Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation.

Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case. It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.

Right atrial extension of primary venous leiomyosarcoma: pulmonary embolism and Budd-Chiari syndrome at presentation-a case report.

Venous leiomyosarcomas are rare and arise predominantly in the inferior vena cava (IVC). The clinical findings are nonspecific and may precede the diagnosis by several years. IVC leiomyosarcoma is predominantly seen in women. Intracavitary extension of vascular tumors tends to result from embolization or propagation along great veins, and this is a serious risk factor for pulmonary embolism and sudden death when it reaches the right heart. Modern imaging modalities using computed tomography, magnetic resonance imaging, individually or in combination with cavography, ultrasound, and echocardiography, allow an early and accurate preoperative diagnosis, resulting in a higher rate of surgical resection and improved survival. The authors present a 72-year-old woman who presented with pulmonary embolism and Budd-Chiari syndrome. Pathological examination revealed a leiomyosarcoma. The tumor, involving the IVC, was diagnosed with imaging techniques that showed intracardiac extension of a primary venous leiomyosarcoma. The literature discussing leiomyosarcoma of the IVC is briefly reviewed.