Hostname: page-component-cd9895bd7-7cvxr Total loading time: 0 Render date: 2024-12-23T15:57:35.441Z Has data issue: false hasContentIssue false
  • English
  • Français

Multi-modality management of extraosseous Ewing sarcoma: 10-year experience from a tertiary care centre

Published online by Cambridge University Press:  10 March 2020

Arvind Sathyamurthy Open the ORCID record for Arvind Sathyamurthy [Opens in a new window] ,
Ashish Singh ,
Tarun Jose ,
Patricia Sebastian ,
Rajesh Balakrishnan ,
Anne Jennifer Prabhu  and
Selvamani Backianathan
Arvind Sathyamurthy
Affiliation:
Department of Radiation Oncology, Ida B Scudder Cancer Centre, Christian Medical College, Vellore, Tamil Nadu, India
Ashish Singh*
Affiliation:
Department of Medical Oncology, Christian Medical College, Vellore, Tamil Nadu, India
Tarun Jose
Affiliation:
Department of Radiation Oncology, Ida B Scudder Cancer Centre, Christian Medical College, Vellore, Tamil Nadu, India
Patricia Sebastian
Affiliation:
Department of Radiation Oncology, Ida B Scudder Cancer Centre, Christian Medical College, Vellore, Tamil Nadu, India
Rajesh Balakrishnan
Affiliation:
Department of Radiation Oncology, Ida B Scudder Cancer Centre, Christian Medical College, Vellore, Tamil Nadu, India
Anne Jennifer Prabhu
Affiliation:
Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
Selvamani Backianathan
Affiliation:
Department of Radiation Oncology, Ida B Scudder Cancer Centre, Christian Medical College, Vellore, Tamil Nadu, India
*
Author for correspondence: Ashish Singh, Associate Professor, Department of Medical Oncology, Christian Medical College, Vellore, Tamil Nadu, India. E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Aim:

To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018.

Methods:

Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship.

Results:

The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months.

Conclusion:

Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.

Keywords

extraosseous Ewing sarcomamulti-modality managementradiotherapyimmunohistochemical diagnosispatterns of care
Type
Original Article
Information
Journal of Radiotherapy in Practice , Volume 20 , Issue 2 , June 2021 , pp. 189 - 195
Check for updates
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Maheshwari, A V, Cheng, E Y. Ewing sarcoma family of tumors. J Am AcadOrthopSurg 2010; 18 (2): 94–107Google ScholarPubMed
Cash, T, McIlvaine, E, Krailo, MD, et al. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children’s Oncology Group. Pediatr Blood Cancer 2016; 63 (10): 1771–1779.10.1002/pbc.26096CrossRefGoogle ScholarPubMed
Grünewald, T G P, Cidre-Aranaz, F, Surdez, D et al. Ewing sarcoma. Nat Rev Dis Primers 4, 5 2018; doi: 10.1038/s41572-018-0003-x.CrossRefGoogle ScholarPubMed
Applebaum, M A, Worch, J, Matthay, K K et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma, Cancer. Author manuscript; available in PMC 2012 Jul 10.Published in final edited form as. Cancer 2011; 117 (13): 3027–3032. Published online 2011 Jan 10. doi: 10.1002/cncr.25840CrossRefGoogle Scholar
Galyfos, G, Karantzikos, G A, Kavouras, N, Sianou, A, Palogos, K, Filis, K. Extraosseous Ewing sarcoma: Diagnosis, prognosis and optimal management. Indian J Surg. 2016; 78 (1): 49–53.10.1007/s12262-015-1399-0CrossRefGoogle ScholarPubMed
Sharma, S, Kamala, R, Nair, D, Ragavendra, T R. Round cell tumors: classification and immunohistochemistry. Indian J Med Paediatr Oncol. 2017; 38 (3): 349–353. doi: 10.4103/ijmpo.ijmpo_84_16.Google ScholarPubMed
Javery, O, Krajewski, K, O’Regan, K, et al. A to Z of extraskeletal ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. Am J Roentgenol 2011; 197 (6), W1015–W1022.10.2214/AJR.11.6667CrossRefGoogle Scholar
Womer, R B, West, D C, Krailo, M et al. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children’s Oncology Group. J ClinOncol 2012; 30: 4148–4154.10.1200/JCO.2011.41.5703CrossRefGoogle ScholarPubMed
El Weshi, A, Allam, A, Ajarim, D et-al. Extraskeletal Ewing’s sarcoma family of tumours in adults: analysis of 57 patients from a single institution. Clin Oncol (R Coll Radiol 2010; 22 (5): 374–381. doi: 10.1016/j.clon.2010.02.010.CrossRefGoogle ScholarPubMed
Rud, N P, Reiman, H M, Pritchard, D J, Frassica, F J, Smithson, W A. Extraosseous Ewing’s sarcoma. A study of 42 cases. Cancer 1989; 64: 1548–155310.1002/1097-0142(19891001)64:7<1548::AID-CNCR2820640733>3.0.CO;2-W3.0.CO;2-W>CrossRefGoogle ScholarPubMed
Qureshi, S S, Laskar, S, Kembhavi, S et al. Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. Pediatr Surg Int 2013; 29: 1303–1309.10.1007/s00383-013-3409-2CrossRefGoogle ScholarPubMed
Schuck, A, Ahrens, S, Paulussen, M et al. Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. Int J RadiatOncolBiol Phys 2003; 55: 168–177.Google ScholarPubMed