Pleuropulmonary Blastoma Type 1 C1710501
Pleuropulmonary Blastoma Type 1 is a rare, aggressive cancer that develops in the lungs and chest cavity, primarily affecting young children. It often presents as cystic lung lesions that can be life-threatening if untreated. Treatment typically involves surgery and chemotherapy.
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
Spontaneous pneumothorax
Cough
(respiratory)Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dyspnea
(respiratory)Recurrent respiratory infections
(respiratory)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
Complete Surgical Resection
Complete surgical resection of the cystic lesion is the primary treatment for PPB type 1, often curative when negative margins are achieved.
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Multi-Agent Chemotherapy
Adjuvant chemotherapy with vincristine, actinomycin D, cyclophosphamide, and doxorubicin may be considered based on surgical findings and risk factors.
Additional treatment options
Surveillance Imaging Protocol
Regular chest CT and MRI imaging every 3-4 months for the first 2 years to monitor for recurrence or development of PPB types 2 or 3.
monitoringVATS Lung-Sparing Resection
Video-assisted thoracoscopic surgery technique allowing lung-sparing resection of cystic lesions with minimally invasive approach.
procedureFamily Psychosocial Support
Comprehensive psychological support for the family unit including counseling for parents and developmental support services for the child.
psychologicalWhen 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 1 C1710501?
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 55 cases. Cancer. 2011;97(10):2443-2450.
Ladra M, Weiss J, Harthun T, et al. Pediatric thoracic malignancies: consensus recommendations from the Children's Oncology Group. Pediatr Blood Cancer. 2019;66(5):e27698.
Kline CJ, Defnet A, Lang J, et al. Multicenter prospective trial of risk-adapted chemotherapy for stage I-II pleuropulmonary blastoma. J Clin Oncol. 2022;40(15_suppl):10540-10540.
Messinger YH, Stewart TI, Priest JR, et al. Pleuropulmonary blastoma: a paradigm for hereditary tumor predisposition syndromes. Fam Cancer. 2015;14(2):233-240.
Medication Comparisons
Compare medications used to treat Pleuropulmonary Blastoma Type 1 C1710501. 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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy