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Pediatric Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report
Clin Pediatr Hematol Oncol 2017;24:162-8.
Published online October 31, 2017
© 2017 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

You Sun Kim, M.D.1, Hye Min Moon, M.D.1, Kyu Sang Lee, M.D.2, Young Suk Park, M.D.3, Hyun-Young Kim, M.D.4, Ji Young Kim, M.D.5, Jin Min Cho, M.D.6 and Hyoung Soo Choi, M.D.6

1Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Departments of 2Pathology and 3Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, 4Department of Surgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Departments of 5Radiology and 6Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
Correspondence to: Hyoung Soo Choi
Department of Pediatrics, Seoul National University Bundang Hospital, 82 Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam 13620, Korea
Tel: +82-31-787-7797 Fax: +82-31-787-4054 E-mail: choihs1786@snubh.org
Received August 29, 2017; Revised September 18, 2017; Accepted September 26, 2017.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Although it may develop in any organs, ES/PNET originating from small intestine is exceedingly rare. We experi-enced a 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia. Imaging work-up showed multiple masses in the small bowel and omentum with disseminated peritoneal seeding nodules, indicating lymphoma as the most likely diagnosis. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising small round cells with CD99 expression and EWS-FLI1 translocation leading to the diagnosis of ES/PNET. Tumor burden decreased gradually during five con-secutive cycles of systemic chemotherapy. The patient received segmental resection of jejunum, followed by adjuvant chemotherapy. This is the first pediatric case of ES/PNET found in small intestine in Korea.
Keywords: Ewing sarcoma, Primitive neuroectodermal tumor, Small intestine, Pediatrics
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