Pleuropulmonary blastoma

Key points

Malignant mesenchymal tumours

3 types of paediatric lung tumour:

1. Pleuropulmonary blastoma (PPB)

2. Pulmonary blastoma

3. Foetal adenocarcinoma

3 types of PPB

1. Cystic

2. Solid/Cystic

3. Solid

Incidence is younger to older in order by types 1,2,3

Patients are usually younger than 4 years

DICER 1 mutations in 40%

Polysomy of chromosome 8

Can arise from mediastinum and diaphragm

Symptoms

Type 1: Respiratory distress

Types 2 + 3: Chest pain and cough

Management

Type 1: Resection

Some authors advocate adjuvant chemotherapy, but there is no consensus. No radiotherapy

Types 2 and 3: Resection recommended if feasible + adjuvant chemotherapy (doxorubicin-based) and/or radiation therapy

Outcome

Recurrence is often local

Type 1: 80-90% 5 year disease-free survival

Type 2 + 3: <50% 5 year disease-free survival

References

Mlika M, Anjum F, El Mezni F. Pleuropulmonary Blastoma. [Updated 2023 Apr 27]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK534211/