蛋白的表达情况。提取三组石蜡包埋标本的 RNA，采用巢式逆转录聚合酶链反应和限制稀释分析比较肝素酶-1 、 smoothened 和 glioma-associated oncogene homologue-1 的信使 RNA 表达。
肝素酶-1 、 patched 、 smoothened 和 glioma-associated oncogene homologue-1 蛋白在 91.3%、87.0%、91.3%和 78.3%的肺泡眼眶横纹肌肉瘤标本中表达。未治疗的横纹肌肉瘤标本染色强度较高，但化疗组组织染色较弱。肝素酶-1 、 smoothened 和 glioma-associated oncogene homologue-1 信使 RNA 的表达水平在未治疗组和化疗组与免疫组化结果一致，化疗组与正常肌肉组之间肝素酶-1 、 smoothened 和 glioma-associated oncogene homologue-1 信使 RNA 水平无显著差异（P > 0.05）。然而，未治疗组的信使 RNA 均明显高于化疗组和正常肌肉组（P < 0.01）。
肝素酶-1 和刺猬信号通路均参与肺泡眼眶横纹肌肉瘤的发病机制，化疗和/或放疗可显著抑制其上调。
