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Successful Treatment of Recurrent Li-Fraumeni Syndrome-related Choroid Plexus Carcinoma

McEvoy, Matthew MD*,†; Robison, Nathan MD; Manley, Peter MD§; Yock, Torunn MD; Konopka, Kristine MD; Brown, Robert E. MD#; Wolff, Johannes MD, PhD**; Green, Adam L. MD*,†

Journal of Pediatric Hematology/Oncology: November 2017 - Volume 39 - Issue 8 - p e473–e475
doi: 10.1097/MPH.0000000000000965
Online Articles: Clinical and Laboratory Observations

The management of choroid plexus carcinoma (CPC) is challenging and multifaceted. Here, we discuss a 3-year-old girl with CPC and Li-Fraumeni syndrome who achieved full remission after surgery and chemotherapy, with radiation therapy spared. At recurrence, we used a novel, standard-dose cytotoxic chemotherapy regimen, focal proton radiation therapy, and targeted agents based on morphoproteomic analysis to achieve long-term survival. We highlight the rationale for our therapy at recurrence, as well as the risk-benefit analyses necessary in decision making for these patients. Our strategy may be effective in managing other patients with recurrent CPC and Li-Fraumeni syndrome.

*Department of Pediatrics, University of Colorado School of Medicine

Center for Cancer and Blood Disorders, Children’s Hospital Colorado, Aurora, CO

Pediatric Neuro-Oncology, Children’s Hospital Los Angeles, Los Angeles, CA

§Department of Pediatric Oncology, Dana-Farber Cancer Institute

Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA

Department of Pathology, University of Michigan School of Medicine, Ann Arbor, MI

#Department of Pathology, UTHealth McGovern Medical School, Houston, TX

**AbbVie, Chicago, IL

The authors declare no conflict of interest.

Reprints: Adam L. Green, MD, University of Colorado Anschutz Medical Campus, 12800 E. 19th Avenue, Mail Stop 8302, Aurora, CO 80045 (e-mail:

Received December 16, 2016

Accepted July 24, 2017

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