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CASE REPORT
Year : 2015  |  Volume : 18  |  Issue : 3  |  Page : 143-146

Ewing's sarcoma, a rare but dangerous tumor


1 Department of Orthopaedics and Trauma Surgery, College of Medical Sciences, University of Maiduguri, Maiduguri, Nigeria
2 Department of Histopathology, College of Medical Sciences, University of Maiduguri, Maiduguri, Nigeria

Date of Web Publication10-Nov-2015

Correspondence Address:
Theophilus Maksha Dabkana
Department of Orthopaedics and Trauma Surgery, College of Medical Sciences, University of Maiduguri, Maiduguri
Nigeria
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DOI: 10.4103/1118-8561.169280

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  Abstract 

Ewing's sarcoma or Ewing tumor is a rare primary bone tumor that affects mainly children and adolescents. It belongs to a group of cancers known collectively as Ewing sarcoma family tumors or Ewing family tumors. By the time, the patients present and diagnosis is made, the disease is usually advanced. We reviewed the case files of two patients managed in our hospital within one (2013). Fine-needle aspirations for cytology (FNAC) and tissue biopsy were used for diagnosis in the two patients we had. The two patients, both males aged 20 years and 38 years presented late and FNAC and tissue biopsy revealed Ewing's sarcoma. They were referred for radio- and chemotherapy. However, due to poor socioeconomic status, they died of their primary disease. Unless diagnosed early, and in the absence of a multidisciplinary approach, Ewing's sarcoma is a fatal disease.

Keywords: Ewing's sarcoma, late presentation, poor prognosis, rare


How to cite this article:
Dabkana TM, Nggada HA. Ewing's sarcoma, a rare but dangerous tumor. Sahel Med J 2015;18:143-6

How to cite this URL:
Dabkana TM, Nggada HA. Ewing's sarcoma, a rare but dangerous tumor. Sahel Med J [serial online] 2015 [cited 2019 Oct 15];18:143-6. Available from: http://www.smjonline.org/text.asp?2015/18/3/143/169280


  Introduction Top


In the 1920s, Dr. James Ewing, an American Pathologist, described a malignant, round, blue cell tumor arising from the bone in children.[1],[2] It is a rare disease (three cases per million) and has remained so since 1975,[3] on the average. Furthermore, data from the U.S. National Cancer Institute surveillance, epidemiology, and end results confirm this. In patients aged 10–19 years, however, the incidence is between 9 and 10 cases/1 million. It is also known to be 9 times greater in Caucasians then African Americans.[4]

The median age of patients with Ewing's sarcoma is 15 years, >50% of patients being adolescents with a male: female ratio of 1.6:1. The disease has been described in neonates [5],[6] and in patients over 30 years of age, the oldest known patient 76-year-old, from the Mercer country, New Jersey, U.S. Geographical preponderance has been suggested when four unrelated teenagers were diagnosed within a 2-year period (2009–2011) in New Carolina, USA.[7]

The disease is known to represent 16% of primary bone sarcomas. The tumor often goes unnoticed or misdiagnosed due to its subtle and gradual onset, usually following trivial injuries, often leading to late diagnosis, 25% having metastasis at the time of diagnosis.[3] Osseous and extra-osseous primary sites have been described making it difficult to classify. For the osseous sites, the lower extremities, spine, hand and foot and skull, in order of decreasing frequency have been described. The extra-osseous sites occur on the trunk, extremity, head, and neck, retroperitoneal, and other sites have been described, in order of decreasing frequency. We present two cases that did present very late.[8],[9]


  Case Reports Top


Case 1

A 20-year-old student presented with a 1-year history of pain and swelling of the right heel following a football game. The pain and swelling got worse and walking became difficult. When examined, we found a healthy looking boy with a swelling around the insertion of the right tendon Achilles [Figure 1]. A large solitary popliteal lymph node was noted [Figure 2], with discrete right inguinal nodes. X-ray revealed a destructive lesion of the posterior one-third of the calcaneus with no cortical disruption [Figure 3]. A working diagnosis of bone cyst, to rule out a sarcoma was made. Fine-needle aspiration was taken from the swelling and popliteal node and sent to University of Maiduguri Teaching Hospital. A tissue biopsy was then taken from swelling and the popliteal node excised and 1-week later the result came back as Ewing's and metastatic Ewing's sarcoma [Figure 4]. We referred the patient to Ahmadu Bello University Teaching Hospital (ABUTH), Zaria for management. A chest X-ray done was normal. A letter was issued, but the patient could not travel due to financial constrains. He developed chest symptoms 3 months later and died.
Figure 1: Swollen right heel

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Figure 2: Huge solitary popliteal node

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Figure 3: X-ray of affected right calcaneus

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Figure 4: Histology of Ewing's sarcoma

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Case 2

A 38-year-old man presented with a 14 months history of a dull ache and later pain right gluteal area following a fall from a camel. He initially sought treatment traditionally. 3 months prior to presentation, he visited a clinic in Yobe state where I and D was attempted. Swelling thereafter got bigger rapidly and was referred to us. Examination revealed a healthy looking man with an antalgic gait right lower limb. There was a huge swelling over the right gluteal area with a surgical [Figure 5]a and [Figure 5]b. X-ray revealed total destruction of the right ilium and sacroiliac area [Figure 6]. We made a diagnosis of osteosarcoma and sent him for fine needle aspiration for cytology, and the result came back malignant cells. We then took an incisional biopsy, and the result came back as Ewing's sarcoma. We also referred him for radiotherapy and chemotherapy at ABUTH Zaria but could not go due to financial constraints. He died of his disease 4 months later.
Figure 5: (a) Lateral view of the gluteal mass (b) Posterior view of the gluteal mass

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Figure 6: X-ray of destroyed ilium and sacroiliac joint

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  Discussion Top


James Ewing (1866–1943) described the tumor, establishing the fact that the disease was separated from lymphomas and other types of cancer known at that time.[1],[2] The tumor unlike other cancers is as a result of translocation between chromosome 11 and 22 where the EWS gene of chromosome 22 fuses to the FLI1 gene on chromosome 11,[10] with the resultant product functioning as the master regulator gene,[11] leading to loss of control of cell activity. These cells are positive for CD99[10] and negative for CD45.[12]

Like in the index cases illustrated above, diagnosis of Ewing tumor can be missed until it is late because the onset is often subtle and gradual, usually following some form of trauma, and fever suggesting infection like the second case above. In fact, most patients have asymptomatic metastasis at the time of diagnosis and because of it rarity, a tertiary hospital like ours do not have protocols for managing the disease which usually requires a multidisciplinary approach.

The diagnosis depends on histomorphologic findings, immunochemistry, and molecular pathology. The last two are not available at our center. Histomorphologic features include sheets of uniform small round cells that are slightly larger and more cohesive than lymphocytes. They have scanty cytoplasm and typical clear cytoplasm due to rich glycogen on hematoxylin and eosin staining.

Once diagnosis is made, a multi-drug therapy is initiated (neo-adjuvant chemotherapy) which will include vincristine, doxorubicin and cyclophosphamide with ifosfamide and etoposide to shrink the tumor [10] and after about 3 months of 3 weekly cycle, surgical resection of residual tumor is done, often requiring an amputation followed by radiotherapy. Another course of chemotherapy is then done for another 6–8 weeks plus or minus radiotherapy. In women of childbearing age, they may need to preserve oocytes or ovarian tissue by cryopreservation. If the later is used, it must be examined for cancer cells to avoid reseeding of the disease after the treatment.[13] In patients with early disease, a 5-year survival is 70–80% when properly treated. This may drop to <10/% in advanced disease.[14]


  Conclusion Top


Ewing's sarcoma is a disease that requires very early diagnosis. A very high index of suspicion is, therefore, important since local tumor control has a better prognosis. Tertiary health centers should have basic to manage cases of cancer so as to improve the life of patients. Oncology study group or team should be proactive to handle such cases.

 
  References Top

1.
Synd/2367, 'Who Named It'. Available from: http://www.whonamedit.com/synd.cfm/2367.html. [Last accessed on 2014 Jul 10].  Back to cited text no. 1
    
2.
Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc 1921;21:17-24.  Back to cited text no. 2
    
3.
Esiashvili N, Goodman M, Marcus RB Jr. Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results (SEER) data. J Pediatr Hematol Oncol 2008;30:425-30.  Back to cited text no. 3
    
4.
Jawad MU, Cheung MC, Min ES, Schneiderbauer MM, Koniaris LG, Scully SP. Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: An analysis of 1631 cases from the SEER database, 1973-2005. Cancer 2009;115:3526-36.  Back to cited text no. 4
    
5.
Kim SY, Tsokos M, Helman LJ. Dilemmas associated with congenital ewing sarcoma family tumors. J Pediatr Hematol Oncol 2008;30:4-7.  Back to cited text no. 5
    
6.
van den Berg H, Dirksen U, Ranft A, Jürgens H. Ewing tumors in infants. Pediatr Blood Cancer 2008;50:761-4.  Back to cited text no. 6
    
7.
8.
Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, et al. Ewing's sarcoma of soft tissues in childhood: A report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 1997;15:574-82.  Back to cited text no. 8
    
9.
Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011;117:3027-32.  Back to cited text no. 9
    
10.
Turc-Carel C, Aurias A, Mugneret F, Lizard S, Sidaner I, Volk C, et al. Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12). Cancer Genet Cytogenet 1988;32:229-38.  Back to cited text no. 10
    
11.
Owen LA, Kowalewski AA, Lessnick SL. EWS/FLI mediates transcriptional repression via NKX2.2 during oncogenic transformation in Ewing's sarcoma. PLoS One 2008;3:e1965.  Back to cited text no. 11
    
12.
Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, et al. Ewing's sarcoma family of tumors: Current management. Oncologist 2006;11:503-19.  Back to cited text no. 12
    
13.
Abir R, Feinmesser M, Yaniv I, Fisch B, Cohen IJ, Ben-Haroush A, et al. Occasional involvement of the ovary in Ewing sarcoma. Hum Reprod 2010;25:1708-12.  Back to cited text no. 13
    
14.
Thacker MM, Temple HT, Scully SP. Current treatment for Ewing's sarcoma. Expert Rev Anticancer Ther 2005;5:319-31.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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