RESUMO
Pulmonary fibrosis is typically a bilateral, progressive interstitial lung disease that is often idiopathic but can be associated with risk factors such as advanced age, environmental exposure, and drug toxicity. The pathophysiology is incompletely understood but involves transforming growth factor. The treatment choices for idiopathic disease include medical therapy that manipulates epigenetic pathways and lung transplantation. Here we present a 30-year-old female with no identifiable risk factors who developed unilateral pulmonary fibrosis. Clinical investigation eventually revealed a congenitally absent right pulmonary artery which was presumed to cause her unilateral disease. In contrast to idiopathic pulmonary fibrosis, treatment options for pulmonary fibrosis due to unilateral absence of a pulmonary artery include ipsilateral pulmonary vasculature embolization and/or pneumonectomy if disease is unmanageable without therapy.
RESUMO
BACKGROUND: Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a central nervous system tumor that shares many qualities with oligodendroglioma but is rarely and only very recently described as a distinct entity in the literature. CASE DESCRIPTION: A previously healthy, 19-year-old man presented with new onset of seizures. Imaging showed an intracranial mass, which was treated with surgical removal. Preoperative and postoperative magnetic resonance imaging, histopathologic examination, genetic testing, and immunohistochemical staining all supported a diagnosis of PLNTY. CONCLUSIONS: Diagnostic investigation of PLNTY shows many similarities with oligodendroglioma, and thus these entities can be mistaken for one another. Certain studies are needed to distinguish PLNTY and other dysembryoplastic neuroepithelial tumors, such as oligodendroglioma.