BY MOHAMMED HASSAN-ALI, MSC, & AHMED RAZIUDDIN, MD
A 56-year-old black man presented to the ED complaining of face and neck swelling. An initial history was difficult to obtain because the patient did not speak English fluently and had slurred speech. Symptoms started approximately a week earlier when the patient was at his nursing home. He denied dysphagia or odynophagia, he had no rashes, pruritus, dyspnea, or peripheral edema, his face was flushed, and he had visibly engorged neck veins. He noticed that his tongue was mildly swollen, so he took Benadryl for a week with no relief. The patient said he believed that the symptoms might have been because a nurse gave him an incorrect medication at the nursing home. He had been a pack-a-day smoker for 30 years, and had a history of schizoaffective disorder, bipolar disorder with psychotic features, and GERD.
A CBC with differential, PT, PTT, BMP, and UA were all within normal limits. A chest x-ray confirmed a large mediastinal mass, and a soft tissue neck CT scan with contrast found a large mass in the upper mediastinum with extensive adenopathy in the base of the right side of his neck on the right, a right lobe thyroid mass, and occlusion of the superior vena cava and left subclavian vein. The report suggested that the findings were consistent with aggressive lymphoma or lung cancer.
The patient was admitted to the hospital with a scheduled emergent biopsy of the neck mass/lymph nodes. He was diagnosed with superior vena cava (SVC) syndrome and small cell lung carcinoma (SCLC) by tissue biopsy and pathology.
The SVC drains venous blood from the head, neck, upper extremities, and thorax. Obstruction of the SVC is usually because of neoplasms that invade the tissue wall or put pressure on the SVC. (J Clin Oncol 1984;2:961.) SCLC accounts for 25 percent of SVC syndrome because of malignancy. (HemOnc Today Feb. 10, 2012; http://bit.ly/1H3Uggt.) Clinical features of SVC syndrome include facial edema, difficulty swallowing, engorged neck veins, cough, and dyspnea. (Int J Radiat Oncol Biol Phys 1987;13:531.) Obstruction of the SVC can divert blood flow to the azygous veins, internal mammary veins, superior and inferior epigastric veins, and femoral and vertebral veins, but the venous pressure remains elevated. Complications of this condition include cerebral edema, coma, and death. (Joint Bone Spine 2002;69:416.)
The patient presented with only neck swelling and difficulty speaking. The speech impediment was most likely from the combination of SVC syndrome and right-sided thyroid mass occluding the right recurrent laryngeal nerve. The prognosis of SVC syndrome is poor because of the underlying neoplasm. The patient was promptly started on a dose of approximately 2900 cGy in 13 fractions to the mediastinum and neck nodes and scheduled to receive chemotherapy. After three weeks, he was medically stabilized, and had tolerated chemotherapy and radiation. The SVC syndrome did not compromise his breathing. The patient was advised that he could be discharged with a PORT-A-CATH for future chemotherapy, but the prognosis of his condition remained poor.
Lung cancer is the leading cause of cancer-related death in the United States. (CA Cancer J Clin 2008;58:71.) Its incidence is decreasing, but 13 percent of all new cancers are still from lung cancer. ("Lung Cancer Statistics," American Cancer Society, March 4, 2015; http://bit.ly/1JSd82D.) Even with current screening techniques, approximately half of patients have a metastatic presentation and a 45 percent five-year survival rate. Approximately 90 percent of lung cancer patients are active smokers or had recently stopped.
Signs and symptoms of this cancer include shortness of breath, cough, bone pain, weight loss, fatigue, and neurologic dysfunction. (Medscape, March 26, 2014; http://bit.ly/1dPWx29.) None of these was present in the patient. Most SCLCs are metastasized by the time of diagnosis, and are not curative by surgery. A combination of chemotherapy and radiation therapy is required. SCLCs are centrally located masses found in the lung parenchyma. (J Clin Oncol 2006;24:4526.) These pale gray tumors are fusiform-shaped and quite fragile. They can also express neuroendocrine markers and secrete polypeptide hormones that can cause paraneoplastic syndromes, such as syndrome of inappropriate antidiuretic hormone. (Chest 2003;123[1 Suppl]:97S.)
The time frame of disease development was unclear, but this patient's presentation was unique because he had nonthreatening symptoms. Lung cancer and SVC syndrome symptoms are usually aggressive, and can compromise the quality of life for patients. Our patient is now being treated to try to prevent the expected disease outcomes.
Mr. Hassan-Ali is a third-year medical student at the Windsor University School of Medicine in Cayon, Saint Kitts. Dr. Raziuddin is an internist specializing in emergency medicine at Louis A. Weiss Memorial and Thorek Memorial hospitals in Chicago.