Pleuropulmonary blastoma

Pleuropulmonary blastoma
Synonyms	PPB
Pronounce	N/A
Specialty	N/A
Symptoms	Cough, shortness of breath, chest pain, fever
Complications	Metastasis, pneumothorax
Onset	Typically in infancy or early childhood
Duration	Variable
Types	Type I, Type II, Type III
Causes	Genetic mutations, often associated with DICER1 syndrome
Risks	Family history of DICER1 syndrome
Diagnosis	Chest X-ray, CT scan, biopsy
Differential diagnosis	Congenital pulmonary airway malformation, bronchogenic cyst, neuroblastoma
Prevention	N/A
Treatment	Surgery, chemotherapy, radiation therapy
Medication	N/A
Prognosis	Depends on type and stage; generally poorer for Type III
Frequency	Rare
Deaths	N/A

Pleuropulmonary blastoma (PPB) is a rare and malignant type of lung cancer that primarily affects children. It is characterized by the presence of cysts or solid tumors in the lungs or pleura.

Signs and Symptoms

The symptoms of pleuropulmonary blastoma can vary greatly depending on the type and stage of the disease. Common symptoms include a persistent cough, difficulty breathing, chest pain, and recurrent lung infections. In some cases, the disease may be asymptomatic and only discovered during a routine medical examination.

Causes

The exact cause of pleuropulmonary blastoma is unknown. However, it is believed to be associated with certain genetic mutations. In particular, mutations in the DICER1 gene have been linked to an increased risk of developing this disease.

Diagnosis

Diagnosis of pleuropulmonary blastoma typically involves a combination of physical examination, imaging tests such as CT scans or MRI, and biopsy. The definitive diagnosis is made by histopathological examination of the tumor tissue.

Treatment

Treatment for pleuropulmonary blastoma usually involves a combination of surgery, chemotherapy, and radiation therapy. The choice of treatment depends on the type and stage of the disease, as well as the patient's overall health.

Prognosis

The prognosis for pleuropulmonary blastoma varies widely depending on the type and stage of the disease. Early detection and treatment can significantly improve the prognosis.

See Also

• Lung cancer
• Pediatric oncology
• DICER1 syndrome

References

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