This patient's clinical presentation is consistent with a diagnosis of superior vena cava syndrome (SVCS), secondary to SCLC.
SCLC is an aggressive, poorly differentiated, high-grade neuroendocrine carcinoma that accounts for approximately 13%-15% of all new lung cancer cases in the United States. SCLC has a propensity for early dissemination; as such, 80%-85% of patients are diagnosed with extensive disease (ES-SCLC). This is common in heavy smokers. Most SCLC tumors are found in hilar or perihilar areas; <5% present in peripheral locations. In many cases, invasion into the peribronchial tissue and lymph node can be clearly identified, with a typical circumferential spread along the submucosa of the bronchi.
Up to 10% of patients with SCLC develop SVCS, which comprises an array of signs and symptoms that result from the obstruction of blood flow through the thin-walled superior vena cava. Clinical symptoms may include cough, dyspnea, and orthopnea; facial edema and plethora, upper extremity swelling, and venous distension of the chest wall and neck are the most commonly encountered signs. Most cases of SVCS occur in patients with mediastinal tumors, although noncancerous causes (eg, thrombosis and fibrosing mediastinitis) can also give rise to it. The diagnosis of SVCS is usually made clinically and then confirmed with imaging (chest radiography, contrast-enhanced CT, duplex ultrasound, conventional venography, and/or magnetic resonance venography).
Though it was traditionally considered a virtual emergency, patients seldom experience life-threatening complications from SVCS. The goals of treatment are to alleviate the symptoms of SVC obstruction and treat the underlying disease process. Treatment approaches include radiation therapy, chemotherapy, open surgery, and endovenous recanalization; however, patients with clinical SVCS often achieve significant improvement in symptoms from conservative treatment approaches, including elevation of the head of the bed and supplemental oxygen. Systemic chemotherapy can effectively relieve the symptoms of SVCS obstruction, typically within 1-2 weeks of treatment initiation. Up to 80% of patients with SCLC and non-Hodgkin lymphoma may experience complete relief of SVCS symptoms with chemotherapy treatment.
Radiation therapy was once considered the standard approach to the management of SVCS in patients with cancer; however, endovenous recanalization can alleviate symptoms faster than radiation therapy — usually within 72 hours, whereas radiation therapy can take up to 2 weeks to provide relief. Endovascular therapy is also associated with higher efficacy rates than is radiation therapy.
Open surgery plays a limited role in the management of SVC obstruction, although it may be the best approach in select cases.
In cases involving brain edema, decreased cardiac output, or upper airway edema, emergency treatment is indicated.
Karl J. D'Silva, MD, Clinical Assistant Professor, Department of Medicine, Tufts University School of Medicine, Boston; Medical Director, Department of Oncology and Hematology, Lahey Hospital and Medical Center, Peabody, Massachusetts.
Karl J. D'Silva, MD, has disclosed no relevant financial relationships.
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