Xinji Yang, Lei Liu, Xiaofei Hou
Background Pelvic solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm with marked clinical and radiological heterogeneity, posing significant diagnostic challenges. Standardized management strategies remain lacking for special clinical scenarios, including paraneoplastic syndromes, synchronous primary malignancies, and pregnancy-associated incidental diagnosis. Methods This single-center retrospective case series included 3 consecutive patients with histologically confirmed pelvic SFT who underwent surgical resection at Peking University Third Hospital between October 2018 and September 2025. Clinical, radiological, surgical, pathological, and long-term follow-up data were systematically collected, and a narrative literature review was conducted to refine clinical management. Results The three patients presented with distinct rare phenotypes: a 17.5 cm giant tumor complicated by Doege-Potter syndrome, synchronous low-grade bladder urothelial carcinoma, and an asymptomatic tumor incidentally detected during twin pregnancy. All achieved R0 radical resection via individualized open, robot-assisted, or laparoscopic approaches, with no Clavien-Dindo grade ≥II perioperative complications. All tumors showed characteristic nuclear STAT6 positivity and CD34 expression, with uncommon MDM2/CDK4 and SMA/desmin co-expression. At median 36-month follow-up (3–84 months), no recurrence or metastasis was observed. Conclusions Complete R0 resection is the cornerstone of pelvic SFT treatment. Individualized surgery is safe even for complex cases. STAT6 positivity is the diagnostic gold standard. High-risk patients may benefit from adjuvant radiotherapy, and lifelong follow-up is mandatory for late recurrence.