Clinicopathological features of uterine intravenous leiomyomatosis

ZHAO Yan; JIANG Shali; LUO Haijun

doi:10.3969/j.issn.1007-8096.2026.01.008

Abstract：Objective To investigate the clinicopathological characteristics of intravenous leiomyomatosis(IVL)in order to improve diagnostic accuracy and reduce the risk of misdiagnosis.Methods A retrospective analysis was performed on the clinical data of 4 patients with IVL.Surgical specimens were examined using HE staining and immunohistochemistry(EnVision method).Their pathological features were discussed in conjunction with a literature review.Results The average age of the patients was 48.5 years.The main clinical manifestations included pelvic masses,menstrual disorders,and lower abdominal pain.Preoperative imaging misdiagnosed 3 cases as multiple uterine leiomyomas and suspected sarcoma with adnexal invasion in 1 case.Postoperative pathology revealed multiple intramural nodules within the uterine myometrium,some with edematous degeneration and soft to medium consistency.Worm-like tumor masses grew within vascular lumens in the myometrium.Tumor extension into the broad ligament was observed in 3 cases,and involvement of the ovarian vein occurred in 1 case.Microscopically,spindle-shaped smooth muscle cells were seen,exhibiting low-grade atypia and absence of necrosis,accompanied by hyaline degeneration and thick-walled blood vessels.Immunohistochemistry showed that the tumor cells were strongly positive for ER and PR,weakly positive for SMA,H-caldesmon,and desmin,and negative for CD10.CD34 and ERG were expressed in the endothelial cells lining the intravascular tumor surfaces.Conclusion The clinical and imaging presentations of IVL lack specificity.Pathological examination is essential for a definitive diagnosis,particularly for large intramural masses with significant edema and broad ligament masses.Standardized specimen sampling can reduce missed diagnoses.The pathological diagnosis of IVL has guiding significance for treatment and follow-up..