Successful Treatment of Recurrent Li-Fraumeni Syndrome-related Choroid Plexus Carcinoma. Issue 8 (November 2017)

• Record Type: Journal Article
• Authors: McEvoy, Matthew; Robison, Nathan; Manley, Peter; Yock, Torunn; Konopka, Kristine; Brown, Robert E.; Wolff, Johannes; Green, Adam L.
• Is Part Of: Journal of pediatric hematology/oncology. Volume 39:Issue 8(2017)
• Publication Date: 2017-11
• Subjects: choroid plexus carcinoma -- Li-Fraumeni syndrome -- proton radiation -- morphoproteomics -- long-term survival; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000000965
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : 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.