Department of Surgery, Takeda General Hospital, Kyoto, Japan.
Diabetic Center, National Hospital Organization Kyoto Medical Center, Kyoto, Japan.
Am J Case Rep. 2022 Oct 13;23:e936806. doi: 10.12659/AJCR.936806.
BACKGROUND Solitary fibrous tumors (SFT), rare soft-tissue neoplasms, are usually found in the thoracic cavity, and a uterine origin is extremely rare. SFTs with insulin-like growth factor-II (IGF-II) production induce non-islet cell tumor-induced hypoglycemia (NICTH), referred to as Doege-Potter syndrome. CASE REPORT A 70-year-old woman presented with urinary retention, and imaging revealed a huge mass occupying almost the entire pelvic space. She had a history of hysterectomy for leiomyoma of the uterus 7 years earlier. In her present course, she developed hypoglycemia, and NICTH was suspected. Her previous uterine specimen was reexamined, and immunohistochemistry (IHC) revealed the specimen to be CD34-positive and alpha-smooth muscle actin-negative, indicating that the uterine specimen was not leiomyoma but SFT. Therefore, the present pelvic tumor was considered to be a recurrence of SFT with NICTH, namely Doege-Potter syndrome. Surgical resection was performed, and the pathological examination showed the same histologic features as the previous uterine specimen, while IHC revealed the present specimen to be positive for CD34, signal transducers and activator of transcription 6, and IGF-II, consistent with the diagnosis of recurrent SFT with IGF-II production. The patient's hypoglycemia improved after tumor resection. To confirm the IGF-II secretion from the SFT, we conducted immunoblotting of the patient's perioperative serum, with results showing that the strong band of IGF-II in the preoperative serum disappeared after surgery. CONCLUSIONS Because SFTs, especially those with Doege-Potter syndrome, often recur, sometimes with a very long interval, long-term cautious surveillance is required, even after complete tumor resection.
孤立性纤维瘤（SFT）是一种罕见的软组织肿瘤，通常发生在胸腔，而子宫来源的则极为罕见。具有胰岛素样生长因子-II（IGF-II）产生的 SFT 可引起非胰岛细胞瘤性低血糖（NICTH），称为 Doege-Potter 综合征。
一名 70 岁女性因尿潴留就诊，影像学检查显示一个巨大的肿块几乎占据了整个盆腔空间。她 7 年前因子宫平滑肌瘤行子宫切除术。在目前的病程中，她出现低血糖，疑似 NICTH。重新检查她之前的子宫标本，免疫组织化学（IHC）显示标本 CD34 阳性，α-平滑肌肌动蛋白阴性，表明子宫标本不是平滑肌瘤，而是 SFT。因此，目前的盆腔肿瘤被认为是具有 NICTH 的 SFT 复发，即 Doege-Potter 综合征。进行了手术切除，病理检查显示与之前的子宫标本具有相同的组织学特征，而 IHC 显示目前的标本 CD34、信号转导子和转录激活子 6 和 IGF-II 阳性，符合具有 IGF-II 产生的复发性 SFT 的诊断。肿瘤切除后，患者的低血糖得到改善。为了确认 SFT 分泌 IGF-II，我们对患者围手术期的血清进行了免疫印迹，结果显示术前血清中 IGF-II 的强带在手术后消失。
由于 SFT，尤其是具有 Doege-Potter 综合征的 SFT，经常复发，有时间隔很长，即使在完全切除肿瘤后，也需要长期谨慎监测。
