A rare primary pulmonary tumor of childhood.
Pleuropulmonary blastoma (PPB) is known to be the pulmonary blastoma of childhood. It has a range of macroscopic and microscopic features which appear to correlate with eventual prognosis. Type 1, presenting as a multicystic lesion, occurs at an earlier age and has a more favorable prognosis than other types. The presented case of type 1 PPB had a microscopic focus of rhabdomyosarcoma. Although this patient was disease-free one year after the initial diagnosis without chemotherapy, he presented at 14 months with local dissemination and cardiac metastasis, revealing the inevitable chemo-radiotherapy need in PPB.
Kotiloğlu
Esin
E
Department of Pathology, Marmara University School of Medicine, Altunizade-Istanbul, Turkey.
Kaya
Handan
H
Kiyan
Gürsu
G
Tüney
Davut
D
Canpolat
Cengiz
C
D002675:Child, Preschool; D003560:Cysts / Q000175:diagnosis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D010997:Pleural Neoplasms / Q000150:complications / Q000175:diagnosis*; D018202:Pulmonary Blastoma / Q000150:complications / Q000175:diagnosis*; D018233:Rhabdomyosarcoma, Embryonal / Q000150:complications / Q000175:diagnosis*; D013479:Superior Vena Cava Syndrome / Q000209:etiology; D014057:Tomography, X-Ray Computed