Department of Internal Medicine, Hospital Santa Maria degli Angeli, Pordenone, Italy.
Department of Endocrinology and Metabolic Diseases, Hospital Santa Maria degli Angeli, Pordenone, Italy.
Endocr Metab Immune Disord Drug Targets. 2023;23(12):1562-1569. doi: 10.2174/1871530323666230623112047.
Doege-Potter syndrome is a rare paraneoplastic entity that is often diagnosed incidentally during the work-up of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia mostly associated with solitary fibrous tumors. These uncommon tumors have been reported in <5% of solitary fibrous tumors. Although not unique in its kind, this case is extremely important as this syndrome often conceals unrecognized tumors that can be surgically resolved.
We present the case of a 59-year-old non-diabetic man with a 2-month history of severe and recurrent fasting hypoglycaemia presenting with severe dyspnea and sweating. Further workup revealed low insulin, C-peptide, and IGF-1 levels and a large right in-trathoracic solitary fibrous tumor. Unfortunately, bioassays for IGF-2 were unavailable at our hos-pital. Nevertheless, as hypoglycemia completely resolved after resection of the mass, Doege-Potter syndrome was highly suspected.
Doege-Potter syndrome is a complication of rare tumors. If hy-poglycemia is unexplained, this syndrome should always be suspected, and the presence of un-known masses should be investigated.
Doege-Potter 综合征是一种罕见的副肿瘤实体，通常在不明病因低血糖的检查过程中偶然诊断。其特征为非胰岛细胞瘤性低血糖，主要与孤立性纤维瘤相关。这些罕见的肿瘤在<5%的孤立性纤维瘤中被报道。虽然这种情况并不独特，但这个病例非常重要，因为这种综合征常常掩盖了未被识别的肿瘤，这些肿瘤可以通过手术解决。
我们介绍了一例 59 岁非糖尿病男性患者，他有 2 个月的严重和反复空腹低血糖史，表现为严重呼吸困难和出汗。进一步检查显示胰岛素、C 肽和 IGF-1 水平低，并且右侧胸腔内有一个大的孤立性纤维瘤。不幸的是，我们医院没有 IGF-2 的生物测定。然而，由于切除肿块后低血糖完全缓解，高度怀疑 Doege-Potter 综合征。
Doege-Potter 综合征是罕见肿瘤的并发症。如果低血糖原因不明，应始终怀疑这种综合征，并应调查是否存在未知肿块。
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