Rare. Embryonic tumor of the lung or pleura, often associated with a mutation of the DICER1 gene (see this term).

Four types have been described:

I:        cystic tumors that can become malignant in childhood (median age < 2 years): respiratory distress, pneumothorax, sometimes discovered by chance

IC:        cystic tumors without malignant elements

II:        malignant cystic tumor (median age: 35 months)

III:        malignant solid tumors (median age: 41 months).

Types II and III forms may present with fever, dyspnea, pneumothorax or pleural effusion, and are often associated with metastases, in particular cerebral, already present when the diagnosis is made.

Anesthetic Implications:

according to the tumor and its localization; thoracotomy; single-lung ventilation

References :

-        Robertson JC, Jorcyk CL, Oxford  JT.
DICER1 syndrome: DICER1 mutations in rare cancers.
Cancers 2018; 10: 143; doi: 10.3390/cancers10050143

Updated: April 2019