PICTURES IN DIGESTIVE PATHOLOGY

 

Duodenal Ewing's sarcoma: Unusual location and atypical EWRS-1 translocation

Sarcoma de Ewing duodenal: localización inusual y traslocación atípica EWRS-1

 

 

Ernesto Jesús Barzola-Navarro¹, José Ángel Flores-García¹, Diego López-Guerra¹, Cristina Tejera-Pérez², Nerea Rodríguez-Díez³, Aurea Gómez-Durán⁴, Alejandro Rubio-Fernández⁴ and Gerardo Blanco-Fernández¹

¹ Hepatobiliary and Pancreatic Surgery Unit, ² Endocrine Unit, ³ Digestive Unit, and ⁴ Pathology Unit. Hospital Universitario de Badajoz. Badajoz, Spain

 

 

Case report

A 20-year-old woman was admitted, because 5 months before admission, she started having epigastric abdominal pain and lost weight. On examination the patient was pale, had abdominal pain when deep palpation was applied.

In laboratory tests, a microcytic hypochromic anemia was found. Results of computed tomography (CT) of the abdomen showed a rounded heterogeneous mass adjacent to the pancreas (Fig. 1).

 

 

An oral endoscopy revealed in the fourth duodenum portion a neoplastic appearance of an ulcer, where a biopsy was taken (Fig. 2). The pathological and immunohistochemistry studies showed neoplastic cells positive for CD99, FLI-1 (friend leukemia integration 1 transcription factor), and CD 117. With a suspected sarcoma, a molecular study of traslocation t (11; 22), (q24; q12) in the locus 22q12 of EWSR1 gene (Ewing sarcoma breakpoint region 1) was done (Fig. 3). These findings were compatible with Ewing's sarcoma (ES).

 

 

 

The surgery performed was cephalic pancreaticoduodenectomy (Fig. 4).

 

 

After that, the adjuvant chemotherapy was administered. After 9 months of follow up treatment the patient has recovered.

 

Discussion

Only a few cases of gastrointestinal location of ES have been reported, being mostly located in the small intestine and more frequently in young people (1). The symptoms are aggravated by duodenal compression and ulcers. Total surgical removal is the best solution for a full recovery (2).

Systematic use of chemotherapy in ES in the last two decades had increased the survival level (3). ES is a radiotherapy sensitive tumor and this treatment is applied in cases of affected margins.

 

References

1. Kie JH, Lee MK, Kim CJ, Kwon K-W, Yang W-I. Primary Ewing's sarcoma of the duodenum: A case report. Int J Surg Pathol 2003;11:331-7.         [ Links ]

2. Tural D, Molinas Mandel N, Dervisoglu S, Oner Dincbas F, Koca S, Colpan Oksuz D, et al. Extraskeletal Ewing's sarcoma family of tumours in adults: Prognostic factors and clinical outcome. Jpn J Clin Oncol 2012;42:420-6.         [ Links ]

3. Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, et al. Primary disseminated multifocal Ewing sarcoma. Results of the Euro-EWING 99 Trial. J Clin Oncol 2010;28:3284-91.         [ Links ]