Man With Facial Plethora and Edema

A 61-year-old man presents with swelling and discoloration of his face and neck of several weeks’ duration. He noted tightness of his shirt and tie, which has worsened. Friends and family members have commented on his appearance and have asked about his state of health. He has had no pain or changes in mentation, and he has been able to perform his full-time job as a car salesman.

HISTORY

He is a long-time, heavy cigarette smoker with a chronic “smoker’s cough” that likely has worsened recently. He denies dyspnea on exertion, but on careful questioning he reveals that he does indeed experience shortness of breath with effort. There are no nocturnal symptoms.

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PHYSICAL EXAMINATION

The patient has a somewhat plethoric face. Respiration rate is 20 breaths per minute; heart rate, 100 beats per minute; and blood pressure, 140/80 mm Hg. His neck and face seem swollen, and the veins of his neck are distended. There are venous collaterals across both shoulders. No gross nodes are palpable in his neck or supraclavicular fossae. Heart is normal. Wheezes and rhonchi are heard in the lung fields, but no stridor is appreciated.

IMAGING RESULTS

A chest radiograph reveals a widened mediastinum with probable paratracheal and hilar adenopathy on the right side.

Which of the following statements about this patient is correct?

A. Any biopsy procedure should await acute management of his presenting syndrome.

B. He should have stent placement emergently.

C. There is a roughly 50:50 chance that the cause of his symptoms is benign.

D. His presenting syndrome will not affect his survival prognosis.

Correct Answer: D

This patient’s history and physical findings are typical of the superior vena cava (SVC) syndrome, a constellation of findings that result from some form of blockage or compression of the SVC as blood is returned to the heart. The obstruction impedes blood flow and leads to upper body edema and over time the formation of collaterals across the upper body wall as was manifest in this patient. In acute situations, airway edema and cerebral edema with life-threatening effects can occur; however, objective assessment of a large body of experience shows such situations are quite uncommon (or actually rare) effects of SVC syndrome. Thus, this syndrome is not the dire emergency it was once thought to be in previous decades¹ and, as will be further discussed, there is time for accurate diagnosis and definitive planning of care.

ETIOLOGY

The etiology of SVC syndrome has dramatically changed over the past 50 years from a benign causation (eg, tuberculosis, syphilitic aortitis) to a 90% malignant etiology (eg, lung cancers, lymphoma) to a current 2:1 ratio of malignant to benign due to the advent of central venous catheters, which now account for almost all of the benign causation.² Catheter etiology is usually obvious by the presence of a catheter and/or a recent history of catheterization. The preferred imaging study in SVC syndrome, CT scanning with contrast, will confirm either intrinsic thrombotic occlusion or extrinsic neoplastic compression to easily differentiate between the two. Because this patient has no history of catheter placement, the probability of malignancy is very high and choice C is not correct.

DIAGNOSIS

This leads to the next step in the evaluation—making a definitive diagnosis. Again, older lore has yielded to more modern, accurate data. Fears of complications such as elevated venous pressure–induced bleeding and contrast reactions are unfounded, and appropriate diagnostic techniques can and should be used to quickly make an accurate diagnosis.^3,4 Thus, depending on the clinical findings, bronchoscopy, lymph node biopsy, and mediastinoscopy can and should be used to obtain tissue and arrive at a definitive diagnosis. Choice A is therefore incorrect.
Yields for bronchoscopy with or without transthoracic needle biopsy are in the 50% to 75% range, and mediastinoscopy techniques have yields in the 90% range.³

MANAGEMENT

Definitive management, of course, depends on the specific diagnosis. Neoplasms such as small cell lung cancer and lymphoma are exquisitely sensitive to chemotherapy, and systemic chemotherapy will quickly result in resolution of SVC syndrome; whereas other solid tumors usually require radiation therapy as well.^1,5 In rare cases of severe life-threatening airway or cerebral symptoms (most frequently when a large occlusive thrombosis from a catheter is the cause), intravascular stenting to bypass the obstruction can be performed.⁶ This is an uncommon situation.

This patient has a subacute onset, does not have life-threatening symptoms or signs, has already developed collaterals, and therefore does not appear to be a stent candidate, certainly in an urgent setting, making choice B incorrect. Rather, he should have a measured approach to prompt diagnosis; mediastinoscopy would seem to fit his symptoms and history that suggest lung cancer, as well as his chest radiograph that shows a widened mediastinum. Interestingly and importantly, the ultimate prognosis in cases of SVC syndrome is rarely related to the syndrome itself. This complication almost always means advanced disease, but the prognosis does not appear to differ from survival among patients with the same tumor type and stage who do not have SVC syndrome; thus, choice D is the correct statement here.^1,5

OUTCOME OF THIS CASE

A mediastinal biopsy procedure was performed without incident and revealed a non–small cell carcinoma of the lung. Combination radiation and chemotherapy was initiated, and within weeks clinical improvement in the SVC syndrome findings resulted. The definitive therapy for the lung cancer is ongoing. n