Herrmann B L, Saller B, Kiess W, Morgenroth K, Drochner K, Schröder T, Mann K
Department of Medicine, University of Essen, Germany.
Exp Clin Endocrinol Diabetes. 2000;108(8):515-8. doi: 10.1055/s-2000-11007.
Augmented glucose utilisation or secretion of insulin-like-growth-factor II (IGF-II) are discussed as important pathogenetic factors in tumor-associated hypoglycemia (Doege-Potter Syndrome) with suppressed insulin and C-peptide levels. Primary malignant fibrous histiocytoma of the lung is an uncommon neoplasia and its association with hypoglycemia is rare and the causal relationship remains unclear. - We report a 57-year-old male with spontaneous hypoglycemia (1.67 mmol/l) due to a primary malignant fibrous histiocytoma of the lung, secreting IGF-II. Insulin (0.10 nmol/l; normal range 0.33-1.2) and C-peptide (3.0 mIU/l; 5-25) levels were suppressed in combination with low levels of growth hormone (<0.5 ng/ml; <7 ng/ml) and IGF-I (<66.0 ng/ml; 70-246). The elevated IGF-II level (787 ng/ml; 300-500) and decreased IGF-binding protein 3 (1.6 mg/l; 2-5) indicated a high free IGF-II activity. After surgery (resection of the right upper lobe), glucose (4.4 mmol/l), insulin (9.0 mIU/L) and C-peptide (0.84 nmol/l) levels returned to normal. Serum IGF-I (289 ng/ml) and the IGF-I/IGF-II ratio (<0.08 preoperative vs. 0.41 postoperative; >0.20) increased to the normal reference range. - In conclusion, malignant fibrous histiocytoma (MFH) is rarely described presenting as tumor-induced hypoglycemia. Doege-Potter Syndrome in MFH seems to be related to tumor-associated IGF-II production.
在胰岛素和C肽水平受抑制的肿瘤相关性低血糖症（多伊格-波特综合征）中，葡萄糖利用增加或胰岛素样生长因子II（IGF-II）分泌增加被认为是重要的致病因素。肺原发性恶性纤维组织细胞瘤是一种罕见的肿瘤，其与低血糖症的关联很少见，因果关系仍不清楚。——我们报告一例57岁男性，因肺原发性恶性纤维组织细胞瘤分泌IGF-II而出现自发性低血糖（1.67 mmol/l）。胰岛素（0.10 nmol/l；正常范围0.33 - 1.2）和C肽（3.0 mIU/l；5 - 25）水平受抑制，同时生长激素水平低（<0.5 ng/ml；<7 ng/ml）和IGF-I水平低（<66.0 ng/ml；70 - 246）。IGF-II水平升高（787 ng/ml；300 - 500）和IGF结合蛋白3水平降低（1.6 mg/l；2 - 5）表明游离IGF-II活性高。手术后（右上叶切除），血糖（4.4 mmol/l）、胰岛素（9.0 mIU/L）和C肽（0.84 nmol/l）水平恢复正常。血清IGF-I（289 ng/ml）和IGF-I/IGF-II比值（术前<0.08 vs. 术后0.41；>0.20）升至正常参考范围。——总之，恶性纤维组织细胞瘤（MFH）很少被描述为肿瘤诱导的低血糖症。MFH中的多伊格-波特综合征似乎与肿瘤相关的IGF-II产生有关。
