Atypical Presentation of Ewing’s Sarcoma

Abstract

Background: Ewing’s sarcoma (ES) is an osseous malignancy of small round blue cells which may manifest even in soft tissue. It is the second most common primary tumor of bone in childhood. The common areas of occurrence are the diaphyses of femur followed by tibia, humerus, pelvis, and clavicle. However, there are many unusual presentations of ES on the basis of the site, e.g., craniofacial bones, paravertebral mass, and visceral organs. Chemotherapy with radiation therapy and cytoreductive surgery/limb salvage surgery is the mainstay treatment along with a strict patient compliance and counseling, critically being important for long-term survival. This study depicts presentations of ES who either presented with some unusuality at the time of diagnosis or developed peculiar features uncharacteristic of ES during their course of treatment. Context: This study provides an insight into the patterns of unusual presentation of ES and prognosis of such patients with current line of management. Multiple sites of metastases have a very poor dismal outlook. Aims: This study purports the importance of considering ES as a systemic disease rather an osseous malignancy and reviews the pattern of unusual sites of presentation. Setting and Design: This observational study was carried out in the Department of Radiotherapy, Gandhi Medical College, Bhopal. Only patients with unusual patterns of metastases were included in the study cohort. Materials and Methods: We systematically reviewed patients with confirmed and immunohistochemistry-proven ES from July 2014 to July to 2017. A total of 69 patients were registered within the time frame. Of the 69 patients, 36 (52.2%) were males and 33 (47.8%) were females. Of the 11 (16%) unusual presentations, 8 were males and 3 were females. The mean age of presentation was 22.5 (3–52 years). All patients received chemotherapy as per treatment guidelines. Each patient was individually followed up and metastatic workup was performed in a systematic manner. Statistical Analysis: Since the pattern of metastasis was observed, no significant statistical analysis was required for this study. Results: Of the 11 patients, six succumbed during the course of treatment, one patient was lost to follow-up, while four patients are on regular follow-up. Of the six patients who succumbed, four had multiple metastases at the time of presentation to our outpatient department, while the other two patients had paraspinal ES with paraplegia. Two patients, one male and one female, had associated comorbidities with spherocytosis and bronchial asthma. The youngest patient was a 3-year-old child with paraspinal ES, while the oldest was a 52-year-old with extraosseous ES of the right thigh who presented with lung, liver, and supraclavicular metastases. Conclusion: In our study, pattern of metastases determined the patients’ survival. Those patients who presented with multiple metastases at the time of presentation had a very poor prognosis, with death occurring within 2–3 months. The chemotherapy protocols were neither modified nor changed. They were evaluated after every cycle. Those patients who had developed multiple metastases at the time of admission to our outpatient clinic fared the worst. Those who had metastases to the brain and lung also succumbed to the malignancy. It is time we consider ES as an osseo-systemic malignancy and incorporate newer techniques such as circulating tumor cells in the investigation and evaluation portfolio for better and aggressive management. A multidisciplinary tumor board is absolutely essential and weekly meetings mandatory to individualize the treatment scenario. In our study, uncustomary as it may seem, their metastatic sites demand a certain degree of limelight in the Annals of Oncology.

Keywords

Age - Ewing’s family of tumors - metastases - unusual sites

Publication History

Article published online:
24 May 2021

• References

• 1 Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc 1921; 21: 17-24
• 2 Ewing J. Further report on endothelial myeloma of bone. Proc N Y Pathol Soc 1924; 24: 93-101
• 3 Kuk JY, Yoon SY, Kim MJ, Lee JW, Kim BG, Bae DS. A case of primitive neuroectodermal tumor of the ovary. Korean J Obstet Gynecol 2012; 55: 777-81
• 4 Hakky TS, Gonzalvo AA, Lockhart JL, Rodriguez AR. Primary Ewing sarcoma of the kidney: A symptomatic presentation and review of the literature. Ther Adv Urol 2013; 5: 153-9
• 5 Gopalakrishnan CV, Shrivastava A, Easwer HV, Nair S. Primary Ewing’s sarcoma of the spine presenting as acute paraplegia. J Pediatr Neurosci 2012; 7: 64-6
• 6 Toomey EC, Schiffman JD, Lessnick SL. Recent advances in the molecular pathogenesis of Ewing’s sarcoma. Oncogene 2010; 29: 4504-16
• 7 Craft A, Cotterill S, Malcolm A, Spooner D, Grimer R, Souhami R. et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: The second United Kingdom children’s cancer study group and the medical research council Ewing’s tumor study. J Clin Oncol 1998; 16: 3628-33
• 8 Womer RB, West DC, Krailo MD, Dickman PS, Pawel BR, Grier HE. 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 Clin Oncol 2012; 30: 4148-54
• 9 Ozaki T, Hillmann A, Hoffmann C, Rübe C, Blasius S, Dunst J. et al. Significance of surgical margin on the prognosis of patients with Ewing’s sarcoma. A report from the cooperative Ewing’s sarcoma study. Cancer 1996; 78: 892-900
• 10 Schuck A, Ahrens S, von Schorlemer I, Kuhlen M, Paulussen M, Hunold A. et al. Radiotherapy in Ewing tumors of the vertebrae: treatment results and local relapse analysis of the CESS 81/86 and EICESS 92 trials. Int J Radiat Oncol Biol Phys 2005; 63: 1562-7
• 11 Lisanne G, Johan VR, Verswijvel G, Kurt van der S. An unusual location of extra osseous Ewing’s sarcoma. Case Rep Oncol 2013; 6: 293-302