Subscribe to RSS
Download PDF
DOI: 10.1055/s-0043-1764148
Ewing Sarcoma with Ganglion Cells Post-Chemotherapy: A Case Report with Review of Literature
Funding None.
Abstract
Ewing sarcoma arises in both bones (most common) and soft tissues and it commonly affects young adults. The tumor is composed of small round cells showing positivity for CD99 and FLI1 on immunohistochemistry (IHC). We describe ganglion cell differentiation post-chemotherapy in Ewing sarcoma which is a rare phenomenon.
A 13-year-old girl presented with a chest wall mass. On biopsy correlating with IHC, the diagnosis was rendered as Ewing sarcoma. She underwent neoadjuvant chemotherapy followed by resection of the tumor. On microscopic evaluation, the tumor showed prominent ganglionic differentiation with expression of neuronal markers.
Although maturation post-chemotherapy is an established finding with better prognosis in other primitive pediatric tumors, such neural differentiation is rare with only a few case reports in Ewing sarcoma both post- and pre-chemotherapy. Clinical significance and prognosis of such differentiation which appear to be better are not yet established and needs to be elucidated.
Publication History
Article published online:
01 March 2023
© 2023. MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
- 1 Endo M, Mertens F, Miettinen M. Ossifying fibromyxoid tumor. In WHO Classification of Tumors of Soft Tissue and Bone. Lyon, France: IARC Press; 2020: 274-276
- 2 Amin MB, Edge S, Greene F. et al, Eds. AJCC Cancer Staging Manual 8th edition. Chicago: Springer International Publishing; 2017
- 3 Lloyd R, Osamura R, Kloppel G, Rosai J. WHO Classification of Tumours of Endocrine Organs. 4th ed. Lyon, France: IARC Press; 2017
- 4 Weissferdt A, Kalhor N, Moran CA. Ewing sarcoma with extensive neural differentiation: a clinicopathologic, immunohistochemical, and molecular analysis of three cases. Am J Clin Pathol 2015; 143 (05) 659-664
- 5 Maeda G, Masui F, Yokoyama R. et al. Ganglion cells in Ewing's sarcoma following chemotherapy: a case report. Pathol Int 1998; 48 (06) 475-480
- 6 Erdoğan KE, Deveci MA, Hakkoymaz ZR, Gönlüşen G. Therapy-induced neural differentiation in Ewing's sarcoma: a case report and review of the literature. Turk Patoloji Derg 2019; 35 (02) 139-143
- 7 Salet MCW, Vogels R, Brons P, Schreuder B, Flucke U. Maturation toward neuronal tissue in an Ewing sarcoma of bone after chemotherapy. Diagn Pathol 2016; 11 (01) 74
- 8 Collini P, Mezzelani A, Modena P. et al. Evidence of neural differentiation in a case of post-therapy primitive neuroectodermal tumor/Ewing sarcoma of bone. Am J Surg Pathol 2003; 27 (08) 1161-1166
- 9 Ushigome S, Shimoda T, Nikaido T. et al. Primitive neuroectodermal tumors of bone and soft tissue. With reference to histologic differentiation in primary or metastatic foci. Acta Pathol Jpn 1992; 42 (07) 483-493
- 10 von Levetzow C, Jiang X, Gwye Y. et al. Modeling initiation of Ewing sarcoma in human neural crest cells. PLoS One 2011; 6 (04) e19305