Pleuropulmonary blastoma in extrapulmonary lung tissue: A case report☆

We report a case of pleuropulmonary blastoma (PPB) occurring in a very unusual location, extrapulmonary lung tissue, as possible extralobar pulmonary sequestration. The patient was a girl aged two years and six months with respiratory symptoms. Infection of a congenital pulmonary malformation was suspected based on computed tomography findings. The operative findings revealed a partly adherent mass located between the upper and middle right lobes, and a feeding vessel. Histological analysis showed the mass to be PPB. A small portion of normal-looking pulmonary tissues was confirmed in the subcapsular area of the mass, suggesting that the tumor originated in extrapulmonary lung tissue. The patient underwent postoperative chemotherapy. No recurrence or metastasis of the PPB has occurred during the 10-year follow-up. When considering the treatment options for an extralobar pulmonary lesion, the possibility of PPB should be considered, although its occurrence may be extremely rare.