The first 60 days: Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)
Primary lung tumours in children are rare and unlike adult lung cancer. Pleuropulmonary blastoma starts as a lung cyst in infants and results from a faulty DICER1 gene that also predisposes to thyroid, ovarian and kidney tumours; removing cysts early, guided by an international registry and family gene testing, prevents progression to the aggressive solid forms. Below, week by week, is what OnCo's record of Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Type I or Ir pleuropulmonary blastoma, DICER1 carriers.
- RadiologistNamed in the standard of care for: DICER1 carriers.
- SurgeonNamed in the standard of care for: Type I or Ir pleuropulmonary blastoma, Type II or III pleuropulmonary blastoma, Tracheobronchial carcinoid or mucoepidermoid carcinoma.
- Medical oncologistNamed in the standard of care for: Type I or Ir pleuropulmonary blastoma, Type II or III pleuropulmonary blastoma, Tracheobronchial carcinoid or mucoepidermoid carcinoma.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Type II or III pleuropulmonary blastoma.
- Palliative and supportive care teamNamed in the standard of care for: Type II or III pleuropulmonary blastoma.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Complete surgical resection of the cystic lesion; adjuvant chemotherapy is optional and registry-guided; germline DICER1 testing for the child and first-degree relatives.
- 2.Type II or III pleuropulmonary blastomaInternational PPB/DICER1 Registry recommendations (Cancer 2015)
Neoadjuvant or adjuvant multi-agent chemotherapy (ifosfamide, vincristine, dactinomycin, doxorubicin) with complete resection; radiotherapy for unresectable residual disease.
Bronchoscopic assessment and parenchyma-sparing surgical resection (sleeve resection); systemic therapy rarely needed.
Surveillance per international consensus: chest imaging in infancy and early childhood, abdominal ultrasound, thyroid ultrasound, and awareness of ovarian and other syndrome tumours.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Germline DICER1 loss-of-function variant, Somatic DICER1 RNase IIIb hotspot mutation, PPB typeon pathology, Chest CT for cystic lung lesions in DICER1 carriers, ALK rearrangement), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Pleuropulmonary blastoma type I, Pleuropulmonary blastoma type Ir, Pleuropulmonary blastoma type II.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Type I or Ir pleuropulmonary blastoma
- For my situation (type i or ir pleuropulmonary blastoma), which of the standard options do you recommend and why?Guideline options include: Complete surgical resection of the cystic lesion; adjuvant chemotherapy is optional and registry-guided; germline DICER1 testing for the child and first-degree relatives.
Type II or III pleuropulmonary blastoma
- For my situation (type ii or iii pleuropulmonary blastoma), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant or adjuvant multi-agent chemotherapy (ifosfamide, vincristine, dactinomycin, doxorubicin) with complete resection; radiotherapy for unresectable residual disease.
- Am I a candidate for Ifosfamide, Vincristine, Dactinomycin (actinomycin D) or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Tracheobronchial carcinoid or mucoepidermoid carcinoma
- For my situation (tracheobronchial carcinoid or mucoepidermoid carcinoma), which of the standard options do you recommend and why?Guideline options include: Bronchoscopic assessment and parenchyma-sparing surgical resection (sleeve resection); systemic therapy rarely needed.
DICER1 carriers
- For my situation (dicer1 carriers), which of the standard options do you recommend and why?Guideline options include: Surveillance per international consensus: chest imaging in infancy and early childhood, abdominal ultrasound, thyroid ultrasound, and awareness of ovarian and other syndrome tumours.
Any stage
- Are there clinical trials I could join, for example of Germline (hereditary) testing?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Whether adjuvant chemotherapy helps type I PPB; the registry is comparing outcomes with and without it”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Type III and recurrent PPB have poor outcomes and no targeted therapy; DICER1-dependent biology (miRNA processing) has not yet yielded a drug”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours): the full pagePrimary lung tumours in children are rare and unlike adult lung cancer. Pleuropulmonary blastoma starts as a lung cyst in infants and results from a faulty DICER1 gene that also predisposes to thyroid, ovarian and kidney tumours; removing cysts early, guided by an international registry and family gene testing, prevents progression to the aggressive solid forms.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Bronchoscopy (EBUS, robotic navigation): Passing a camera down the windpipe into the lungs to biopsy tumours and lymph nodes without surgery.
- Hereditary cancer syndromes: About 5-10% of cancers arise from an inherited gene fault.
Every term links to the glossary.