Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature
In 2002, due to extensive histomorphologic, immunohistochemical, and cytogenetic similarities, the World Health Organization unified undifferentiated small round blue cell neoplasms of soft tissue and bone (previously segregated as Ewing sarcoma or Primitive Neuroectodermal tumor) into one category: Ewing family of tumors (EFT). Osseous EFT are more common, and while extra-osseous EFT can occur anywhere in the body, those of the pancreas are rare and likely to be seen in the second decade of life in the head of the pancreas. We report the case of a 39-year-old Caucasian male with a large heterogeneously enhancing mass in the pancreatic body. Pathologic examination showed a malignant round blue cell tumor diffusely positive for CD99, chromogranin, and synaptophysin; Ki-67 proliferation index was greater than 80%. FISH showed EWSR1 gene rearrangement in 90% of cells and Archer FusionPlexTM-targeted RNA sequencing analysis identified the EWSR1-FLI1 fusion transcript. The diagnosis of EFT of the pancreas was rendered. Unfortunately, the patient had minimal improvement and was transitioned to oral pain medications to continue care at a different institution.
KeywordsEwing sarcoma Ewing family of tumors Pancreas Molecular analysis FISH
The authors would like to thank Dr. Meera Hameed from the Department of Pathology, Memorial Sloan Kettering Cancer Center, NY, for her diagnostic expertise.
Compliance with ethical standards
The study was conducted in accordance with the informed consent law of the USA.
Conflict of interest
The authors declare that they have no conflict of interest.
- 1.Stout A (1918) Tumor of the ulnar nerve. Proc NY Pathol Soc 18(2):12Google Scholar
- 2.Ewing J (1921) Diffuse endothelioma of bone. Proc NY Pathol Soc 21(17):24Google Scholar
- 7.Goldblum JR, Folpe A, Weiss SW (2014) Chapter 33: malignant soft tissue tumors of uncertain type. In: Enzinger and Weiss’s soft tissue tumors. Elsevier/Saunders, p 1028–112Google Scholar
- 8.Kumar V, Abbas A, Aster JC (2015) Chapter 26: bones, joints, and soft tissue tumors. In: Robbins and Cotran pathologic basis of disease. Elsevier/Saunders, Philadelphia, p 1179–226Google Scholar
- 9.Golhar A, Ray S, Haugk B, Singhvi SK (2017) Cytogenetically confirmed primary Ewing’s sarcoma of the pancreas. BMJ Case Rep. https://doi.org/10.1136/bcr-2017-219219
- 10.Nishizawa N, Kumamoto Y, Igarashi K, Nishiyama R, Tajima H, Kawamata H, Kaizu T, Watanabe M (2015) A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature. Surg Case Rep 1:80. https://doi.org/10.1186/s40792-015-0084-7 CrossRefPubMedPubMedCentralGoogle Scholar
- 12.Jayant K, Agrawal S, Agarwal RK, Dayama K (2016) A review of a rare entity in pancreas-extraosseous Ewing’s sarcoma. J Pancreas (Online) 17(6):574–579Google Scholar
- 17.Doi H, Ichikawa S, Hiraoka A, Ichiryu M, Nakahara H, Ochi H, Tanabe A, Kodama A, Hasebe A, Miyamoto Y, Ninomiya T, Horiike N, Takamura K, Kawasaki H, Kameoka C, Kan M, Doi S, Soga Y, Tamura H, Maeda T, Asaki A, Seno S, Iguchi H, Hasegawa T (2009) Primitive neuroectodermal tumor of the pancreas. Intern Med 48:329–333CrossRefPubMedGoogle Scholar
- 20.Mao Y, Sang X, Liang N, Yang H, Lu X, Yang Z, du S, Xu Y, Zhao H, Zhong S, Huang J, Millis JM (2013) Peripheral primitive neuroectodermal tumors arising in the pancreas: the first case report in Asia and a review of the 14 total reported cases in the world. Hepatobiliary Surg Nutr 2:51–60. https://doi.org/10.3978/j.issn.2304-3881.2012.08.01 PubMedPubMedCentralCrossRefGoogle Scholar
- 21.Maxwell L, Hederman A, Jackson C, Sawaya D, Giles H, Nowicki MJ (2011) Uncommon presentation of rare disorder-duodenal ulcer secondary to invasive pancreatic primitive neuroectodermal tumor: case report and review of the literature. J Pediatr Hematol Oncol 33:543–548. https://doi.org/10.1097/MPH.0b013e31821041af CrossRefPubMedGoogle Scholar
- 29.Takeuchi M, Kuwae Y, Hamana K et al (2003) Primitive neuroectodermal tumor of the pancreas. Arch Histopathol D D 10:23–26Google Scholar
- 30.Wakao J, Ono S, Hosoi H, Tsuda T (2011) Ewing sarcoma family tumor in the pancreatic head incidentally diagnosed by abdominal trauma. Jpn Soc Pediatr Hematol/Oncol 48:7–11Google Scholar
- 35.Teixeira U, Goldoni M, Unterleider M et al (2015) Primitive neuroectodermal tumor of the pancreas; a case report and review of the literature. Case Rep Surg 276869. https://doi.org/10.1155/2015/276869