Watch the Mime Carefully! A Refractory Interstitial Lung Disease.
Paolo GrazianoPaolo FusoCristiano CarbonelliPublished in: Diagnostics (Basel, Switzerland) (2022)
Epithelioid hemangioendothelioma (EHE) is a rare neoplasm of a vascular origin which can arise in different locations such as the lungs, liver, soft tissue, and rarely, in the bones. In the lungs, pulmonary hemangioendothelioma (PEH) shows a variable clinical behavior, displaying a range from either an asymptomatic course to a highly aggressive progression with metastases. Based on radiological features, PEH differential diagnosis mainly includes primary or metastatic lymphangitic carcinomatosis, granulomatous infections, and diffuse interstitial lung diseases where ground glass pattern predominates. In this case, a transbronchial biopsy and subsequent histological and immunohistochemical analysis allowed for the attribution of the scenario to a pulmonary epithelioid hemangioendothelioma. Clinicians should always consider bronchoscopy as a useful and effective tool to better investigate indeterminate and questionable clinical pictures, sparing patients the morbidity and mortality associated with more invasive techniques such as surgical or thoracoscopic biopsy.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- ultrasound guided
- fine needle aspiration
- end stage renal disease
- pulmonary hypertension
- soft tissue
- rheumatoid arthritis
- squamous cell carcinoma
- chronic kidney disease
- ejection fraction
- newly diagnosed
- idiopathic pulmonary fibrosis
- small cell lung cancer
- peritoneal dialysis
- palliative care
- robot assisted