Parham D M, Kelly D R, Donnelly W H, Douglass E C
Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.
Mod Pathol. 1991 Sep;4(5):648-53.
Hepatic sarcomas of childhood, which appear heterogeneous by standard morphologic criteria, pose challenging diagnostic and nosologic problems to the pediatric surgical pathologist. To identify features of these tumors that might help to clarify their origin and histologic classification, we studied 13 undifferentiated (embryonal) sarcomas of the liver (UESL) and two intrinsic hepatic rhabdomyosarcomas (RMS) using immunohistochemical and electron microscopic techniques. Immunohistochemical staining was performed on 14 tumors with use of commercially available antibodies against a variety of markers, as well as peanut agglutinin lectin; electron microscopy was performed on five UESL and both RMS. Desmin was expressed by 6/12 UESL and 2/2 RMS, muscle-specific actin by 5/12 UESL and 2/2 RMS, neuron-specific enolase by 1/12 UESL and 1/2 RMS, alpha-1-antitrypsin by 8/12 UESL and 1/2 RMS, and alpha-1-antichymotrypsin by 10/12 UESL and 1/2 RMS. Cytokeratin expression was observed in only four UESL. The overlap of immunohistochemical staining patterns and ultrastructural features shown by these obstensibly different tumors suggests a common histogenesis, perhaps from a multipotential mesenchymal stem cell.
儿童肝肉瘤按标准形态学标准表现出异质性，给儿科外科病理学家带来了具有挑战性的诊断和分类问题。为了确定这些肿瘤可能有助于阐明其起源和组织学分类的特征，我们使用免疫组织化学和电子显微镜技术研究了13例肝未分化（胚胎性）肉瘤（UESL）和2例原发性肝横纹肌肉瘤（RMS）。对14例肿瘤进行了免疫组织化学染色，使用了针对多种标志物的市售抗体以及花生凝集素；对5例UESL和2例RMS进行了电子显微镜检查。12例UESL中的6例和2例RMS表达结蛋白，12例UESL中的5例和2例RMS表达肌肉特异性肌动蛋白，12例UESL中的1例和2例RMS中的1例表达神经元特异性烯醇化酶，12例UESL中的8例和2例RMS中的1例表达α1抗胰蛋白酶，12例UESL中的10例和2例RMS中的1例表达α1抗糜蛋白酶。仅在4例UESL中观察到细胞角蛋白表达。这些明显不同的肿瘤所显示的免疫组织化学染色模式和超微结构特征的重叠提示了共同的组织发生，可能源自多能间充质干细胞。
