We experienced a case of small, round-cell malignant neoplasm diagnosed by touch smear cytology and histopathology when an open biopsy was performed in a 50-year-old Japanese woman. She was suspected of having a cervical spine tumor after surgery for cervical spine foraminal stenosis. After consent, the cervical spine tumor histologically diagnosed by an open biopsy was confirmed to be Ewing sarcoma (EWS) by genetic testing. EWS belongs to a group of small, round-cell tumors that are morphologically similar and often difficult to differentiate. After the open biopsy, the present patient received radiotherapy, and her plasma level of Pro-Gastrin-Releasing-Peptide was decreased (217.2 pg/ml before surgery to 30.3 pg/ml; reference value: 0 - 80 pg/ml). We herein report the process for making the final diagnosis by focusing on the intraoperative cytology, histopathology, and immunohistochemical findings. Our diagnosis was validated by karyotyping and a fluorescence in-situ hybridization analysis.
References
[1]
Hesla, A.C., Papakonstantinou, A. and Tsagkozis, P. (2021) Current Status of Management and Outcome for Patients with Ewing Sarcoma. Cancers, 13, 1202. https://doi.org/10.3390/cancers13061202
[2]
Khan, S., Abid, Z., Haider, G., Bukhari, N., Zehra, D., Hashmi, M., Abid, M. and Ibrahim, U. (2021) Incidence of Ewing’s Sarcoma in Different Age Groups, Their Associated Features, and Its Correlation With Primary Care Interval. Cureus, 13, e13986. https://doi.org/10.7759/cureus.13986
[3]
Cotterill, S.J., Ahrens, S., Paulussen, M., Jurgens, H.F., Voute, P.A., Gadner, H. and Craft, A.W. (2000) Prognostic Factors in Ewing’s Tumor of Bone: Analysis of 975 Patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. Journal of Clinical Oncology, 18, 3108-3114. https://doi.org/10.1200/JCO.2000.18.17.3108
[4]
Esiashvili, N., Goodman, M. and Marcus Jr., R.B. (2008) Changes in Incidence and Survival of Ewing Sarcoma Patients over the Past 3 Decades: Surveillance Epidemiology and End Results Data. Journal of Pediatric Hematology/Oncology, 30, 425-430. https://doi.org/10.1097/MPH.0b013e31816e22f3
[5]
Stahl, M., Ranft, A., Paulussen, M., Bolling, T., Vieth, V., Bielack, S., Gortitz, I., Braun-Munzinger, G., Hardes, J., Jurgens, H. and Dirksen, U. (2011) Risk of Recurrence and Survival after Relapse in Patients with Ewing Sarcoma. Pediatric Blood & Cancer, 57, 549-553. https://doi.org/10.1002/pbc.23040
[6]
de Alava, E., Lessnick, S.L. and Stamenkovic, I. (2020) Undifferentiated Small, Round-Cell Sarcomas of Bone and Soft Tissue. Ewing Sarcoma. In: Bridge, J.A., Ed., WHO Classification of Tumours: Soft Tissue and Bone Tumours, International Agency for Research on Cancer (IARC), Lyon.
[7]
Paulussen, M., Ahrens, S., Dunst, J., Winkelmann, W., Exner, G.U., Kotz, R., Amann, G., Dockhorn-Dworniczak, B., Harms, D., Muller-Weihrich, S., Welte, K., Kornhuber, B., Janka-Schaub, G., Gobel, U., Treuner, J., Voute, P.A., Zoubek, A., Gadner, H. and Jurgens, H. (2001) Localized Ewing Tumor of Bone: Final Results of the Cooperative Ewing’s Sarcoma Study CESS 86. Journal of Clinical Oncology, 19, 1818-1829. https://doi.org/10.1200/JCO.2001.19.6.1818
[8]
Ilaslan, H., Sundaram, M., Unni, K.K. and Dekutoski, M.B. (2004) Primary Ewing’s Sarcoma of the Vertebral Column. Skeletal Radiology, 33, 506-513. https://doi.org/10.1007/s00256-004-0810-x
[9]
Ushigome, S., Machinami, R. and Sorensen, P.H. (2002) Ewing Sarcoma/Primitive Neuroectodermal Tumour (PNET). In: Fletcher, C.D.M., Unni, K.K., Mertens, F., Eds., WHO Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone, IARC Press, Lyon, 298-300.
[10]
Heymann, D. (2014) Bone Cancer: Primary Bone Cancers and Bone Metastases. Academic Press, Cambridge.
[11]
Welsch, T., Mechtersheimer, G., Aulmann, S., Mueller S.A., Buechler, M.W., Schmidt, J. and Kienle, P. (2006) Huge Primitive Neuroectodermal Tumor of the Pancreas: Report of a Case and Review of the Literature. World Journal of Gastroenterology, 12, 6070-6073. https://doi.org/10.3748/wjg.v12.i37.6070
[12]
Ikeda, K. and Tsuta, K. (2016) Effusion Cytomorphology of Small, Round-Cell Tumors. Journal of Cytology, 33, 85-92. https://doi.org/10.4103/0970-9371.182529
[13]
Riggi, N. and Stamenkovic, I. (2007) The Biology of Ewing Sarcoma. Cancer Letters, 254, 1-10. https://doi.org/10.1016/j.canlet.2006.12.009
[14]
Baldauf, M.C., Orth, M.F., Dallmayer, M., Marchetto, A., Gerke, J.S., Rubio, R.A., Kiran, M.M., Musa, J., Knott, M.M.L., Ohmura, S., Li, J., Akpolat, N., Akatli, A.N., Ozen, O., Dirksen, U., Hartmann, W., de Alava, E., Baumhoer, D., Sannino, G., Kirchner, T. and Grunewald, T.G.P. (2018) Robust Diagnosis of Ewing Sarcoma by Immunohistochemical Detection of Super-Enhancer-Driven EWSR1-ETS Targets. Oncotarget, 9, 1587-1601. https://doi.org/10.18632/oncotarget.20098
[15]
Vural, C., Uluoglu, O., Akyurek, N., Oguz, A. and Karadeniz, C. (2011) The Evaluation of CD99 Immunoreactivity and EWS/FLI1 Translocation by Fluorescence in Situ Hybridization in Central PNETs and Ewing’s Sarcoma Family of Tumors. Pathology and Oncology Research, 17, 619-625. https://doi.org/10.1007/s12253-010-9358-3
[16]
McCuiston, A. and Bishop, J.A. (2018) Usefulness of NKX2.2 Immunohistochemistry for Distinguishing Ewing Sarcoma from Other Sinonasal Small Round Blue Cell Tumors. Head and Neck Pathology, 12, 89-94. https://doi.org/10.1007/s12105-017-0830-1
[17]
Yamaguchi, K., Katagiri, H., Takahashi, M., Ishida, Y., Ono, A., Takahashi, T., Ohshima, K., Mochizuki, T., Urakami, K., Muramatsu, K., Kameya, T., Ito, I. and Nakajima, T. (2015) ProGRP Is a Possible Tumor Marker for Patients with Ewing Sarcoma. Biomedical Research, 36, 273-277. https://doi.org/10.2220/biomedres.36.273
[18]
Honda, Y., Katagiri, H., Takahashi, M., Murata, H., Wasa, J., Hosaka, S., Ishida, Y., Ito, I., Muramatsu, K., Mochizuki, T., Matsuyama, Y. and Yamaguchi, K. (2019) Pro-Gastrin-Releasing Peptide as a Marker for the Ewing Sarcoma Family of Tumors. International Journal of Clinical Oncology, 24, 1468-1478. https://doi.org/10.1007/s10147-019-01492-0
