1. Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis
- Author
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Maansi Parekh, Vinay Kandula, Achala Donuru, David Saul, and Stephan S Leung
- Subjects
Ribonuclease III ,Pathology ,medicine.medical_specialty ,Lung Neoplasms ,Pleuropulmonary blastoma ,Multimodal Imaging ,030218 nuclear medicine & medical imaging ,Metastasis ,DEAD-box RNA Helicases ,Diagnosis, Differential ,03 medical and health sciences ,0302 clinical medicine ,Germline mutation ,Cystic Adenomatoid Malformation of Lung, Congenital ,Neuroblastoma ,Humans ,Medicine ,Radiology, Nuclear Medicine and imaging ,Child ,business.industry ,Infant, Newborn ,Infant ,Congenital pulmonary airway malformation ,Wilms' tumor ,medicine.disease ,Child, Preschool ,Differential diagnosis ,business ,Pulmonary Blastoma ,030217 neurology & neurosurgery ,Rare disease - Abstract
Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.
- Published
- 2022
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