A broad ligament solitary fibrous tumor with Doege-Potter syndrome

Medicine (Baltimore). 2018 Sep;97(39):e12564. doi: 10.1097/MD.0000000000012564.

Abstract

Introduction: 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.

Patient concerns: A 37-year-old woman who presented with life-threatening hypoglycemia and a pelvic mass (16 × 15 × 15 cm).

Diagnoses: The patient was diagnosed with broad ligament SFT with Doege-Potter syndrome.

Interventions: Tumor resection, sub-extensive hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy were performed, and 6 cycles of adjuvant chemotherapy were administered.

Outcomes: 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.

Conclusion: 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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adnexal Diseases / complications*
  • Adnexal Diseases / surgery
  • Adult
  • Broad Ligament* / surgery
  • Chemotherapy, Adjuvant
  • Female
  • Humans
  • Hypoglycemia / complications*
  • Hysterectomy
  • Lymph Node Excision
  • Ovariectomy
  • Paraneoplastic Syndromes / complications*
  • Salpingectomy
  • Solitary Fibrous Tumors / complications*
  • Solitary Fibrous Tumors / surgery