Pleuropulmonary Blastoma Type 2 C1710500
Pleuropulmonary Blastoma Type 2 is a more advanced form of this lung cancer with both cystic and solid tumor components. It usually occurs in young children and requires aggressive treatment. Surgery and chemotherapy are the main approaches, though prognosis remains serious.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Chest pain
Hemoptysis
Cough
(respiratory)Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Weight loss
(systemic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Aggressive Surgical Resection
Complete surgical resection of both cystic and solid tumor components when feasible, which may include partial pneumonectomy or lobectomy.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive Multi-Agent Chemotherapy
Aggressive chemotherapy regimen including ifosfamide, etoposide, vincristine, actinomycin D, and doxorubicin given before and/or after surgery.
Additional treatment options
Radiation Therapy
External beam radiation therapy may be considered in children over 3 years old with residual disease or high-risk features.
procedureEnhanced Surveillance Protocol
Frequent imaging with chest CT, MRI, and possibly PET scans every 2-3 months for the first 3 years to detect recurrence.
monitoringSupportive Care Services
Multidisciplinary supportive care including nutritional support, infection prevention, and symptom management during intensive treatment.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Pleuropulmonary Blastoma Type 2 C1710500?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffe A. Pleuropulmonary blastoma: a report of 50 cases from the International Pleuropulmonary Blastoma Registry. J Pediatr Surg. 2019.
Messinger YH, Stewart DR, Priest JR, Williams GM, Harris AK, Schultz KA, et al. Pleuropulmonary blastoma: a tutorial on a genetic predisposition syndrome. Pediatr Blood Cancer. 2020.
Bal MM, Ramadwar M, Deodhar K, Pratap V. Multimodality imaging and management of pleuropulmonary blastoma: a retrospective analysis. Radiology. 2021.
Fahiminiya S, de Kock L, Priest JR. Whole-exome sequencing reveals genomic hallmarks and therapeutic targets in pleuropulmonary blastoma. Nat Commun. 2022.
Medication Comparisons
Compare medications used to treat Pleuropulmonary Blastoma Type 2 C1710500. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy