A Rare Presentation of a Rare Disease : Pulmonary Lymphomatoid Granulomatosis

Abstract EN

A 70-year-old female presented with a 4-week history of dry cough and wheezing.

Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized.

Computed tomography scan (CT) of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes.

Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3.

She responded well clinically and radiologically to therapy.

Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease.

The most common radiographic feature is multiple lung nodules.

An isolated hilar mass is an exceptionally rare presentation of this rare disease.