Mica Ladislav, Gianom Duri, Bode Beata, Jaklin Peter, Hollinger Albert
Department of Surgery, University Hospital of Zürich, Zürich, Switzerland.
Case Rep Gastroenterol. 2007 Jun 20;1(1):7-14. doi: 10.1159/000104225.
Liposarcoma represents one of the most frequent (10-20%) malignant mesenchymal tumors in the adult, affecting mostly the soft tissue of extremities, the trunk or the retroperitoneum. This tumor type occurs exceptionally rarely in the gastrointestinal tract with only few cases described in the literature. In this case we present a 73-year-old male patient who was admitted due to loss of weight, anorexia and postprandial emesis with dysphagy. Gastrographin esophagography failed to make precise diagnostics. CT scan of the upper gastrointestinal tract revealed a large esophageal tumor filling out the whole length of the esophagus. The tumor was removed by parasternocleidomastoidal approach with a stapler. Histopathological examination revealed a well-differentiated liposarcoma (grade I). Well-differentiated liposarcomas are characterised by amplified material of the 12q13-15 chromosomal region, present in the form of giant or ring chromosomes and leading to the overexpression of MDM2 and CDK4 genes. MDM2 and CDK4 proteins can be detected immunhistochemically, which was the case in the reported tumor. Overexpression of these proteins leads to suppression of tumor suppressor genes, leading to increased cell survival.
脂肪肉瘤是成人中最常见的（10 - 20%）恶性间充质肿瘤之一，主要累及四肢、躯干或腹膜后的软组织。这种肿瘤类型在胃肠道极为罕见，文献中仅有少数病例报道。在此病例中，我们介绍一位73岁男性患者，因体重减轻、厌食、餐后呕吐伴吞咽困难入院。泛影葡胺食管造影未能做出准确诊断。上消化道CT扫描显示一个巨大的食管肿瘤，占据食管全长。通过胸锁乳突肌旁入路用吻合器切除了肿瘤。组织病理学检查显示为高分化脂肪肉瘤（I级）。高分化脂肪肉瘤的特征是12q13 - 15染色体区域的物质扩增，以巨大染色体或环状染色体的形式存在，导致MDM2和CDK4基因过表达。MDM2和CDK4蛋白可通过免疫组织化学检测到，本报道的肿瘤即是如此。这些蛋白的过表达导致肿瘤抑制基因的抑制，从而增加细胞存活率。
