Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease
Shakeel Ahmed, Mansoor Hameed, Merlin M. Thomas, Khezar S. Syed, Irfan U. Haq, Abbas AlAbbas
Department of Pulmonary Medicine, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar
Am J Case Rep 2021; 22:e927556
Available online: 2020-11-19
Fibrosing mediastinitis is a rarely seen, progressive disease. It results from an excessive fibrotic reaction in the mediastinum. We describe a presentation of fibrosing mediastinitis that, to our knowledge, has never been seen before.
CASE REPORT: A 30-year-old female Colombian flight attendant presented with a right eyelid droop. Examination revealed partial right-sided ptosis and miosis but no anhidrosis. An ill-defined firm swelling was palpable at the root of the neck. Chest radiography revealed a widened mediastinum, and computerized tomography (CT) showed a right paratracheal mass without calcification extending to the thoracic inlet, encasing multiple blood vessels. All basic blood tests, magnetic resonance imaging of the head, and ultrasound Doppler of the neck vessels were normal. History and work up for infections including fungal diseases, granulomatous diseases, vasculitis, and autoimmune diseases were negative. Positron emission tomography (PET) showed significant FDG uptake in the mediastinum. Mediastinal biopsy was histologically consistent with fibrosing mediastinitis. All relevant immunohistochemistry and microbiological studies were negative. Subsequently, the patient developed signs of superior vena cava compression; this was managed by balloon angioplasty, which resulted in improvement of symptoms. However, over time, her symptoms worsened progressively, resulting in a left-sided ptosis and radiological progression of the mass on CT. She received treatment with rituximab and concomitant steroids, which yielded excellent results: the treatment led to both resolution of her symptoms and regression of the mass and its metabolic activity on PET scan.
CONCLUSIONS: Fibrosing mediastinitis can present with an incomplete Horner’s syndrome. Treatment with rituximab and steroids shows promising results in select cases of metabolically active idiopathic fibrosing mediastinitis.
Keywords: Fibrosis, Fluorodeoxyglucose F18, Tomography, Spiral Computed, mediastinitis, Positron-Emission Tomography, Vena Cava, Superior