Coffin C M, Dehner L P
Department of Pathology, University of Utah Health Science Center, and Primary Children's Medical Center, Salt Lake City 84113, USA.
Am J Clin Pathol. 1998 Apr;109(4 Suppl 1):S38-52.
The subject of soft tissue tumors in children has some parallels with the same topic in adults, but many of the details clearly establish the existence of distinct differences. Most soft tissue tumors in adults arise in the extremities or in the superficial and deep soft tissues elsewhere in the body, and most tumors are neoplastic, whether they are benign or malignant. In children, some of the most common soft tissue tumors may not be neoplastic at all, but rather are malformations of the supporting mesenchymal tissues. There is a common classification of soft tissue tumors that is applicable to both pediatric- and adult-type neoplasms. Although some of the "adult"-type soft tissue tumors may occur in children, the reverse situation is very uncommon. Because pediatric soft tissue tumors are not seen with any regularity by many pathologists in a practice dominated by adult cases, there is less familiarity and more uncertainty about these tumors in children. The differential diagnosis of a malignant soft tissue tumor in a child encompasses the broad morphologic spectrum of spindle cells, round cells, or combined-pattern neoplasms. The diagnostic process is complex and may necessitate an array of ancillary studies, including immunohistochemistry, electron microscopy, and molecular diagnostics. After a decision is reached to excise a soft tissue sarcoma in a child, many of the steps in the examination of the specimen and the reporting of results for diagnostic and staging purposes are similar to those in the evaluation of a soft tissue sarcoma in an adult. However, it is important for the pathologist and other members of the multidisciplinary health care team to remember that a soft tissue tumor in a child may have genetic implications that directly influence the care of the patient and the family and for which the excised tissue offers a potentially invaluable resource for future studies.
儿童软组织肿瘤这一主题与成人的同一主题有一些相似之处，但许多细节清楚地表明存在明显差异。成人的大多数软组织肿瘤发生在四肢或身体其他部位的浅表和深部软组织中，并且大多数肿瘤是肿瘤性的，无论它们是良性还是恶性。在儿童中，一些最常见的软组织肿瘤可能根本不是肿瘤性的，而是支持性间充质组织的畸形。有一种适用于儿童和成人类型肿瘤的软组织肿瘤通用分类。虽然一些“成人”型软组织肿瘤可能发生在儿童中，但相反的情况非常罕见。由于在以成人病例为主的实践中，许多病理学家并不经常见到儿童软组织肿瘤，因此对这些儿童肿瘤的了解较少且不确定性更大。儿童恶性软组织肿瘤的鉴别诊断包括梭形细胞、圆形细胞或混合模式肿瘤的广泛形态学谱。诊断过程很复杂，可能需要一系列辅助研究，包括免疫组织化学、电子显微镜和分子诊断。在决定切除儿童的软组织肉瘤后，用于诊断和分期目的的标本检查和结果报告中的许多步骤与成人软组织肉瘤评估中的步骤相似。然而，病理学家和多学科医疗团队的其他成员必须记住，儿童的软组织肿瘤可能具有遗传意义，直接影响患者及其家庭的护理，并且切除的组织为未来研究提供了潜在的宝贵资源。
