Metastasis of Ewing Sarcoma to the Pancreas: Case Report and Literature Review.

Hyma Polimera,Shady Piedra Abusharar,Monali Vasekar,Jayakrishna Chintanaboina,Prashanth Moku

doi:10.1155/2020/7075048

Hyma Polimera, Shady Piedra Abusharar + Show 3 more

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https://doi.org/10.1155/2020/7075048

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Abstract

Ewing sarcoma (ES) is a highly aggressive malignant bone cancer. ES is part of the Ewing sarcoma family of tumors (ESFT), which express characteristic t(11;22) translocation as well as higher levels of CD99. Given that metastasis and tumor burden are significant prognostic factors in patient's response to treatment, prompt diagnosis is needed to effectively treat ESFT patients. However, the challenges in classifying and characterizing ESFT complicate effective management and treatment of ES. In this report, we present a rare case of ES metastasis to the pancreas. Upon review of the literature, we found 39 cases of ESFT involving the pancreas, but only 3 were metastatic to the pancreas while the remaining cases of ESFT primarily originated from the pancreas. Given the rarity of such metastasis, the positive outcome in our patient's case may explain the importance of prompt diagnosis in order to initiate appropriate treatment.

Highlights

Ewing sarcoma (ES) is highly aggressive and is the most common malignant bone cancer in children and young adults after osteosarcoma [1]
The Ewing sarcoma family of tumors are comprised of Ewing sarcoma of the bone (ESB), extraosseous Ewing sarcoma (EES), peripheral primitive neuroectodermal tumor, and Askin tumor [2]
While Ewing sarcoma is a primary malignancy of the bone, EES/peripheral primitive neuroectodermal tumor (pPNET) often originate in the soft tissue of thoraco-pulmonary, pelvic, and lower extremity regions, but rarely from the pancreas [5]

Summary

Introduction

Ewing sarcoma (ES) is highly aggressive and is the most common malignant bone cancer in children and young adults after osteosarcoma [1]. Primitive neuroectodermal tumors (PNET) have a similar t(11;22) chromosomal translocation [1]. ES and PNET are part of the Ewing sarcoma family of tumors (ESFT). Ewing sarcoma family of tumors expresses significantly higher levels of CD99 (MIC2) protein than observed in normal tissue [3]. While Ewing sarcoma is a primary malignancy of the bone, EES/pPNETs often originate in the soft tissue of thoraco-pulmonary, pelvic, and lower extremity regions, but rarely from the pancreas [5]. In this case report, we present a 29-year-old man with cytogenetically confirmed metastatic EES to the pancreas

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