Superior mediastinal syndrome (SMS) is an uncommon presentation of childhood malignancy, of which Non-Hodgkin’s lymphoma is the commonest. Fungal infection as a cause of mediastinal mass leading to SMS is described mainly in immunocompromised patients. We report an 11-year-old thriving immunocompetent boy who presented with symptoms and signs of SMS along with chest wall swelling, due to Aspergillus mediastinitis.
CASE REPORT
An 11-year-old thriving boy presented to us with history of cough and fever for 2 months, along with swelling on the anterior aspect of left upper hemithorax noticed for 1 month, followed by left shoulder pain and orthopnea for 15 days. On examination, he had Horner syndrome on the left side, left-sided cervical and supraclavicular lymphadenopathy, and a swelling in the left parasternal area in the second and third intercostal spaces with a few prominent and tortuous veins. The trachea was deviated to the right. Air entry was reduced in the left infraclavicular, mammary and interscapular areas, with dullness on percussion and increased vocal resonance. In view of these features, the possibility of neoplastic lesion with SMS was considered and the child was worked up for the same.
Chest radiograph showed mediastinal widening. Contrast-enhanced computed tomography of the chest demonstrated a 12 × 11 cm mass in the anterior mediastinum, reaching inferiorly to the left hilum and encasing the vessels; the lung fields appeared normal. In the laboratory investigations, the blood counts showed hemoglobin 100 g/L, and total leukocyte count of 10.3 × 109/L with 63% neutrophils and eosinophils of 9%; platelet count was 328 × 109/L. Electrolytes, liver and renal functions were normal. Fine needle aspiration from the mass was done. It was cellular and showed scattered, epithelioid cell granulomas, multinucleated giant cells and polymorphs. Scattered Periodic acid Schiff positive, septate fungal profiles, with acute angled branching suggestive of Aspergillus, were visualized. Smear for acid fast bacilli was negative. Considering the strong possibility of malignancy and the rarity of Aspergillus presenting as a fungal mediastinitis repeat fine needle aspiration was done which showed similar findings as the previous. For further confirmation, culture of the aspirate was done which showed growth of Aspergillus flavus. Other corroborative findings suggestive of fungal infection were a positive serum galactomannan and a gel diffusion test showing precipitation against Aspergillus species. HIV enzyme-linked immunosorbent assay was negative. The immunoglobulin profile was normal, phagocytic function test was normal. CD markers for T and B cell CD3 and CD19 were normal.
The child was started on intravenous voriconazole at 5 mg/kg/day in divided doses. Two weeks following the start of therapy clinically the mass had reduced in size. The child was then shifted to oral therapy with a plan to continue the therapy for at least 6–8 weeks.
DISCUSSION
SMS includes superior vena cava syndrome (signs and symptoms resulting from compression or thrombosis of the superior vena cava) along with tracheal compression. SMS in the pediatric age group is commonly due to malignancy of which Non-Hodgkin’s lymphoma is the leading cause.1 Fungal mediastinitis in an immunocompetent patient; with no other predisposing factors; leading to SMS in pediatric patient has not been reported to the best of our knowledge. There have been a few case reports in adults; of immunocompetent patients presenting with these manifestations.2 In pediatrics cases of mediastinal lymphadenopathy due to histoplasmosis has been reported.3
Aspergillus mediastinitis is rare and has been described following cardiac surgeries. Pasqualotto and Denning5 described 500 cases of postoperative aspergillosis of which 11 patients had mediastinitis; but all these patients were beyond the pediatric age group. In immunocompetent individuals, it also induces an allergic response, manifesting as sinusitis and asthma.6 The definition for proven aspergillosis requires histopathologic documentation of infection and a positive culture of a specimen from a normally sterile site.6 In our child, the histopathology of the mediastinal mass was suggestive of Aspergillus and culture of the aspirate obtained following fine needle aspiration cytology was positive for A. flavus. Antigen-based test-serum galactomannan, a constituent of fungal cell wall is relatively specific for invasive aspergillosis, which was positive in our patient.
Voriconazole and deoxycholate amphotericin B are the only compounds licensed for primary treatment of invasive aspergillosis; voriconazole is the recommended treatment.7 It is generally recommended that treatment for invasive aspergillosis should be continued for a minimum of 6–12 weeks. In our case, intravenous voriconazole was started and 2 weeks after therapy a clinical reduction in the chest wall swelling was noted. Child was then transitioned to oral therapy and did well clinically.
ACKNOWLEDGMENT
The authors thank Prof. Arunaloke Chakraboti, Department of Microbiology, PGIMER, Chandigarh for the helpful assistance in performing the cultures.
REFERENCES
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