Gray Zone Lymphoma Arising in the Neck of a Teenager With a Germline Mutation in TP53. Issue 6 (August 2019)

• Record Type: Journal Article
• Authors: Gatineau-Sailliant, Sophie; Turcotte, Karine; Quintal, Marie-Claude; Turpin, Sophie; Champagne, Josette; Petrella, Tony; Roussy, Mathieu; Cellot, Sonia; Bouron-Dal Soglio, Dorothée
• Is Part Of: Journal of pediatric hematology/oncology. Volume 41:Issue 6(2019)
• Publication Date: 2019-08
• Subjects: gray zone lymphoma -- pediatric -- Li-Fraumeni syndrome; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001308
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : 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. Abstract : Supplemental Digital Content is available in the text.