title: Pulmonary blastomas of childhood: histologic, immunohistochemical, ultrastructural aspects and therapeutic considerations.
abstract: 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.
authors.affiliation: Department of Pathology, Bambino Gesù Pediatric Hospital, IRCCS, Rome, Italy. boldrini@opbg.net
authors.forename: Renata
authors.initials: R
authors.lastname: Boldrini
authors.forename: Rita
authors.initials: R
authors.lastname: Devito
authors.forename: Francesca
authors.initials: F
authors.lastname: Diomedi-Camassei
authors.forename: Paola
authors.initials: P
authors.lastname: Francalanci
authors.forename: Alessandro
authors.initials: A
authors.lastname: Inserra
authors.forename: Camillo
authors.initials: C
authors.lastname: Boglino
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Alessandro
authors.initials: A
authors.lastname: Jenkner
authors.forename: Francesco
authors.initials: F
authors.lastname: Callea
mesh_terms: 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*
chemical_list: D014408:Biomarkers, Tumor; D019009:Proto-Oncogene Proteins c-kit