Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review

Abstract

Ewing's Sarcoma (ES) is an rare, small round-cell sarcoma that predominantly occurs in children and young adults, with both skeletal and extraskeletal manifestations. However, pancreatic ES, due to its rarity, is infrequently featured in scholarly literature, with only a scant 43 reported instances. Our study describes a case of pancreatic ES in an 8-year-old boy who was found to have an abdominal mass. Following an exhaustive examination, the boy was diagnosed with a neoplasm in the pancreatic head and underwent a complex surgical procedure encompassing pancreatoduodenectomy and partial transverse colectomy. Immunohistochemical assays confirmed the neoplastic cells' positivity for Cluster of Differentiation 99(CD99), Vimentin, and NK2 Homeobox 2(NKX2.2), while genomic testing identified an EWSR1-FLI1(Ewing Sarcoma Breakpoint Region 1-Friend Leukemia Integration 1) gene fusion. This led to a conclusive diagnosis of pancreatic Ewing's Sarcoma. The patient underwent seven cycles of adjuvant chemotherapy, alternating between VDC (Vincristine, Doxorubicin, Cyclophosphamide) and IE (Ifosfamide, Etoposide) tri-weekly, but did not undergo radiotherapy. At present, the patient remains neoplasm-free. Through our case analysis and comprehensive review of the existing literature, we aim to underscore th rarity of pancreatic Ewing's sarcoma and to highlight the efficacy of our individualized therapeutic approach.

Keywords: Ewing sarcoma; child; pancreatic neoplasms; pancreaticoduodenectomy; small round cell malignant tumor.

Figure 1

Abdominal computed tomography scan + enhancement show a primarily solid, mixed-density mass in the pancreatic head area, with significant enhancement of the solid component, interspersed low-attenuation foci, and visible tortuous small vessels within. (A) Pre-contrast phase; (B) Artery stage; (C) Portal vein stage. : Tumor; : Visible tortuous small vessels; : Interspersed low-attenuation foci.

Figure 2

(A) The positional relationship between the tumor and the artery. ↓: Celiac trunk; ➨: Superior mesenteric artery; ▲: Right renal artery. (B) The positional relationship between the tumor and the portal vein. ↓: Portal vein; :Superior mesenteric vein. (C) The tumor’s positional relationship with the inferior vena cava and right renal vein.↓: Right renal vein; ➨: Inferior vena cava. (D) The positional relationship between the tumor and the adjacent organs. : Duodenum; ➨: Right kidney.

Figure 3

Postoperative gross specimen.

Figure 4

Gross resection of the visible tumor. : Portal vein; : Common hepatic artery. : Superior mesenteric vein. The pancreatic cut surface after tumor resection.

Figure 5

(A) Small round-cell malignant tumor (HE,100×). (B) Positive immunohistochemical staining for CD99(100×). (C) Positive immunohistochemical staining for NKX2.2 (100×). (D) Positive immunohistochemical staining for Syn (100×).