The treatment of DICER1 syndrome must always be based on the tumor disease present in the patient. For some tumors, the age of the patient also plays a decisive role in the choice of therapy. This often consists of an initial surgical therapy followed by radiation and/or chemotherapy.
Pleuropulmonary blastomas are first surgically removed as completely as possible. This is followed by chemotherapy, the intensity of which depends on the type of PPB. 5 years after diagnosis, 89% of patients with type I PPB still live, 74% with type II and 53% with type III. Type Ir PPB, on the other hand, has a survival rate of 100% after 5 years.
In the case of tumors of the ovaries (Sertoli-Leydig cell tumor, juvenile granulosa cell tumor and gynandroblastoma), depending on the stage of the disease, chemotherapy is sometimes carried out prior to surgical treatment. The prognosis for patients with these tumors is generally very good.
Cystic nephromas usually have to be treated surgically. If a kidney is interspersed with many cysts, it may be necessary to remove it completely.