Background Dedifferentiated liposarcoma is an uncommon variant of liposarcoma, with poor prognosis and higher preponderance to local recurrence. Only nine cases of dedifferentiated liposarcoma of small bowel mesentery have been reported till now. This is a case of giant dedifferentiated liposarcoma of the small bowel mesentery, weighing nearly 9?kg (19.8?lbs), with synchronous lesions in the extraperitoneal space, which is the first such case to be reported. Case presentation We report a case of a 62-year-old man, who presented with a huge abdominal mass occupying nearly the entire abdomen. A contrast enhanced computed tomography of abdomen and pelvis revealed a large, poorly enhancing, heterogeneous, lobulated mass of size 27?×?16?cm, displacing the bowel loops peripherally. At laparotomy, a large mass arising from the small bowel mesentery was found. In addition, many other smaller synchronous lesions were studded in the entire small bowel mesentery and a couple more in the extraperitoneal space. A palliative excision of the giant mass along with the adjacent small bowel was done. The other smaller swellings were not causing any mass effect and were left behind as they were numerous, virtually ruling out any possibility of a curative excision. The histopathological examination suggested the diagnosis of dedifferentiated liposarcoma. On immunohistochemistry, S-100 was positive in the well-differentiated sarcomatous areas. The CD 117 and SMA were strongly negative ruling out the possibility of a gastrointestinal stromal tumour. The CD 34 however was positive in the tumour cells. Conclusions Dedifferentiated liposarcoma of the small bowel mesentery is rare. Involvement of nearly whole of the small bowel mesentery in the disease process virtually rules out the possibility of a curative resection, the mainstay of management. This report would add to the knowledge of this rare disease and the possible therapeutic problem that may be encountered in case of multifocal disease.
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机译：背景去分化脂肪肉瘤是脂肪肉瘤的罕见变体，预后较差，局部复发率较高。迄今为止，仅报道了9例小肠系膜去分化脂肪肉瘤。这是一例小肠系膜的巨大去分化脂肪肉瘤，重近9千克（19.8磅），在腹膜外间隙有同步性病变，这是首次报道。病例介绍我们报告了一例62岁男子的病例，他的腹部巨大肿块几乎占据了整个腹部。腹部和骨盆的对比增强计算机断层扫描显示，大小不等的异质叶状肿块较大，大小为27？×？16？cm，在周边移位了肠loop。在剖腹手术中，发现了由小肠系膜引起的巨大肿物。此外，在整个小肠系膜中还散布着许多其他较小的同步病变，在腹膜外间隙中还散布着许多其他病变。姑息性切除了巨大肿块以及邻近的小肠。其他较小的肿胀并没有引起任何肿块效应，而是由于数量众多而被抛在后面，实际上排除了治愈性切除的任何可能性。组织病理学检查提示诊断为未分化脂肪肉瘤。在免疫组织化学上，S-100在分化良好的肉瘤区域呈阳性。 CD 117和SMA强烈否定了胃肠道间质瘤的可能性。然而，CD 34在肿瘤细胞中是阳性的。结论小肠系膜的去分化脂肪肉瘤少见。几乎整个小肠系膜均参与疾病过程，实际上排除了根治性切除的可能性，而根除性切除是治疗的主要手段。该报告将增加对这种罕见疾病的了解，以及在多灶性疾病情况下可能遇到的治疗问题。
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