Greenson Joel K
Department of Pathology, University of Michigan Health System, Ann Arbor, Michigan 48109-0054, USA.
Mod Pathol. 2003 Apr;16(4):366-75. doi: 10.1097/01.MP.0000062860.60390.C7.
In the past 5 years, there has been a paradigm shift in our understanding of gastrointestinal stromal tumors (GISTs). Once thought to be smooth muscle tumors, these uncommon neoplasms are now thought to differentiate along the lines of interstitial cells of Cajal, the pacemaker cells of the gut. Along with this understanding comes an exciting new drug therapy (Gleevec) that for the first time offers real hope to patients with malignant stromal tumors. Overall, approximately 60-70% of stromal tumors are from the stomach, 20-30% are from the small intestine, and <10% come from the esophagus, colon, rectum, omentum, and mesentery. Between 10 and 30% of GISTs are malignant. Stromal tumors should be studied in a site-specific fashion, as tumors from a given location in the gut have unique growth patterns and corresponding behaviors. Although the most important tool needed to diagnose a GIST is still a hematoxylin and eosin-stained section, a confirmatory CD117 stain is recommended (and may be required for drug therapy). True smooth muscle tumors, inflammatory fibroid polyps, fibromatoses, schwannomas, inflammatory myofibroblastic tumors, and solitary fibrous tumors all enter into the differential diagnosis of GISTs. This article reviews the histologic features of these tumors in the context of recent molecular genetic and immunohistochemical advances.
在过去5年里，我们对胃肠道间质瘤（GISTs）的认识发生了范式转变。这些罕见的肿瘤曾经被认为是平滑肌肿瘤，现在则被认为是沿着肠道起搏细胞—— Cajal间质细胞的方向分化。随着这种认识的出现，一种令人兴奋的新药物疗法（格列卫）首次为恶性间质瘤患者带来了真正的希望。总体而言，大约60 - 70%的间质瘤来自胃，20 - 30%来自小肠，<10%来自食管、结肠、直肠、网膜和肠系膜。10%至30%的GISTs是恶性的。间质瘤应以特定部位的方式进行研究，因为来自肠道特定位置的肿瘤具有独特的生长模式和相应的行为。虽然诊断GIST所需的最重要工具仍然是苏木精和伊红染色切片，但建议进行CD117染色以作确认（药物治疗可能需要）。真正的平滑肌肿瘤、炎性纤维瘤性息肉、纤维瘤病、神经鞘瘤、炎性肌纤维母细胞瘤和孤立性纤维瘤都需列入GISTs的鉴别诊断。本文结合近期分子遗传学和免疫组织化学进展，综述了这些肿瘤的组织学特征。
