Chen Sijing, Zheng Ying, Chen Lin, Yi Qihua
Department of Gynecologic Oncology, West China Second Hospital, Sichuan University.
Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, China.
Medicine (Baltimore). 2018 Sep;97(39):e12564. doi: 10.1097/MD.0000000000012564.
Solitary fibrous tumors (SFTs) are uncommon mesenchymal neoplasms and are particularly rare in the female genital tract. Doege-Potter syndrome is a paraneoplastic syndrome involving SFT-associated hypoglycemia. We report, for the first time, on a broad ligament SFT with Doege-Potter syndrome; additionally, we review 30 cases of women with SFTs reported in the literature.
A 37-year-old woman who presented with life-threatening hypoglycemia and a pelvic mass (16 × 15 × 15 cm).
The patient was diagnosed with broad ligament SFT with Doege-Potter syndrome.
Tumor resection, sub-extensive hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy were performed, and 6 cycles of adjuvant chemotherapy were administered.
Serum glucose levels returned to normal as soon as the tumor was resected. Forty-3 months after operation, there was recurrence in the posterior peritoneal tissues. She underwent tumor resection and has remained tumor-free 28 months after this excision.
Even though it is extremely rare, SFT should be quickly identified to prevent undue treatment delay and avoid unnecessary examination; surgery and long-term follow-up are recommended. SFT can be considered a highly invasive cancer, and intraoperative bleeding may occur. Although no correlation between adjuvant therapy and improved prognosis was found, further studies are required because of the small number of cases reported to date.
孤立性纤维瘤(SFTs)是一种罕见的间叶组织肿瘤,在女性生殖道中尤为罕见。多伊格 - 波特综合征是一种与SFT相关的低血糖副肿瘤综合征。我们首次报告了一例伴有多伊格 - 波特综合征的阔韧带SFT;此外,我们回顾了文献中报道的30例患有SFT的女性病例。
一名37岁女性,出现危及生命的低血糖和盆腔肿块(16×15×15cm)。
患者被诊断为伴有多伊格 - 波特综合征的阔韧带SFT。
进行了肿瘤切除术、次广泛子宫切除术、双侧输卵管卵巢切除术和盆腔淋巴结清扫术,并给予6个周期的辅助化疗。
肿瘤切除后血糖水平立即恢复正常。术后43个月,后腹膜组织出现复发。她接受了肿瘤切除术,此次切除术后28个月一直无肿瘤复发。
尽管SFT极为罕见,但应迅速识别以防止治疗延误并避免不必要的检查;建议进行手术和长期随访。SFT可被视为一种高侵袭性癌症,术中可能发生出血。尽管未发现辅助治疗与改善预后之间存在相关性,但由于迄今为止报道的病例数量较少,仍需要进一步研究。