• Ana M Fita, Esther Llinares-Riestra, Ernesto Doménech-Abellán, Mar Bermúdez-Cortés, Ana M Galera-Miñarro, Agueda Bas-Bernal, and José L Fuster-Soler.
• Department of Pediatric Hematology and Oncology, University Hospital Virgen de la Arrixaca, Murcia, Spain.
• Pediatr Blood Cancer. 2017 Aug 1; 64 (8).

AbstractPleuropulmonary blastoma (PPB) is a rare malignancy of childhood. It often represents a manifestation of a hereditary tumor predisposition syndrome (DICER1 syndrome). Because of its malignant potential, surgical resection of cystic lung lesions is recommended in germline DICER1 mutation carriers. We present a case of a 3-year-old male child with type III PPB successfully managed with ifosfamide, vincristine, actinomycin-D, and doxorubicin (IVADo) chemotherapy and surgery. A heterozygous germline pR688X mutation of DICER1 gene was demonstrated. Six years after primary diagnosis, several small lung cysts remained stable without further therapy. The management of residual asymptomatic lung cysts represents a clinical challenge in these patients.© 2017 Wiley Periodicals, Inc.

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