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Chemoradiotherapy for Unresectable INI1-negative Chordoma in a Child

Miyahara, Hiroyuki MD*; Nodomi, Seishiro MD*; Umeda, Katsutsugu MD; Itasaka, Satoshi MD; Waki, Kenji MD*; Imai, Tsuyoshi MD*

Journal of Pediatric Hematology/Oncology: October 02, 2018 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001318
Clinical and Laboratory Observations: PDF Only

The characteristics of chordomas in children are distinct from those in adults. In particular, the prognosis of patients with INI1-negative chordoma is dismal. The standard treatment for localized chordoma, complete surgical resection with a wide margin, is seldom feasible for chordomas arising at the clivus in children, mainly due to associated complications. Therefore, other treatments for unresectable chordomas in children, including chemoradiotherapy, must be explored. Here, we report a 7-year-old girl with an INI1-negative chordoma of the clivus, who responded to conventional chemotherapy plus radiotherapy. Without surgical resection, she remains alive after 1 year and 7 months of the initial diagnosis.

*Department of Pediatrics, Kurashiki Central Hospital

Department of Radiation Oncology, Kurashiki Central Hospital, Okayama

Department of Pediatrics, Kyoto University, Kyoto, Japan

Parents provided informed consent for the publication of photographs under the guidelines of the Medicine Ethics Committee in Kurashiki Central Hospital.

The authors declare no conflict of interest.

Reprints: Seishiro Nodomi, MD, 1-1-1 Miwa, Kurashiki City, Okayama 710-8602, Japan (e-mail:

Received May 1, 2018

Accepted September 4, 2018

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