Superior vena cava (SVC) syndrome includes a constellation of signs and symptoms resulting from SVC obstruction that produces elevated pressure in the afferent veins and increased blood flow through collateral vessels.
A man in his 60s was referred for a subcutaneous nodule that had appeared in the right supraclavicular region about 1 year earlier. The patient, who had a 5-year history of chronic obstructive pulmonary disease, complained of hoarseness, cough, and tachycardia. At physical examination, the nodule, measuring about 4 cm in diameter, was round and fixed with irregular ill-defined margins. Edema and erythema of the neck area and a well-defined net of superficial dilated vessels in the thoracic and epigastric areas were also detected (Figure 1A).
A, Clinical view of edema and erythema of the neck and presence of a well-defined net of dilated vessels in the thorax and the epigastric region. B, Computed tomography of the chest showing an expansive solid mass involving the mediastinum and infiltrating the right wall of the trachea, the superior vena cava and the left brachiocephalic vein; mass dimensions indicated by green lines.
Blood tests showed elevated levels of lactate dehydrogenase (1180 U/L; normal range, 250-500 U/L) and slightly raised levels of carcinoma antigen (CA) 15-3 (29 U/mL; normal range, 0-25 U/mL). Levels of CA 19-9, carcinoembryonic antigen, and α-fetoprotein were within the normal range. Otolaryngology consultation revealed paralysis of the right vocal cord. Chest radiography showed an opaque thoracic mass approximately 12 cm in diameter located on the right apical side that caused left tracheal deviation. Thoracic computed tomography confirmed the presence of an expansive solid mass in the mediastinum infiltrating the right side of the trachea, the SVC, and the left brachiocephalic vein (Figure 1B). The mass also compressed the interazygos esophageal system, with vascular shunts detectable in the right paramediastinal side.
A punch biopsy of the nodule revealed a large-cell neuroendocrine carcinoma showing a proliferation of atypical medium and large undifferentiated cells and areas of necrosis and hemorrhage (Figure 2A). Immunohistochemical staining was positive for the neuroendocrine marker synaptophysin (Figure 2B). The diagnosis of mediastinal neuroendocrine carcinoma of unknown origin associated with SVC syndrome prompted referral for complete surgical excision. The patient died a few weeks later after multiple postsurgical complications.
A, Histopathology specimen showing a proliferation of atypical and undifferentiated cells of medium and large size and areas of necrosis and hemorrhage (hematoxylin-eosin, original magnification ×100). B, Under synaptophysin immunohistochemical staining, specimen tested positive (original magnification ×200).
Historical causes of SVC syndrome are usually reported as infective, such as tuberculosis or syphilis. Currently, in 60% to 90% of cases, the cause is determined to be a malignant condition, predominantly bronchopulmonary cancer ( ~ 85%), whereas causative benign conditions are less commonly reported and include mediastinal fibrosis, retrosternal goiter, lipomatous hypertrophy, and transvenous cardiac pacemakers.1,2
Superior vena cava syndrome is related to extrinsic compression or intraluminal stenosis of the SVC. Consequent collateral circulation depends on the level of the obstruction that may be located (1) just at, (2) just above, or (3) just below the outlet of the azygos vein. In the first case, as in our case, dilated superficial collateral vessels are evident in the skin, involving internal mammary veins, epigastric veins, and intercostal veins. Signs and symptoms of SVC syndrome include facial or neck plethora, arm swelling, dilated chest veins, chest pain, dyspnea, light-headedness, dizziness, or syncope; presence of orthopnea and facial plethora may be exacerbated when the patient is supine.3,4
Mediastinal neuroendocrine carcinoma is one of the possible, although unusual, reported causes of SVC syndrome, with most patients showing a progressively worsening facial edema.5 A case of SVC syndrome due to mediastinal carcinoid tumor has been previously reported in a 36-year-old man who presented with facial swelling, mild dyspnea, pain in the right arm, slurring of the voice, and deviation of the tongue to the left side.6 However, development of cutaneous varicosities with multiple dilated venules on the anterior thoracic wall, as in this present report, has not been previously described to our knowledge in patients with mediastinal neuroendocrine carcinoma.
Recognition of SVC syndrome may be crucial for revealing a possible underlying malignant condition. In this regard, the dermatologist may play a decisive role because cutaneous manifestations may suggest this diagnosis when systemic symptoms are still lacking or negligible.
Corresponding Author: Giuseppe Micali, MD, Dermatology Clinic, University of Catania, AOU “Policlinico–Vittorio Emanuele,” PO “Gaspare Rodolico,” Via S. Sofia 78, 95123 Catania, Italy (firstname.lastname@example.org).
Published Online: January 14, 2015. doi:10.1001/jamadermatol.2014.4988.
Conflict of Interest Disclosures: None reported.
Lacarrubba F, Nasca MR, Cammisuli B, Micali G. Edema and Telangiectasia of the Chest Caused by Neuroendocrine Carcinoma. JAMA Dermatol. 2015;151(5):562-564. doi:10.1001/jamadermatol.2014.4988