Pediatric Ewing's Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report
10.15264/cpho.2017.24.2.162
- Author:
You Sun KIM
1
;
Hye Min MOON
;
Kyu Sang LEE
;
Young Suk PARK
;
Hyun Young KIM
;
Ji Young KIM
;
Jin Min CHO
;
Hyoung Soo CHOI
Author Information
1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Ewing sarcoma;
Primitive neuroectodermal tumor;
Small intestine;
Pediatrics
- MeSH:
Abdominal Pain;
Anemia, Iron-Deficiency;
Biopsy;
Chemotherapy, Adjuvant;
Child;
Diagnosis;
Drug Therapy;
Female;
Humans;
Intestine, Small;
Jejunum;
Korea;
Lymphoma;
Melena;
Neural Plate;
Neuroectodermal Tumors;
Neuroectodermal Tumors, Primitive;
Omentum;
Pediatrics;
Sarcoma, Ewing;
Tumor Burden
- From:Clinical Pediatric Hematology-Oncology
2017;24(2):162-168
- CountryRepublic of Korea
- Language:English
-
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 experienced 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 consecutive 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.
