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Very Long-term Sequelae After Nonradical Surgery Combined With Brachytherapy in an Infant With a Chemotherapy-resistant Rhabdomyosarcoma of the Tongue

van Rijswijk, Petra M. MD*; van den Heuvel-Eibrink, Marry M. MD, PhD*; van den Akker, Erica L.T. MD, PhD; Slagter, Cleo MD; Lequin, Maarten H. MD, PhD*,§; Aarsen, Femke K. PhD; van Atteveld, Jenneke E. Msc*; Wagner, Anja MD, PhD; van Grotel, Martine MD, PhD*

Journal of Pediatric Hematology/Oncology: October 2017 - Volume 39 - Issue 7 - p 566–569
doi: 10.1097/MPH.0000000000000935
Clinical and Laboratory Observations

In 2003, van Grotel and colleagues reported an infant suffering a chemotherapy-resistant eRMS of the tongue, that was treated with subtotal tumor resection and brachytherapy after major medical ethical discussions. As no long-term sequelae of such a procedure have been described, perspectives were uncertain at that time. Now, after 15 years, we describe hypoplasia of the mandibula, compromised dentation, osteopenia, neuropsychological deficits, and moderate speech impairment as the most prominent late effects. Also, mandibular cysts and basal cell carcinomas in the irradiated area, eventually led to the diagnosis Gorlin syndrome.

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*Princess Máxima Center for Pediatric Oncology

§Department of Radiology, University Medical Center Utrecht, Utrecht, the Netherlands

Department of Pediatrics, Erasmus MC-Sophia Children’s Hospital

Department of Radiotherapy, Erasmus MC

Department of Pediatric Oncology, Erasmus MC-Sophia Children’s Hospital

Department of Clinical Genetics, Erasmus MC, Rotterdam, the Netherlands

The authors declare no conflict of interest.

Reprints: Martine van Grotel, MD, PhD, Consultant Pediatric Oncologist, Princess Máxima Center for Pediatric Oncology, Lundlaan 6, 3584 EA Utrecht, The Netherlands (e-mail:

Received August 2, 2016

Accepted July 10, 2017

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