IS Case 298: Pleuropulmonary blastoma
Imaging Sciences URMC 2009
Publication Date: 2009-08-05
Patient is a 15-year old male with respiratory distress.
Pleuropulmonary blastoma (PPB) is a rare aggressive malignant primary neoplasm of the pleuropulmonary mesenchyme occurring almost exclusively in children under the age of 5. This neoplasm is analogous to Wilm's tumor (of the kidney) and neuroblastoma (of the adrenal gland) and is histologically different from adult pulmonary blastoma .
Earliest pathological stage is manifested as a cyst in the lung, often multiloculated. The early cystic stage of PPB, stage I, occurs in the first two years of life (mean age = 10 months) and can resemble other benign cystic lung lesions such as congenital cystic adenomatoid malformation, bronchogenic cyst, and pulmonary sequestration. Thus, is the basis for the argument for surgical excision of all lung cysts found in children. Progression from stage I PPB to stage II PPB entails the development of a solid lesion in a preexisting cystic lesion; with stage III PPB representing a purely solid lesion .
Overall survival in stage I PPB ranges from 80-85% and 45-50% in stage III PPB, with stage II PPB somewhere in between [3, 4]. Children with stage I PPB are traditionally treated with surgical excision and/or adjuvant chemotherapy. It should be noted recurrent stage I PPB frequently progresses to more malignant stages II-III PPB. Adjuvant chemotherapy in stage I PPB has been linked to improved recurrence-free survival . Stage II-III PPB are treated with surgical excision and/or adjuvant chemotherapy, with neoadjuvant chemotherapy considered/reserved for stage III PPB .
- Donnelly L, Jones B, O'Hara S, et al. Diagnostic Imaging: Pediatrics. Amirsys. 2005: 2:90-93.
- Priest JR, Hill DA, Williams GM, et al. Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol. 2006 Sep 20;24(27):4492-8. PMID: 16983119 [PubMed]
- Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP. Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer. 1997 Jul 1;80(1):147-61. PMID: 9210721 [PubMed]
- Hill DA, Williams GM, Finkelstein MJ, et al. Significance of histologic type and anaplasia in pleuropulmonary blastoma. Presented at the Annual Meeting of the Society for Pediatric Pathology, February 10-12, 2006, Atlanta, GA (abstr).