Pulmonary blastomas of childhood: histologic, immunohistochemical, ultrastructural aspects and therapeutic considerations.
Pulmonary blastomas are rare neoplasms typically occurring in patients of pediatric age, clinically characterized by fever, respiratory distress, and radiologic findings of a pulmonary cystic and/or solid mass with partial or complete obliteration of emithorax. Their behavior is aggressive and outcome is poor due to frequent relapses and metastases. The histological, immunohistochemical, and ultrastructural aspects of a personal series of 6 cases of pulmonary blastoma are described and the differences between childhood and adult types are stressed. Due to the aggressiveness of these rare tumors, therapeutic management is quite difficult. The expression of the transmembrane tyrosin kinase receptor c-kit in all the solid cases of this series leads the authors to hypothesize new possible therapeutic implications for these tumors.
Boldrini
Renata
R
Department of Pathology, Bambino Gesù Pediatric Hospital, IRCCS, Rome, Italy. boldrini@opbg.net
Devito
Rita
R
Diomedi-Camassei
Francesca
F
Francalanci
Paola
P
Inserra
Alessandro
A
Boglino
Camillo
C
Donfrancesco
Alberto
A
Jenkner
Alessandro
A
Callea
Francesco
F
D014408:Biomarkers, Tumor / Q000032:analysis*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008175:Lung Neoplasms / Q000378:metabolism / Q000628:therapy* / Q000648:ultrastructure*; D008297:Male; D046529:Microscopy, Electron, Transmission; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D018202:Pulmonary Blastoma / Q000378:metabolism / Q000628:therapy* / Q000648:ultrastructure*
D014408:Biomarkers, Tumor; D019009:Proto-Oncogene Proteins c-kit