Large Thoracic Tumor Without Superior Vena Cava Syndrome

Folia Morphol (Warsz). 2017 Apr 10. doi: 10.5603/FM.a2017.0034. Online ahead of print.


A 62 year-old male with long-standing smoking history presented with hemoptysis. Plain chest x-ray showed abnormal findings proximate to the right pulmonary hilum. Bronchoscopy revealed a fragile exophytic tumor of the right wall of the lower third of the trachea, infiltrating the right main bronchus (75% stenosis) and the right upper lobar bronchus (near total occlusion). Contrast-enhanced chest CT demonstrated a 7.2x4.9 cm tumor contiguous to the above-mentioned structures, mediastinal lymph node pathology, and a vessel coursing inferiorly to the left of the aortic arch and anterior to the left hilum. Despite the tumor constricting the right superior vena cava, no signs of superior vena cava syndrome were present. In this case, the patient does not present with Superior Vena Cava (SVC) syndrome, as expected due to the constriction of the (right) SVC caused by the tumor, since head and neck veins drain through the Persistent Left Superior Vena Cava (PLSVC). PLSVC is the most common thoracic venous anomaly with an incidence of 0.3% to 0.5% of the general population and it is a congenital anomaly caused by the failure of the left anterior cardinal vein to regress and to consequently form the ligament of Marshall during fetal development. It is associated with absence of the left brachiocephalic vein and in 10 to 20% of cases the right SVC is absent. Two potential draining points of the PLSVC have been previously reported. In the majority of cases PLSVC drains directly into the coronary sinus, but less frequently it drains into the left atrium or the left superior pulmonary vein. In cases where the PLSVC drains into the coronary sinus, congenital heart defects are rare. The patient usually remains asymptomatic and PLSVC is an incidental finding during radiographic imaging or medical procedures. When the PLSVC drains into the left atrium or the left superior pulmonary vein, a right-to-left shunt is formed; a condition usually asymptomatic. In some reported cases this PLSVC variant presents with persistent, unexplained hypoxia or cyanosis and embolisation causing recurrent transient ischemic attacks and/or cerebral abscesses. This PLSVC variant is more often associated with absence of the right SVC and congenital heart abnormalities.

Keywords: PLSVC; superior vena cava; thoracic; tumor.