Superior vena cava syndrome due to mediastinal Gardner fibroma presenting as syncope

Abstract

Superior vena cava (SVC) syndrome refers to the clinical manifestation due to an obstruction in the SVC; resulting in decreased venous return from the head, neck and upper extremities. The obstruction can occur either due to tumour invasion of the vessel wall with associated thrombus or due to vessel wall compression by the tumour mass. The patient being reported is a young male who presented with recurrent episodes of syncope and was found to have mediastinal Gardner fibroma causing SVC syndrome. Gardner fibroma is a benign soft tissue lesion; and its occurrence in the mediastinum resulting in SVC syndrome has not been reported yet.

Facial Swelling Mimicking Anaphylaxis: A Case of Superior Vena Cava Syndrome in the Emergency Department

“…SVC syndrome represents the clinical manifestations of obstruction of the SVC, resulting in decreased venous return from the head, neck, and upper extremities [ 4 ]. It was first described by William Hunter in the 18th century in a patient with a syphilitic aortic aneurysm [ 5 ].…”

“…The obstruction can be due to a neoplastic invasion of the venous wall with associated intravascular thrombosis or by extrinsic compression exerted by a tumor mass against the wall of the SVC. Most cases are caused by malignant mediastinal tumors [ 4 ], of which 85% are due to lung cancers, particularly small-cell and squamous cell carcinomas. The non-malignant etiologies include aortic aneurysm, mediastinal fibrosis, thyromegaly, thymoma, Behcet’s syndrome, and infections such as tuberculosis, histoplasmosis, syphilis, and actinomycosis [ 4 ].…”

“…Most cases are caused by malignant mediastinal tumors [ 4 ], of which 85% are due to lung cancers, particularly small-cell and squamous cell carcinomas. The non-malignant etiologies include aortic aneurysm, mediastinal fibrosis, thyromegaly, thymoma, Behcet’s syndrome, and infections such as tuberculosis, histoplasmosis, syphilis, and actinomycosis [ 4 ]. The clinical presentation of SVC syndrome may include swelling of the face, arms, and neck; dyspnea and cough; and dilated neck veins.…”

“…Other less common symptoms include headache, syncope, nasal congestion, lingual swelling, hoarseness of voice, dysphagia, epistaxis, and hemoptysis [ 6 ]. Syncope is an unusual presentation [ 4 ].…”

Upper Mediastinal Syndrome Caused by Non-Hodgkin Lymphoma

“…SGŠV podrazumijeva kliničke manifestacije koje nastaju zbog opstrukcije protoka krvi kroz gornju šuplju venu. Opstrukcija može biti posljedica vanjskog pritiska tumorske mase ili neoplastične invazije venske stijenke s pridruženom intravaskularnom trombozom 4 . SGM označava stanje istovremenog postojanja SGŠV-a i opstrukcije dušnika.…”