Case Presentation: A 66-year-old man with a history of Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia(SLL/CLL) in remission, hypogammaglobulinemia and atrial fibrillation presented to an outside hospital with shortness of breath, facial swelling and bilateral upper extremity edema.Computed tomography scanning done prior to this admission showed that the patient had a right mediastinal mass posterior to the superior vena cava and azygous vein with possible invasioninto the right pulmonary artery. Repeat imaging showed interval enlargement of the mass over 6 months causing narrowing of the right mainstem bronchus. The patient underwent a thoracotomyfor biopsy of the mediastinal mass that was inconclusive for malignancy. He was subsequently transferred to our hospital for higher level of care. A thoracentesis was performed showing transudative pleural fluid. The fluid cytology was also negative for malignancy. The patient underwent an endobronchialultrasound for fine needle aspiration of the mediastinal mass. Pathology results were positive for Aspergillus fumigatus. The patient was deemed too high risk for surgical intervention and was medically managed with voriconazole and anticoagulation. He was discharged with close follow up with infectious disease, hematology/oncology, and pulmonology.
Discussion: Superior vena cava (SVC) syndrome is most often seen in patients with oncologic disorders. It is most often caused by a malignant mass; however, there are other notable causes, including fungal infections. There have been only two previously reported cases of SVC syndrome caused by Aspergillus. In both cases, the patients were actively receiving treatment for hematologic disorders when they were diagnosed with SVC syndrome. These patients were in their mid-30s and died shortly after diagnosis. Our patient had not been receiving any treatments for SLL/CLL for several months when he presented to the hospital. It is unknown when the patient first became infected with Aspergillus. Despite treatment with antifungals, the prognosis is still poor. There is concern forangioinvasion of the aspergilloma, causing massive bleed. However, given the risk of bleed and possible invasion into the vessels, these patients are also poor surgical candidates. More information on early detection of aspergillosis and prevention of SVC syndrome is required. There is also a need for better management options for patients who have already developed SVC syndrome from the fungus.
Conclusions: SVC syndrome caused by aspergillosis is rare with a poor prognosis. These patients generally have multiple comorbidities and are not surgical candidates. Treatment guidelines currently suggest conservative management with antifungals. More research into the disease process and the best treatment options to improve prognosis is warranted.