Department of Radiation Oncology, AIIMS, Jodhpur, Rajasthan, India.
Department of Radiation Oncology, AIIMS, Jodhpur, Rajasthan, India.
Curr Probl Cancer. 2023 Jun;47(3):100961. doi: 10.1016/j.currproblcancer.2023.100961. Epub 2023 May 14.
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.
大多数软组织肉瘤发生在四肢;然而,腹膜后腔也很少受到影响。腹膜后肉瘤相对无症状。尽管肿瘤诱导性低血糖在胰岛素瘤以外的肿瘤中很少见,但胰外肿瘤是表现出这种现象的一个亚组。低 GH 和 IGF-1 伴低胰岛素血症低血糖症的发生应促使考虑分泌一种抑制胰岛素和 GH 分泌的低血糖物质,例如 IGF-2 或其相关物质之一。本病例报告为一名 38 岁男性,患有腹膜后圆形细胞肉瘤伴肝转移,伴有严重症状性低血糖症,采用多管齐下的对症治疗和肿瘤学管理,此后他在低血糖发作和症状谱方面有了显著改善。文献复习显示,我们的病例报告是首例报道的与腹膜后肉瘤相关的年轻男性(多见于老年人群)发生低血糖症的病例,该患者表现为肝转移,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预后不良的疾病中预后更差。所有这些都表明需要进一步研究强化肿瘤治疗,对高危人群进行基于证据的预后评估,并对症状性低血糖症的管理方法进行研究,例如生长抑素类似物和胰高血糖素,这些方法有助于控制症状。