Progressive massive fibrosis

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Chest pain and dyspnea.

Patient Data

Age: 70 years
Gender: Male

The hallmark of the examination is the presence of bilateral upper lobar and perihilar large conglomerate mass-like consolidations showing internal air-brocnhogram, irregular margins, nodular densities and associated with radiating strands.

Left upper lobar large cavity showing internal septae.

Left mild pleural effusion with underlying relaxation collapse.

Pathologically enlarged mediastinal retro-caval, pre-vascular, pre-aortic and subcarinal LNs are noted; some of them appear hyperdense,

Large sliding hiatus hernia.

Case Discussion

Upon further questioning about the patient's occupation, the patient mentioned that he had worked for 30 years in gold and silver industry and has been exposed to coal dust as well.

Progressive massive fibrosis (PMF) is a complication for pneumoconiosis, it refers to formation of large mass like lesions mainly in both upper lung lines with radiating strands. Sometimes they may contract and become within the perihilar regions.

Radiologically

On CT scan, they appear as large conglomerate mass-consolidations mainly within both upper lung lobes with radiating strands, usually with calcific regions or calcific mediastinal lymph nodes.

On MRI, T2 is important in differentiating it from neoplastic masses, as consolidations in progressive massive fibrosis elicits remarkable low T2 signal.

On PET/CT, they may show increased FDG uptake.

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