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Gray Zone Lymphoma Arising in the Neck of a Teenager With a Germline Mutation in TP53

Gatineau-Sailliant, Sophie MD*; Turcotte, Karine MD, PhD; Quintal, Marie-Claude MD; Turpin, Sophie MD§; Champagne, Josette MD*; Petrella, Tony MD; Roussy, Mathieu BSc; Cellot, Sonia MD, PhD; Bouron-Dal Soglio, Dorothée MD, PhD

Journal of Pediatric Hematology/Oncology: August 2019 - Volume 41 - Issue 6 - p e405–e408
doi: 10.1097/MPH.0000000000001308
Online Articles: Clinical and Laboratory Observations

Gray zone lymphoma is an aggressive disease for which appropriate management is still debated. We report a 15-year-old girl with a cervical mass, an enlarged ipsilateral tonsil, and anemia. Both sites showed hypermetabolism on F18-FG positron emission tomography/CT. Surgical resection was diagnostic of Epstein-Barr virus–negative gray zone lymphoma cervical and tonsillar involvement. No abnormality was found in cytogenetic analysis on tumor cells. However, exome sequencing in peripheral blood DNA revealed a germline mutation in TP53. Complete response was achieved after surgery and 6 cycles of rituximab with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin regimen.

Departments of *Haematology-Oncology

Anatomical Pathology

Pediatric Surgery

§Nuclear Medicine

Charles Bruneau Cancer Center, University Hospital Center Ste-Justine, Research Center Montreal University

Department of Anatomical Pathology, Maisonneuve Hospital, Montréal, QC, Canada

The authors declare no conflict of interest.

Reprints: Dorothée Bouron-Dal Soglio, MD, PhD, Department of Anatomical Pathology, University Hospital Center Ste-Justine, 3175 Chemin de la Côte-Sainte-Catherine, Montréal, QC, Canada, H3T 1C4 (e-mail:

Received March 7, 2018

Accepted June 1, 2018

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