Abstract：
Most soft tissue sarcomas afflict the extremities; however, the retro peritoneum can also be affected rarely. Retroperitoneal sarcomas are relatively asymptomatic. Although tumor-induced hypoglycemia is rare in tumors other than insulinomas, extrapancreatic tumors are a subset that displays this phenomenon. The occurrence of hypo-insulinemic hypoglycemia with low GH and IGF-1 should prompt consideration of the secretion of a hypoglycemic substance impeding the secretion of insulin and GH, such as IGF-2 or one of its related substances. The present case report is of a 38-year-old male with retroperitoneal round cell sarcoma with liver metastasis with severe symptomatic hypoglycemia who was managed with multipronged symptomatic therapy and oncological management after which he had shown significant improvement in hypoglycemic episodes and symptom profile. A literature review revealed our case report to be the first reported case of a young male (preponderance in the older population) with hypoglycemia associated with retroperitoneal sarcoma which presented with liver metastasis and the only one treated with Gemcitabine /Docetaxel. The presence of these features might point toward a poorer prognosis in a disease with an already dismal course. All these points towards the need for further research regarding intensified oncological treatment after evidence-based prognostication of high-risk groups and modalities for the management of symptomatic hypoglycemia such as Somatostatin analogs and glucagon which aid in symptom control.
摘要：
大多数软组织肉瘤影响四肢；然而，腹膜后也很少受到影响。腹膜后肉瘤相对无症状。尽管肿瘤引起的低血糖在胰岛素瘤以外的肿瘤中很少见，胰腺外肿瘤是表现出这种现象的一个子集。低GH和IGF-1的低胰岛素血症性低血糖的发生应促使人们考虑分泌阻碍胰岛素和GH分泌的降血糖物质，如IGF-2或其相关物质之一。本病例报告是一名38岁的男性，患有腹膜后圆形细胞肉瘤，肝转移并伴有严重的症状性低血糖，他接受了多管齐下的对症治疗和肿瘤治疗，此后他在低血糖发作和症状方面表现出显着改善。文献综述显示，我们的病例报告是首例报道的年轻男性（在老年人群中占优势）与腹膜后肉瘤相关的低血糖并伴有肝转移，并且是唯一接受吉西他滨/多西他赛治疗的病例。这些特征的存在可能表明在已经令人沮丧的疾病中预后较差。所有这些都表明，在对高危人群进行循证预后后，需要进一步研究强化肿瘤治疗，以及治疗症状性低血糖的方式，例如生长抑素类似物和胰高血糖素，这有助于症状控制。
