Tuberculosis related fibrosing mediastinits

Case contributed by Shimalis Tadasa Fayisa
Diagnosis almost certain

Presentation

Completed anti-tuberculosis medication 1 year ago. Now with swelling of the face, neck and both upper extremities for 10 days.

Patient Data

Age: 45 years
Gender: Male

Chest CT with contrast

ct
  • poorly enhancing, heterogeneous soft tissue attenuating mediastinal mass extends from the SVC origin to the proximal region of the bilateral major bronchus and subcarinal space. Mediastinal fat planes obliterated by the mass
  • major mediastinal vessels were also enclosed by the lesion: SVC, ascending aorta, arch and proximal portion of the descending aorta
  • SVC narrowed with collateral vein development, and contrast filling of the left dorsal scapular vein, internal thoracic vein, superior epigastric vein, ascending lumbar vein, and azygos vein
  • the focal hepatic hot spot sign, also known as the hot quadrate lobe sign, is caused by preferential enhancement of the medial segment of the left hepatic lobe suggesting collateral pathways with paraumblical veins
  • related pleural effusion is visible on a bilateral pleural cavity
  • calcifications can be seen in the hepatic parenchyma, splenic parenchyma, and mediastinal hilum. Additionally, there are spots of calcification in the mass at the left upper paratracheal region. All of these in keeping with granulomata
  • no evidence of pulmonary infiltrates

Case Discussion

The instance mentioned above illustrates fibrosing mediastinum with narrowing of the superior vena cava. Fibrosing mediastinitis is distinguished by the presence of thick fibroblasts. a non-cancerous and infrequently occurring fibrous tissue in the mediastinum.

This typically affects young age groups, and patients present with symptoms and signs associated with obstruction of critical mediastinal structures. Additionally, the main factor contributing to non-malignant SVC thrombosis is fibrosing mediastinum.

In the majority of cases, the source and pathophysiology are unknown, and linkages to infectious (histoplasmosis, tuberculosis) and noninfectious (immune-related) causes have been theorized.

The diagnosis of fibrosing mediastinitis is almost certain given the patient's prior TB treatment history (treated for disseminated TB involving lung, liver and spleen), multiple mediastinal, hepatic, and splenic parenchymal calcifications, typical imaging findings, as well as bronchoscopically guided biopsy were negative for fungi and acid-fast bacilli while demonstrating chronic inflammation without malignant cells.

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