Clin Pediatr Hematol Oncol.  2017 Oct;24(2):162-168. 10.15264/cpho.2017.24.2.162.

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

Affiliations
  • 1Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
  • 2Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 3Department of Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 4Department of Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
  • 5Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 6Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea. choihs1786@snubh.org

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.

Keyword

Ewing sarcoma; Primitive neuroectodermal tumor; Small intestine; Pediatrics

MeSH Terms

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
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