Prognostic factors in pleuro‐pulmonary blastoma

Indolfi, Paolo; Bisogno, Gianni; Casale, Fiorina; Cecchetto, Giovanni; Salvo, Gian Luca De; Ferrari, Andrea; Donfrancesco, Alberto; Donofrio, Vittoria; Martone, Antonio; Martino, Martina Di; Tullio, Maria Teresa Di

doi:10.1002/pbc.20842

Cited by 60 publications

(51 citation statements)

References 21 publications

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“…The biologic spectrum of PPB is also illustrated by the facts that the 5-year Kaplan Meier survival in Type I PPB is 85-90%, 30 whereas it is 45-60% for Types II and III disease [Registry unpublished analysis]. 49,62 Finally, as PPB develops its more overtly ''malignant'' pathologic features, it acquires the capacity for metastatic spread, especially to the central nervous system. 63 Type I PPB has not been observed to metastasize; Types II and III PPB metastasize in approximately 10% and 55% of cases, respectively.…”

Section: Progression Of Type I Cystic Ppbmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

264

215

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Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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Section: Progression Of Type I Cystic Ppbmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

264

215

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“…5 These tumors carry a poor prognosis, with a 42-49% 5-year survival rate, despite aggressive surgical and chemotherapeutic treatment. [5][6][7] In addition, in 25% of pleuropulmon-ary blastoma patients or their family members other dysplasias or neoplasias occur. The diseases found to be associated with pleuropulmonary blastoma included cystic lung disease, cystic nephromas, Wilms' tumors, sarcomas, medulloblastomas, thyroid neoplasias, malignant germ cell tumors, Hodgkin's disease, leukemia, and Langerhans cell histiocytosis.…”

mentioning

confidence: 99%

Gain of chromosome 8q is a frequent finding in pleuropulmonary blastoma

et al. 2007

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Pleuropulmonary blastomas are rare malignant intrathoracic tumors of early childhood. They appear as a pulmonary-and/or pleural-based mass and their pathogenesis and relationship to other pediatric solid tumors is not well understood. In this study, paraffin-embedded material of five cases of pleuropulmonary blastoma was analyzed for genetic alterations by comparative genomic hybridization and five genetic loci by fluorescence in situ hybridization. Comparative genomic hybridization identified aberrations in all pleuropulmonary blastomas, including four amplifications in three tumors at chromosomes 5q33-34, 11q22.2-ter, 15q25-ter, and 19q11-13.2. The most frequent DNA gains involved 8q11-22.2 (four cases) and 20q (two cases), whereas the most common losses included 9p21-24 (two cases) and 11p14 (three cases). Chromosome 8 gains were confirmed by fluorescent in situ hybridization, resulting in the detection of up to five copies of chromosome 8 centromeres per nucleus. In the two surviving patients, chromosome 8 gains were the only genetic abnormality, suggesting that this might be an early event in pleuropulmonary blastoma carcinogenesis. The identification of new genetic alterations as well as the confirmation of previously reported ones (especially 8q gains) in pleuropulmonary blastoma should help to improve our understanding of both the molecular mechanisms underlying the tumorigenesis of pleuropulmonary blastoma and the relationship of pleuropulmonary blastoma with other pediatric tumors.

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“…Overall survival is 90 % for Type I and 40-60 % for Types II and III (Priest 2012). Extrapulmonary involvement and incomplete resection confer a worse prognosis (Indolfi et al 2007). …”

Section: Pleuropulmonary Blastomamentioning

confidence: 99%