盆腔腹膜后孤立性纤维瘤：包括Doege-Porter综合征的两例病例
Pelvic Retroperitoneal Solitary Fibrous Tumor: Two Cases Including Doege-Potter Syndrome.
摘要(中文译文)
孤立性纤维性肿瘤（SFT）是一种罕见的间叶组织病变，通常不表现出侵袭性行为，转移潜能低。虽然该病常见于胸膜和胸腔，但发生于腹膜后盆腔者较为少见。我们报告两例在哥伦比亚国家癌症研究所（Instituto Nacional de Cancerología）接受治疗的盆腔腹膜后SFT病例。第一例为76岁男性，盆腔肿块已发展一年。计算机断层扫描（CT）显示前列腺附近有一腹膜后肿瘤，并可见一可疑肺结节。活检证实为高危SFT，信号转导和转录激活因子6（STAT6）阳性，Ki-67指数为40%。患者接受了广泛手术切除，切缘清晰，术中还偶然发现前列腺腺癌。随访12个月，未见复发证据。第二例为35岁男性，因盆腔SFT继发Doege-Potter综合征而出现严重低血糖。影像学显示一巨大的盆腔腹膜后肿块导致梗阻性尿路病变，需行双侧肾造瘘。活检证实为高危SFT，STAT6阳性，Ki-67指数为40%。患者接受了广泛手术切除，术中并发大出血，需行二期手术。病理显示切缘接触；然而术后病程顺利。随访六个月，患者未再出现低血糖发作，影像学亦无进展。SFT仍是一种罕见的疾病，尤其是位于腹膜后盆腔区域时。两例病例均凸显了该病的临床病理学变异性，并强调早期诊断怀疑和多学科管理对于优化治疗结局至关重要。
Abstract
Solitary fibrous tumor (SFT) is a rare mesenchymal lesion that typically exhibits no aggressive behavior and has low metastatic potential. While it is commonly described in the pleura and thorax, its occurrence in the retroperitoneal pelvic cavity is uncommon. We report two cases of pelvic retroperitoneal SFT treated at the Instituto Nacional de Cancerología in Colombia. The first case involved a 76-year-old man with a pelvic mass that developed over one year. Computed tomography (CT) showed a retroperitoneal tumor near the prostate and a suspicious pulmonary nodule. Biopsy confirmed high-risk SFT with signal transducer and activator of transcription 6 (STAT6) positivity and a Ki-67 index of 40%. The patient underwent extensive surgical resection with clear margins, during which an incidental prostatic adenocarcinoma was identified. At 12 months of follow-up, no evidence of recurrence was observed. The second case involved a 35-year-old man with severe hypoglycemia due to Doege-Potter syndrome secondary to pelvic SFT. Imaging demonstrated a large pelvic retroperitoneal mass causing obstructive uropathy, requiring bilateral nephrostomies. Biopsy confirmed high-risk SFT with STAT6 positivity and a Ki-67 index of 40%. The patient underwent extensive surgical resection complicated by massive intraoperative bleeding, requiring a two-stage procedure. Pathology revealed margins in contact; however, the postoperative course was favorable. At six months of follow-up, the patient remained free of hypoglycemic episodes and without radiological progression. SFT remains a rare condition, especially when located in the retroperitoneal pelvic region. Both cases highlight the clinicopathological variability of the disease and emphasize that early diagnostic suspicion and multidisciplinary management are crucial to optimizing treatment outcomes.
如何引用
AMA
Emma C Gómez, Sandra Diaz Casas, Patricia López Correa, Carlos Vega, Laura Arce, Natalia Lasprilla, et al. Pelvic Retroperitoneal Solitary Fibrous Tumor: Two Cases Including Doege-Potter Syndrome.. Cureus. 2026; doi:10.7759/cureus.110061.
APA
Emma C Gómez, Sandra Diaz Casas, Patricia López Correa, Carlos Vega, Laura Arce, Natalia Lasprilla, et al (2026). Pelvic Retroperitoneal Solitary Fibrous Tumor: Two Cases Including Doege-Potter Syndrome.. Cureus. https://doi.org/10.7759/cureus.110061
GB/T 7714
Emma C Gómez, Sandra Diaz Casas, Patricia López Correa, Carlos Vega, Laura Arce, Natalia Lasprilla, et al. Pelvic Retroperitoneal Solitary Fibrous Tumor: Two Cases Including Doege-Potter Syndrome.[J]. Cureus, 2026 doi:10.7759/cureus.110061.
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