Strâmbu Irina Ruxandra, Leonte Diana Gabriela, Bolca Ciprian Nicolae
Department of Pulmonology, "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania;
Rom J Morphol Embryol. 2017;58(1):287-291.
Doege-Potter syndrome is a rare condition consisting of a mesenchymal tumor, either benign or malignant, accompanied by severe hypoglycemia. The syndrome was first described independently by two American physicians, Karl Walter Doege (1867-1932) and Roy Pilling Potter (1879-1968), in 1930, but it was not before 1988 that it was associated with non-islet cell tumor production of insulin growth factor (IGF) that induces hypoglycemia as a paraneoplastic syndrome.
We present the case of a 61-year-old woman with severe hypoglycemia that induced seizures. On the general check-up, a massive tumor occupying the lower part of left hemi-thorax was discovered. Initially, corticosteroids, glucose i.v. and high carbohydrate diet managed to prevent the severe blood glucose drop. Surgery exposed a massive well-defined pleural tumor. After surgical removal, blood glucose stabilized. Histological examination confirmed the fibrous tumor that proved to be malignant on immunochemistry.
The authors discuss other cases reported in the literature of this rare condition and its pathogenic mechanisms, the presented case being the first reported in Romania.
The clinician should be aware of the possible existence of a pleural tumor in a patient presenting an unexplained hypoglycemia because the surgical removal of the tumor can solve the clinical manifestations.
多伊格 - 波特综合征是一种罕见病症，由良性或恶性间充质肿瘤伴严重低血糖组成。该综合征于1930年由两位美国医生卡尔·沃尔特·多伊格（1867 - 1932）和罗伊·皮林·波特（1879 - 1968）首次独立描述，但直到1988年才发现它与非胰岛细胞瘤产生胰岛素样生长因子（IGF）有关，该因子作为一种副肿瘤综合征可诱发低血糖。
我们报告一例61岁女性患者，因严重低血糖诱发癫痫发作。在全面检查中，发现一个巨大肿瘤占据左半胸下部。最初，使用皮质类固醇、静脉注射葡萄糖和高碳水化合物饮食成功预防了严重血糖下降。手术中发现一个巨大的边界清晰的胸膜肿瘤。手术切除后，血糖稳定。组织学检查证实为纤维瘤，免疫化学检查显示为恶性。
作者讨论了文献中报道的关于这种罕见病症的其他病例及其发病机制，本病例是罗马尼亚首次报道。
临床医生应意识到，对于出现不明原因低血糖的患者，可能存在胸膜肿瘤，因为手术切除肿瘤可解决临床表现。
