General thoracic surgeons serve the patient with SVCO in three ways. First and most commonly they are involved in the workup and diagnosis of the patient who presents with the spectrum of SVCO symptoms and a chest mass on radiographic studies. Second, a patient with severe symptoms from SVCO will need expedient management of life-threatening symptoms. Similar to approaching the patient with esophageal cancer a “Captain of the Ship” is needed in both above situations which often will require the coordination of care of a multidisciplinary team (oncology, radiation oncology, interventional radiology, pathology) and nonoperative means of treatment will often be utilized (radiation, chemotherapy, stenting, thrombolytic therapy). Last, the thoracic surgeon may be called upon to resect the SVC for benign or malignant disease.
When called upon to evaluate the patient with SVCO the thoracic surgeon should classify the patient by severity of the SVCO symptoms, achieve an expedient tissue diagnosis, coordinate the care of the multidisciplinary team of physicians, and evaluate if there is any role for therapeutic surgical intervention especially if the patient has a diagnosis of non–small-cell lung cancer (NSCLC), thyroid/thymic cancer, or germ cell neoplasm.
Approximately 35% of SVCO patients will be asymptomatic or have only mild symptoms of head and neck edema and cyanosis. Moderate-to-severe symptoms will be seen in 60% of patients manifested by increasing degrees of cerebral edema resulting in visual disturbances, headache, laryngeal edema, and diminished cardiac reserve. Patients will present with life-threatening symptoms 5% of the time.1
See proposed grading system for superior vena cava syndrome (Table 164-1).
Table 164-1Grading System for Svco ||Download (.pdf) Table 164-1Grading System for Svco
|Grade ||Category ||Estimated |
|0 ||Asymptomatic ||10 ||Radiographic superior vena cava obstruction in the absence of symptoms |
|1 ||Mild ||25 ||Edema in head or neck (vascular distention), cyanosis, plethora |
|2 ||Moderate ||50 ||Edema in head or neck with functional impairment (mild dysphagia, cough, mild or moderate impairment of head, jaw or eyelid movements, visual disturbances caused by ocular edema) |
|3 ||Severe ||10 ||Mild or moderate cerebral edema (headache, dizziness) or mild/moderate laryngeal edema or diminished cardiac reserve (syncope after bending) |
|4 ||Life threatening ||5 ||Significant cerebral edema (confusion, obtundation) or significant laryngeal edema (stridor) or significant hemodynamic compromise (syncope without precipitating factors, hypotension, renal insufficiency) |
|5 ||Fatal ||<1 ||Death |
As proposed by Detterbeck, patients with grade 4 symptoms require a venogram, urgent stenting, and possible thrombolytics. Patients with grade 1, 2, and 3 symptoms require tissue diagnosis and multidisciplinary discussion to decide on treatment modality based on particular tumor response to a specific modality. Surgery is usually considered for NSCLC, thymoma, thymic carcinoma, or a residual germ cell mass. Chemoradiotherapy is the mainstay of treatment for patients with small-cell lung cancer, lymphoma, or germ cell tumor. Supportive care only may be indicated for patients with treatment refractory tumors or poor performance status.
See treatment algorithm of superior vena cava syndrome (Table 164-2).
Table 164-2Treatment Algorithm of Superior Vena Cava Syndrome ||Download (.pdf) Table 164-2Treatment Algorithm of Superior Vena Cava Syndrome
Indications: Malignancy is the most common (90%) cause of SVCO usually due to bronchogenic carcinoma (>50% cases) with mediastinal lymphomas or germ cell neoplasms accounting for the rest.2 Occasionally, patients with SVCO of a benign etiology (sarcoidosis, histoplasmosis, fibrosing mediastinitis, iatrogenic thrombosis) may benefit from venous reconstruction to establish flow from the superior venous system to the right atrium.3,4