The rarity of PEComa tumours has precluded any clinical trials, but surgery remains the most commonly reported treatment modality with just a few reports on treatment plans involving chemoradiation. We describe a patient with a mediastinal PEComa who presented with symptoms concerning for superior vena cava syndrome. She was deemed inoperable and was thus treated exclusively with chemoradiation therapy. The use of chemoradiation in the treatment of PEComa tumours is reviewed.

A 51-year-old man presented with a 5-day history of progressive facial swelling, sensation of head fullness, increasing shortness of breath and paroxysmal nocturnal dyspnea. He denied chest pain, syncope or presyncope. Pastmedical history included mechanical aortic valve replacement 7 years prior and atrial fibrillation treated with warfarin. A clinical diagnosis of acute superior vena cava (SVC) syndrome was made. Portable chest radiograph showed a widened superior mediastinum. Computed tomography scan of the thorax demonstrated a large type A aortic dissection almost completely effacing the SVC. Acute type A aortic dissection (AD) is an emergency requiring prompt diagnosis and treatment. Patients typically present with acute onset of chest and/or back pain, classically described as “ripping” or “tearing.” SVC syndrome is rarely, if ever, mentioned as a presentation, as it is usually due to more chronic conditions. This case illustrates a rare incidence of type A AD actually presenting as SVC syndrome.

Thrombotic venous obstruction in patients with a tunnelled central venous catheter is a cause of superior vena cava syndrome that is not routinely encountered by emergency physicians. Diagnosis requires identifying patients at risk (e.g., those under treatment for cancer and those who have a tunnelled central venous catheter), recognizing the signs and symptoms of superior vena cava syndrome, usually dyspnea and dilated neck or thoracic veins, and imaging the venous obstruction using computer tomography or sonography. Management involves anticoagulation and local thrombolytic administration. We report the case of a 28-year-old woman who presented with a 2-day history of face, chest and bilateral arm swelling who had been receiving maintenance chemotherapy for acute lymphoblastic leukemia through a Hickman® catheter. This case demonstrates the need to be vigilant for thrombus formation in patients with long-term, indwelling central venous catheters.

Venous thromboembolic disease has an estimated annual incidence of one in 1000 people. However, thrombosis of the superior mediastinum and neck veins is less frequent and it is usually due to direct trauma to the neck by intravenous catheters, drug abusers or neck dissection surgery. Local or distant malignancy (Trousseau’s syndrome) is also an important cause.

Thrombosis of the superior mediastinal and internal jugular veins is rarely a cause of primary referral to the otolaryngologist. On these rare occasions, it can present as a painful neck mass, but may also present with stridor, dysphonia or dysphagia. The four patients presented here illustrate different ways of presentation. Different imaging techniques such as ultrasound, computed tomography (CT) scan, magnetic resonance imaging (MRI) and venogram, will produce a diagnosis of thrombosis, occasionally with a mass, but only a biopsy will confirm or rule-out malignancy.

Spontaneous thrombophlebitis can be the first manifestation of an occult neoplasm and any investigation into venous thrombosis must include a thorough general examination and follow up.

A 41-year-old man was brought to the ED after a motor vehicle crash. On presentation, he demonstrated symptoms compatible with superior vena cava (SVC) syndrome, including extreme dyspnea, face and neck cyanosis and facial swelling. A chest tube was inserted and drained large amounts of sanguineous fluid. An exploratory thoracotomy revealed an extensive tumour encasing the SVC and the hilum. Biopsy confirmed the diagnosis of T-cell lymphoma.

The most common cause of SVC syndrome is malignant disease, with bronchogenic carcinoma and lymphoma being most frequent. Review of the literature uncovered only a few anecdotal reports of traumatic SVC syndrome. There are no previous reported cases of malignant SVC syndrome presenting in association with trauma.