Multimodality treatment including whole pleura radiation therapy for DICER1-associated pediatric pleuropulmonary blastoma

Caressa Hui; Danielle H Shin; Adrienne Wakeling; Sarah S Donaldson; Florette K Hazard; Arun Rangaswami; Susan M Hiniker

doi:10.1002/pbc.29004

Multimodality treatment including whole pleura radiation therapy for DICER1-associated pediatric pleuropulmonary blastoma

Pediatr Blood Cancer. 2021 Jun;68(6):e29004. doi: 10.1002/pbc.29004. Epub 2021 Mar 10.

Authors

Caressa Hui¹, Danielle H Shin², Adrienne Wakeling³, Sarah S Donaldson¹, Florette K Hazard⁴, Arun Rangaswami⁵, Susan M Hiniker¹

Affiliations

¹ Department of Radiation Oncology, Stanford University, Stanford, California, USA.
² Department of Pediatric Hematology Oncology, Stanford University, Stanford, California, USA.
³ Department of Cancer Genetic Counseling, University of California San Francisco, San Francisco, California, USA.
⁴ Department of Pathology, Stanford University, Stanford, California, USA.
⁵ Department of Pediatric Hematology Oncology, University of California San Francisco, San Francisco, CA, USA.

PMID: 33751747
DOI: 10.1002/pbc.29004

Abstract

Limited data are available regarding radiation therapy in pediatric pleuropulmonary blastoma (PPB). We report the case of a 3-year-old girl with type II PPB successfully treated with trimodality therapy including multiagent chemotherapy, resection, and whole pleura radiation therapy. While longer follow-up is required to confirm ultimate local tumor control and long-term post-treatment sequelae, currently 3.5 years following therapy, she is well, without recurrent disease or observable toxicity. The goal of this report is to add our experience to the literature regarding PPB, its management, and treatment, as prospective randomized controlled trials are not feasible due to the rarity of this disease.

Keywords: pediatric oncology; pleuropulmonary blastoma; radiotherapy.

MeSH terms

Antineoplastic Agents / therapeutic use
Antineoplastic Combined Chemotherapy Protocols / therapeutic use
Child, Preschool
Combined Modality Therapy
DEAD-box RNA Helicases / genetics*
Dactinomycin / therapeutic use
Doxorubicin / therapeutic use
Female
Frameshift Mutation / genetics
Humans
Ifosfamide / therapeutic use
Lung Neoplasms / genetics*
Lung Neoplasms / therapy*
Pulmonary Blastoma / diagnosis
Pulmonary Blastoma / genetics*
Pulmonary Blastoma / therapy*
Radiotherapy, Conformal / methods
Ribonuclease III / genetics*
Vincristine / therapeutic use

Substances

Antineoplastic Agents
Dactinomycin
Vincristine
Doxorubicin
DICER1 protein, human
Ribonuclease III
DEAD-box RNA Helicases
Ifosfamide

Supplementary concepts

Pleuropulmonary blastoma