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5 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Pleuropulmonary blastoma is a rare lung cancer of young children, and the signature tumour of the inherited DICER1 syndrome. It starts as a lung cyst (type I) that can turn into a solid, aggressive tumour (types II and III). Cysts are removed surgically with an excellent outlook; solid tumours need chemotherapy as well, and every family is offered DICER1 testing.
Pleuropulmonary blastoma is a dysontogenetic mesenchymal tumour of the lung and pleura of early childhood with three subtypes on a progression pathway, from type I (purely cystic) to type II (cystic and solid) and type III (solid), plus type Ir, a regressed cystic form; a germline DICER1 mutation is the cause in the majority (Messinger 2015). In the 350 registry-confirmed cases (85 of 435 submitted cases, 20 percent, proved to be another entity on review), five-year overall survival was 91 percent for type I and Ir, with all deaths in that group from progression to type II or III, and survival was significantly better for type II than type III (Messinger 2015). The DICER1 syndrome also produces ovarian Sertoli-Leydig cell tumours, cystic nephroma, thyroid nodules and other tumours in the same children and families (Gynecologic Oncology 2011).
How it differs from its parent: the parent page is the corpus's umbrella for all childhood lung and airway tumours; this page is the entity itself, with its subtype pathway, its registry survival figures and its syndrome. Adult pulmonary blastoma is a different tumour with its own page.
| Setting | Approach | Guideline |
|---|---|---|
| Type I and Ir | Complete resection of the cyst; chemotherapy debated; DICER1 testing and surveillance. | not mapped |
| Type II and III | Resection and multi-agent chemotherapy (ifosfamide, vincristine, actinomycin, doxorubicin), radiotherapy for residual disease. | not mapped |