Wada Yukiko, Okano Keiichi, Ando Yasuhisa, Uemura Jun, Suto Hironobu, Asano Eisuke, Kishino Takayoshi, Oshima Minoru, Kumamoto Kensuke, Usuki Hisashi, Suzuki Yasuyuki
Department of Gastroenterological Surgery, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan.
Surg Case Rep. 2019 Apr 11;5(1):60. doi: 10.1186/s40792-019-0617-6.
A solitary fibrous tumor (SFT) is a mesenchymal lesion, which commonly develops in the thorax. Non-islet cell hypoglycemia is a rare paraneoplastic phenomenon caused by an extra-pancreatic tumor. We report a rare case of a pelvic SFT with severe hypoglycemia, which was considered to be Doege-Potter syndrome.
A 72-year-old man was referred to our hospital for treatment of hypoglycemia and a large pelvic tumor. His blood glucose level was 52 mg/dl; serum insulin level, 1.0 μIU/ml; C-peptide level, 0.2 ng/ml; and insulin-like growth factor-I (IGF-I) level, 31 ng/ml. Contrast-enhanced computed tomography (CT) showed a 13-cm mass in the pelvic cavity. Magnetic resonance imaging (MRI) revealed a lobulated tumor with iso- and high-intensity areas combined in T2-weighted images. No clear invasion to any adjacent organs was identified. The tumor was resected, and hypoglycemic symptoms disappeared immediately. Pathological diagnosis was an SFT with malignant potential that secreted IGF-II and caused hypoglycemia. There has been no tumor recurrence during the 1 year of follow-up.
Non-islet cell tumor hypoglycemia should be considered in the differential diagnosis of patients presenting with tumors and hypoglycemia.
孤立性纤维瘤(SFT)是一种间叶性病变,通常发生于胸部。非胰岛细胞瘤性低血糖症是一种由胰腺外肿瘤引起的罕见副肿瘤现象。我们报告一例罕见的伴有严重低血糖症的盆腔SFT病例,该病例被认为是多伊格 - 波特综合征。
一名72岁男性因低血糖症和盆腔巨大肿瘤被转诊至我院。他的血糖水平为52mg/dl;血清胰岛素水平为1.0μIU/ml;C肽水平为0.2ng/ml;胰岛素样生长因子 - I(IGF - I)水平为31ng/ml。增强计算机断层扫描(CT)显示盆腔内有一个13厘米的肿块。磁共振成像(MRI)在T2加权图像上显示为一个分叶状肿瘤,有等强度和高强度区域。未发现对任何相邻器官有明确侵犯。肿瘤被切除,低血糖症状立即消失。病理诊断为具有恶性潜能的SFT,其分泌IGF - II并导致低血糖症。在1年的随访期间未出现肿瘤复发。
对于伴有肿瘤和低血糖症的患者,鉴别诊断时应考虑非胰岛细胞瘤性低血糖症。