Idiopathic fibrosing mediastinitis

Area of Science:

• Pulmonology
• Pathology
• Radiology

Background:

• Fibrosing mediastinitis is uncommon in non-endemic areas for histoplasmosis.
• Idiopathic fibrosing mediastinitis can mimic other conditions, necessitating thorough differential diagnosis.
• The case highlights the diagnostic challenges of rare mediastinal diseases.

Observation:

• A 30-year-old female presented with a 2-year history of intermittent hemoptysis without constitutional symptoms.
• Chest CT revealed a mass-like lesion encasing the right pulmonary trunk and adjacent structures, with prominent bronchial arterial circulation.
• Endobronchial ultrasound with transbronchial needle aspiration yielded a paucicellular sample, negative for malignancy or granulomas.

Findings:

• Infectious, granulomatous, autoimmune, and neoplastic etiologies were systematically excluded through comprehensive testing.
• The patient's presentation and negative workup led to a diagnosis of idiopathic fibrosing mediastinitis by exclusion.
• Successful treatment was achieved using oral corticosteroids.

Implications:

• This case underscores the importance of considering idiopathic fibrosing mediastinitis in the differential diagnosis of mediastinal masses, especially in non-endemic regions.
• Methodical exclusion of alternative diagnoses is crucial for accurate identification of this rare condition.
• Corticosteroid therapy may be an effective treatment option for idiopathic fibrosing mediastinitis.