title: Large thoracic tumour without superior vena cava syndrome.
abstract: A 62-year-old male with long-standing smoking history presented with haemoptysis. Plain chest X-ray showed abnormal findings proximate to the right pulmonary hilum. Bronchoscopy revealed a fragile exophytic tumour 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 computed tomography demonstrated a 7.2 × 4.9 cm tumour 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 tumour constricting the right superior vena cava (SVC), no signs of SVC syndrome were present. In this case, the patient does not present with SVC syndrome, as expected due to the constriction of the (right) SVC caused by the tumour, 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 foetal 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 (LSPV). 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 LSPV, 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 ischaemic attacks and/or cerebral abscesses. This PLSVC variant is more often associated with absence of the right SVC and congenital heart abnormalities.
authors.affiliation: Second Dept of Propedeutic Surgery, Medical School, National & Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece.
authors.forename: N
authors.initials: N
authors.lastname: Garmpis
authors.affiliation: 1Second Department of Propedeutic Surgery, Laiko General Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece; 2N.S. Christeas Laboratory of Experimental Surgery and Surgical Research, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: C
authors.initials: C
authors.lastname: Damaskos
authors.affiliation: First Dept of Surgery, Vascular Division, Medical School, National & Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece. patelisn@gmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Patelis
authors.affiliation: Second Dept of Propedeutic Surgery, Medical School, National & Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece.
authors.forename: D
authors.initials: D
authors.lastname: Dimitroulis
authors.affiliation: N.S. Christeas Laboratory of Experimental Surgery and Surgical Research, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: E
authors.initials: E
authors.lastname: Spartalis
authors.affiliation: Second Pulmonary Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece.
authors.forename: I
authors.initials: I
authors.lastname: Tomos
authors.affiliation: Internal Medicine Department, Laiko General Hospital, University of Athens Medical School, Athens, Greece.
authors.forename: A
authors.initials: A
authors.lastname: Garmpi
authors.affiliation: Division of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece.
authors.forename: M
authors.initials: M
authors.lastname: Spartalis
authors.affiliation: Second Dept of Propedeutic Surgery, Medical School, National & Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece.
authors.forename: E A
authors.initials: EA
authors.lastname: Antoniou
authors.affiliation: Second Dept of Propedeutic Surgery, Medical School, National & Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece.
authors.forename: K
authors.initials: K
authors.lastname: Kontzoglou
authors.affiliation: Department of Thoracic Surgery, "Attikon" Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
authors.forename: P
authors.initials: P
authors.lastname: Tomos
keywords: persistent left superior vena cava; superior vena cava; thoracic; tumour