pleuropulmonary blastoma
pleuropulmonary blastoma
A rare solid and biphasic primitive paediatric neoplasm that develops in the lung parenchyma or pleura.Clinical findings
Cough, recurrent upper respiratory tract infections, dyspnea, chest pain.
Prognosis
Generally poor with CNS metastasis; some cases may respond to excision and chemotherapy.
Pleuropulmonary blastoma types
• Type I—Cystic
Affects infants (average, 10 months); multicystic; difficult to diagnose early. It responds better to treatment than other types, and may not progress to higher type.
• Type Ir—(Regressing)
Similar to Type I, but lacks malignant cells.
• Type II—Solid and cystic
Affects older children.
• Type III—Solid
Affects older children; very poor prognosis; requires intensive chemotherapy.