CTICasesParaseptal emphysema as a sequela of erdheim-chester disease
Imaging findings
Initial CT shows bilateral shaggy pleural and mediastinal thickening, perinephric soft tissue cuffs ('hairy kidney'), and bowel wall thickening. Eight years later, the soft-tissue infiltration has resolved, leaving behind advanced bilateral paraseptal/subpleural emphysema in a non-smoker.
Key takeaways
Erdheim-Chester disease is a rare histiocytic neoplasm that can infiltrate the pleura and lungs. Chronic histiocytic infiltration can release tissue-degrading elastases, leading to advanced paraseptal emphysema as a long-term sequela after active inflammation resolves.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Osteosclerosis and myocardial fat in tuberous sclerosisCongenital
- Solitary chest wall plasmacytomaNeoplastic
- EVALI with organizing pneumonia pattern from THC oilExposure
- Sarcoidosis presenting as cluster-like ground-glass nodulesAutoimmune
- Hypoplastic left heart syndrome with coronary-left atrial fistulaCongenital
- Disseminated Trichosporon asahii infectionInfection
- Tracheoesophageal fistula with migrated esophageal stent clipsIatrogenic
- Fibrosing mediastinitis without calcificationInfection
See all cases from August 23, 2019 →
Related Neoplastic cases
- Epicardial and perinephric infiltration in Erdheim-Chester disease
- Typical bronchial carcinoid tumor causing recurrent pneumonia
- Ossified pleural metastases from osteosarcoma
- Phosphaturic mesenchymal tumor causing tumor-induced osteomalacia
- Lymphomatoid granulomatosis
- Langerhans cell histiocytosis (LCH) involving the thyroid and lungs