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Review
. 2019 Jul 25;6(8):86.
doi: 10.3390/children6080086.

Current Management of Pleuropulmonary Blastoma: A Surgical Perspective

Samantha Knight  1 , Tristan Knight  2   3 , Amir Khan  4 , Andrew J Murphy  5   6
Affiliations
Review

Current Management of Pleuropulmonary Blastoma: A Surgical Perspective

Samantha Knight et al. Children (Basel). .

Abstract

Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1-related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age of diagnosis and prognosis. We sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors and their multidisciplinary treatment, with an emphasis on surgical management. We describe the complementary roles of chemotherapy and surgery in the successful management of this disease. We discuss the timing of surgery and options for surgical approaches. We address the differentiation of PPB from congenital pulmonary airway malformation and the role of DICER1 testing for children with pulmonary cysts.

Keywords: DICER1; congenital cystic adenomatoid malformations; congenital pulmonary airway malformations; multidisciplinary management; pleuropulmonary blastoma; surgery.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
Types of pleuropulmonary blastomas (PPB) (A) Type I purely cystic PPB (white arrow). (B) Type II PPB with cystic (white arrow) and solid (black arrow) components. (C) Type III purely solid PPB occupying the entire left hemithorax.
Figure 2
Figure 2
Proposed management algorithm based on type and complexity of PPB. Note: Type I PPB may also be >10 cm in size, but neoadjuvant chemotherapy is not recommended for type I PPB because the purely cystic morphology is not predicted to respond in a manner that would make obtaining negative surgical margins any easier. Furthermore, chemotherapy can be spared entirely if Type I PPB are resected with negative margins, regardless of lesion size.
See this image and copyright information in PMC

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