Pediatric Ewing’s Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report

다국어 초록 (Multilingual 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.

참고문헌 (Reference)

1 Peng L, "Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of the literature" 9 : 1299-1303, 2015

2 Li T, "Primary Ewing's sarcoma/primitive neuroectodermal tumor of the ileum: case report of a 16-year-old Chinese female and literature review" 12 : 37-, 2017

3 Horie Y, "Peripheral primitive neuroectodermal tumor of the small bowel mesentery: a case showing perforation at onset" 50 : 398-403, 2000

4 김준미, "Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study" 대한병리학회 47 (47): 77-81, 2013

5 Maki RG, "Pediatric sarcomas occurring in adults" 97 : 360-368, 2008

6 de Alava E, "Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family" 18 : 204-213, 2000

7 Padma M, "Extraskeletal Ewing’s sarcoma of the small bowel" 2 : 645-647, 2015

8 Kim DW, "Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the small bowel: a rare cause of intestinal obstruction" 22 : 1137-1138, 2007

9 Bernstein M, "Ewing's sarcoma family of tumors: current management" 11 : 503-519, 2006

10 Ludwig JA, "Ewing sarcoma: historical perspectives, current state-of-the-art, and opportunities for targeted therapy in the future" 20 : 412-418, 2008

11 Milione M, "Ewing sarcoma of the small bowel: a study of seven cases, including one with the uncommonly reported EWSR1-FEV translocation" 64 : 1014-1026, 2014

12 Maheshwari AV, "Ewing sarcoma family of tumors" 18 : 94-107, 2010

13 Somers GR, "Ewing family tumours: a paediatric perspective" 20 : 49-55, 2014

14 Javery O, "A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses" 197 : W1015-W1022, 2011

15 김기환, "38세 여자의 공장에서 발생한 원시신경외배엽종양 1예" 대한내과학회 75 (75): 237-241, 2008