Document Type : Case Report(s)
Authors
1 Department of Pathology, King Khalid University, Abha, Saudi Arabia
2 Department of Laboratory Medicine, Aseer Central Hospital, Abha, Saudi Arabia
Abstract
Extraskeletal Ewing sarcomas (EES) is a rare, aggressive malignancy that typically affects adolescents or young adults, primarily involving the deep soft tissues of the lower extremities and paravertebral regions. The occurrence of EES in the pancreas is even rarer. These tumors are characterized by small, round cell sarcomas displaying varying degrees of neuroectodermal differentiation, as revealed through light, electron microscopy, or immunohistochemistry. Diagnosing EES demands a high level of suspicion. Histopathologically, the presence of small round cell tumors in the pancreas, along with CD99 positivity in immunohistochemistry, assists in diagnosing EES. Molecular analysis demonstrating EWSR1 (22q12) rearrangement via interface fluorescence in situ hybridization is required to confirm the diagnosis. A comprehensive review of pancreatic EES cases revealed that the primary treatment modality typically involves surgical intervention, often complemented by chemotherapy and, in some cases, radiotherapy. In this report, we describe the case of a 28-year-old male presenting with abdominal pain and a loss of appetite, which, upon histopathological and molecular examination, was identified as EES of the pancreas. The patient underwent surgical resection of the pancreatic mass, followed by omentum, splenectomy, and chemotherapy. EES is a highly aggressive tumor with an insidious onset, and patients usually exhibit non-specific clinical symptoms. Although exceedingly rare, it should be considered in the differential diagnosis of pancreatic masses.
Highlights
Sohaila Fatima (Google Scholar)
Keywords
Main Subjects
How to cite this article:
Fatima S, Osman TM, Amer S. Primary extraskeletal Ewing sarcoma of the pancreas-A case report. Middle East J Cancer. 2024;15(1):79-84. doi: 10. 30476/mejc.2023.96991.1844.
- de Alava E, Lessnick SL, Sorensen PH. Ewing sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn CW, Mertens F, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.
- de Alava E, Gerald WL. Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. J Clin Oncol. 2000;18(1):204-13. doi: 10.1200/JCO.2000.18.1.204.
- Goldblum, JR. Soft Tissue. In: Goldblum JR, Lamps LW, McKenney JK, Myers, JL, editors. Rosai and Ackerman’s surgical pathology. 11th ed. Vol. 2. Philadelphia, PA, USA: Elsevier. 2017.p.1884-5.
- Patel D, Nandu NS, Reddy A. Extraosseus Ewing's sarcoma in pancreas: a review. Cureus. 2020;12(4): e7505. doi: 10.7759/cureus.7505.
- Nemri SN, Dsa BFV, Fatima S, Haider N, Alwani NM. Extraskeletal Ewing sarcoma at rare sites with review of literature. Annals of Pathology and Laboratory Medicine. 2017;4(1): 9-13. doi: 10.21276/APALM.2017.1110.
- Goldblum JR, Weiss SW, Folpe AL. Malignant soft tissue tumors of uncertain type. In: Goldblum JR, Weiss SW, Folpe AL, editors. Enzinger and weiss soft tissue tumors. Netherlands: Elsevier Elsevier/Saunders. 2019.p. 1028–112.
- Ushigome S, Machinami R, Sorensen PH. Ewing sarcoma / Primitive neuroectodermal tumour (PNET). In: Fletcher D, Unni K, Mertens F, editors. World Health Organization Classification of Tumors. Pathology & Genetics of Tumour of Soft Tissue and Bone,3rded. Lyon; IARC Press 2002.
- de Alava E, Lessnick SL, Stamenkovic I. Ewing sarcoma. In: de Alava E, Lessnick SL, Stamenkovic I editors. The WHO Classification of Tumours Editorial Board. WHO Classification of Tumours of Soft Tissue and Bone Tumours, 5th ed. Lyon: IARC Press. 2020.
- Lüttges J, Pierré E, Zamboni G, Weh G, Lietz H, Kussmann J, et al. Malignant non-epithelial tumors of the pancreas. [In German] Pathologe. 1997;18(3):233-7. doi: 10.1007/s002920050211.
- Tan Y, Zhang H, Ma GL, Xiao EH, Wang XC. Peripheral primitive neuroectodermal tumor: dynamic CT, MRI and clinicopathological characteristics--analysis of 36 cases and review of the literature. Oncotarget. 2014;5(24):12968-77. doi: 10.18632/oncotarget.2649.
- Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348(8):694-701. doi: 10.1056/NEJMoa020890.
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