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Ewing’s Sarcoma Presenting With Pathological Fracture In A 9 Month Old

Vishal KakarRajesh Botchu Raghu SathyanaryanSreenivasa Meruva,  #Kasseti Ravikumar

* Department of Orthopaedics, Maidstone hospital, Maidstone, UK
#Department of Orthopaedics, Kurnool medical college, India

Address for Correspondence

Vishal Kakar
Department of Orthopaedics,
Maidstone hospital, Maidstone, UK


Ewing’s sarcoma sometimes presents with a pathological fracture in a long bone. We describe what we believe is the youngest reported case of a nine month old with a pathological fracture of the right humerus confirmed as Ewing’s sarcoma. Multimodal therapy in patients with isolated lesions offers the best prospect of survival.
Key words
: Pathological fracture, Ewing’s, infant

J.Orthopaedics 2006;3(1)e6

Case Report:

Ewing’s sarcoma accounts for approximately 15% of primary malignant bone tumours with peak incidence between ages 10 and 20. Recent advances in surgical management, adjuvant radiotherapy and combination chemotherapy have significantly improved long term prognosis. The youngest patients described in the literature to date are two years old1,2.  We report a nine month old infant presenting with pathological fracture of the right humerus confirmed as Ewing’s sarcoma.

A nine month old girl presented with painful swelling of the right humerus (figure-1). There was no history of trauma, respiratory symptoms or constitutional upset. Examination revealed diffuse swelling and exquisite tenderness along the mid shaft of right humerus without any distal neurovascular deficit.  Plain radiology showed a pathological fracture through a lytic lesion involving the diaphysis of the humerus. Biopsy of the right humerus displayed histology consistent with a diagnosis of Ewing’s sarcoma (figure 2). Chest and abdominal CT scans did not reveal any metastases and bone scan showed an isolated lesion in the right humerus with no other skeletal metastases (figure 3). However, bone marrow

aspirate suggested malignant infiltration. There was no clinical or haematological evidence of bone marrow failure.  Various management strategies and prognosis were discussed with the parents who refused surgery and radiotherapy, but consented to combination chemotherapy. Initial response to chemotherapy was satisfactory and follow up at six months had not shown any relapse.

Discussion :

In children, Ewing’s sarcoma is the second most common primary bone tumour, but the most lethal. It arises from red marrow and is a form of  primitive neuroectodermal tumour. There is a slight male predominance and it is more common in caucasians.


Clinical presentation is with intense pain and localized signs of inflammation with neurological signs in axial skeletal involvement. Pathological fracture is a rare but recognized presentation. Lungs and bone marrow are the most common sites for metastases, the presence of which greatly diminishes prognosis.


Optimal management involves initial systemic combination chemotherapy followed by surgery (Reconstructive or ablative) with or without radiotherapy. Using these strategies in patients with localized disease, five year survival can be up to 70%1,3,4 . Recognized factors for a poorer prognosis include male sex, anaemia, elevated LDH levels, no use of chemotherapy and poor response to chemotherapy. Good chemotherapy response allows reconstructive surgery to be performed without a significant increase in recurrence or metastatic dissemination, whilst poor response may warrant amputation5 . Inoperable tumours are managed by mega dose chemotherapy and radiotherapy5,6.  In this case unimodal therapy alone had to be adopted by default.


Ewing’s sarcoma should be considered in the differential diagnosis in an infant with a pathological fracture of a long bone.  Early diagnosis and multimodal therapy greatly improves outcome.

Reference :

1.   Cotterill SJ, Ahrens S, Paulussen M, Jurgens HF, Voute PA, Gadner H et al 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 2000; 18 (17): 3108-14 

2.    Saenz NC, Hass DJ, Meyers P,Wollner N, Gollamudi S, Bains M et al.  Pediatric chest wall Ewing's sarcoma.  Journal of  Pediatric Surgery. 2000; Apr;35(4):550-5

3.   Nesbit ME, Gehan EA, Burgert EO Jr, Vietti TJ, Cangir A, Tefft M et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study. Journal of Clinical Oncology 1990; (10): 1664-74.

      4.   Sluga M, Windhager R, Pfeiffer M, Ofner P, Lang S, Dominkus M et al. Osteosarcoma            and Ewing's sarcoma-The most frequent malignant bone tumors in children-therapy and outcome. Zeitschrift fur Orthopadie und Ihre Grenzgebiete. 2002; Nov-Dec;140(6):652-5

      5.   Schovanec J, Mracek J, Havlas V, Trc T. Ewing’s sarcoma in children- current     surgical treatment options, evaluation of our patients. Acta Chirigiae Orthopeadicae Traumatologiae Cechoslovaca .2004; 71(4):220-7

      6.   Shankar AG, Pinkerton CR, Atra A. Local therapy and other factors influencing site of relapse in patients with localised Ewing's sarcoma. United Kingdom Children's Cancer Study Group (UKCCSG). European Journal of Cancer 1999; 35 (12): 1698-704.


This is a peer reviewed paper 

Please cite as : Vishal Kakar: Ewing’s Sarcoma Presenting With Pathological Fracture In A 9 Month Old

J.Orthopaedics 2006;3(1)e6





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