PICTORIAL REVIEW OF EXTRAOSSEOUS EWING’S TUMOUR: A SINGLE CENTER EXPERIENCE
Abstract
Purpose: Ewing’s family tumour is an extremely rare tumour, with annual incidence rates amongst Caucasian children <21 years being in the range of 2–3 cases per million in the U.S. There are mainly three subtypes including Ewing’s sarcoma (ES) of bone, extraosseous (EO) Ewing’s tumour and Peripheral primitive neuroectodermal tumour. Although extremely rare, this study represents a review of various types of cases and the significance of imaging including its baseline and post-treatment response radiological characteristics. There are a very few cases of EO ES in the current literature with variable spectrum of tumour site and their imaging characteristics.
Materials and Methods: Electronic records were retrospectively reviewed from 1 May 2011 to 1 May 2016 with patients who were diagnosed as histologically proven ES. A number of patients, gender and base line computed tomography (CT)/magnetic resonance imaging findings for staging were reviewed.
Results: A total of 568 patients with diagnosed ES were analysed, of which 15 patients had EO type of ES. Of these only 8 patients had baseline imaging available which included tumours arising from the occipital region, orbit, anterior mediastinum, anterior abdominal wall, mesentery, kidney, prostate gland and presacral region.
Conclusion: EO ES is a rare entity and can involve a wide array of soft tissue organs. A cross-sectional imaging with CT and MR has a key role in pre- and post-treatment assessment.
Key words: Computed tomography, Ewing’s sarcoma, extraosseous Ewing’s, magnetic resonance imaging, peripheral primitive neuroectodermal tumour
References
Ludwig JA. Ewing sarcoma: Historical perspectives, current state-of-the-art, and opportunities for targeted therapy in the future. Curr Opin Oncol 2008;20:412-8.
Geens L, Robays JV, Geert V, et al. An unusual location of extraosseous Ewing’s sarcoma. Case Rep Oncol 2013;6:293-302.
Tsokos M. Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis. Perspect Pediatr Pathol 1992;16:27-98.
Toh KL, Tan PH, Cheng WS. Primary extraskeletal Ewing’s sarcoma of the external genitalia. J Urol 1999;162:159-60.
Perouli E, Chrysikopoulos H, Vlachos A, et al. Imaging findings in paraspinal extra osseous Ewing sarcoma. JBRBTR 2006;89:310-2.
Letourneau PA, Shackett B, Xiao L, et al. Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival. J Pediatr Surg 2011;46:332-5.
Shek TW, Chan GC, Khong PL, et al. Ewing sarcoma of the small intestine. J Pediatr Hematol Oncol 2001;23:530-2.
Maeda M, Tsuda A, Yamanishi S, et al. Ewing sarcoma/ primitive neuroectodermal tumor of the kidney in a child. Pediatr Blood Cancer 2008;50:180-3.
Park JY, Lee S, Kang HJ, et al. Primary Ewing’s sarcomaprimitive neuroectodermal tumor of the uterus: A case report and literature review. Gynecol Oncol 2007;106:427-32.
Tanida S, Tanioka F, Inukai M, et al. Ewing’s sarcoma/ peripheral primitive neuroectodermal tumor (pPNET) arising in the omentum as a multilocular cyst with intracystic hemorrhage. J Gastroenterol 2000;35:933-40.
Maisonnette F, Roux ET, Abita T, et al. Ewing sarcoma of the mesocolon. Gastroenterol Clin Biol 2007;31:552-4.
Petković M, Zamolo G, Muhvić D, et al. The first report of extraosseous Ewing’s sarcoma in the rectovaginal septum. Tumori 2002;88:345-6.
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Authors retain copyright and grant the Journal of Cancer & Allied Specialties (JCAS) right-of-first publication. In addition, the work will be simultaneously licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International license. This license allows others to share the work in whole or part (for non-commercial purpose), with an acknowledgement of the work’s authorship and initial publication in JCAS.
Furthermore, authors are free to enter into separate contractual arrangements for the non-exclusive distribution of the journal’s published version of the work, with an acknowledgement of its initial publication in this journal.
Authors are permitted and encouraged to share their work online or in medical or scientific conferences prior to or during submission process.