Hall King-Fair, Lin Chia-Li, Wang Tsung-Hsi, Chang Rung-Ho, Chen Han-Ming
Department of Neurology, Ten-Chan General Hospital, Jhongli City, Taoyuan, Taiwan, ROC.
Chang Gung Med J. 2008 Jan-Feb;31(1):107-11.
Gastrointestinal stromal tumor (GIST) is an uncommon malignant tumor that has recently been drawn to the attention of clinicians because of the protean clinical spectrum, endocrinological pathogenesis, aggressive metastatic features, poor prognosis, oncogenic tyrosine kinase receptor mutation and its brilliant revolutionized inhibitor imatinib. However, very few cases of GIST and hypoglycemia have been reported worldwide and they are usually associated with normal or low insulin levels. Here, we report on a rare case of GIST with postabsorptive hypoglycemia, extraordinary hyperinsulinemia and low insulinlike growth factor I (IGF-I). Its unusual neurological presentation made its diagnosis very difficult. After a complete resection, the symptoms diminished. We point out the unusual endogenous hyperinsulinism, clinical features and postulate possible mechanisms.
胃肠道间质瘤(GIST)是一种罕见的恶性肿瘤,因其多样的临床谱、内分泌发病机制、侵袭性转移特征、预后不良、致癌性酪氨酸激酶受体突变以及其具有开创性变革意义的抑制剂伊马替尼,最近受到了临床医生的关注。然而,全球范围内报道的GIST合并低血糖的病例非常少,且通常与胰岛素水平正常或降低有关。在此,我们报告一例罕见的GIST病例,该病例出现吸收后低血糖、异常高胰岛素血症和低胰岛素样生长因子I(IGF-I)水平。其不寻常的神经学表现使其诊断极具难度。完整切除术后,症状有所减轻。我们指出了这种不寻常的内源性高胰岛素血症、临床特征,并推测了可能的机制。