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CASE REPORT Table of Contents   
Year : 2005  |  Volume : 39  |  Issue : 2  |  Page : 127-129
Metastatic Ewing sarcoma - A case report


Department of Orthopedics, Government Medical College, Anantapur, India

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How to cite this article:
Adisesh AM, Neeraja M, Prasad T. Metastatic Ewing sarcoma - A case report. Indian J Orthop 2005;39:127-9

How to cite this URL:
Adisesh AM, Neeraja M, Prasad T. Metastatic Ewing sarcoma - A case report. Indian J Orthop [serial online] 2005 [cited 2019 Oct 15];39:127-9. Available from: http://www.ijoonline.com/text.asp?2005/39/2/127/36793

   Introduction Top


Ewing sarcoma is a malignant tumor of the bone that usually occurs during the first two decades of life. Although it may develop in any bone, the most frequent sites are in the distal part of the skeleton, especially the femur, ilium and tibia. It is uncommon in the upper extremity except in the humerus. Thirty percent of these tumors are multi centric and metastasis are often present at diagnosis. Presentation in any cranial bone as a secondary is not reported.

We are reporting a case of Ewing sarcoma of the occipital bone as the first presenting site of metastases.


   Case report Top


A 19 yr old young man reported with pain and mild swelling of the upper end of right tibia in August 2000. He had on and off fever, malaise and ill health. Movements of the right knee were normal and he was walking with a limp. X­ray showed involvement of upper 1/4 th of right tibia on the lateral part. The lesion was diffuse with areas of bone destruction in center and periosteal reaction on the outer side. All the blood parameters were normal except the alkaline phosphatase level which was elevated.

MRI done in Aug 2000 showed two well defined hypo­intense lesions in the condyles of distal femur and intermediate intensity lesion on the epiphyseal and metaphyseal region of proximal tibia. The lateral cortex of lateral tibial condyle shows areas of erosion and cortical thinning.

Biopsy was performed and it was confirmed as Ewing sarcoma. Bone scan done in Sept 2000 showed secondary lesions in lower right femur, right frontal bone, proximal third of right humerus, T 8 , L 2 and L 4 bodies, roof of right acetabulum and superior ramus of right pubic bone.

VAIA Chemotherapy was given in three phases. Phase I on 10/11/00, Phase II on 1/12/00 and Phase III on 13/1/01. Radiotherapy was also given appropriately. He was fine till May 2002 when he complained of head ache, dizziness and occasional vomiting. Visual acuity was normal. C T Scan showed an iso-intense lesion in the occipital bone, occupying half of the posterior cranial fossa. The lesion had a broad base to the occipital bone in posterior cranial fossa with anterior enhancing part with obstructive hydrocephalus.

On 8/7/02 he underwent midline sub occipital craniectomy and excision of extradural mass, dural opening and excision of intradural mass with duroplasty and acrylic cranioplasty and V P shunt. H P E showed malignant round cell tumor - Ewing's sarcoma. Again he underwent another course of RT and CT. Postoperative period was uneventful and he had no other complications in the Cranium or CNS.

In Nov 2002 he came back with terrible LBA and inability to sit. X-ray pelvis including both hips showed metastases in both trochanters and both iliac bones.


   Discussion Top


In 30% cases Ewing's sarcoma is multicentric in origin [1] . In 14-50% multiple metastases are present at the time of diagnosis [2] . CNS spread is rare and isolated CNS involvement is not seen [3] . In pelvic lesions CT & MRI are more informative. Bone scan with 99M Tc detects multicentricity and asymptomatic skeletal metastases. This is useful in planning treatment.

Aim of treatment in these cases is to control clinical and sub clinical disease thus enhancing overall survivals. The success of chemotherapy in preventing metastases and effectiveness of radiotherapy in controlling the primary disease have resulted in abandoning surgery as the sole primary modality of treatment [4] .

Currently there is considerable controversy regarding role of surgery in treatment of Ewings. Surgery is recommended for expendable bones. Balance between surgery and radiotherapy should be employed for optimal control and best preservative function [5] . Limb salvage is still considered investigational.

Prior to use of systemic chemotherapy, more than 90% patients died of systemic pulmonary or bone metastases within 2-5 years. The improved long term disease free survival rates above 50%, now being reported, are the results of intensive combination of multi-cycle chemotherapy regimens [6] . Radiation therapy alone results in local control rate of 65-75%. If chemotherapy is added to adequate radiation, this figure raises to 90-95% [7] .

It is better to give initial aggressive chemotherapy before local therapy, be it radiotherapy or surgery [8] . It has the following advantages:

  • Systemic metastases would be treated.
  • Hazards due to delay in surgery or bone marrow depression secondary to radiotherapy of axial or proximal lesions are prevented.
  • It may reduce the primary bulk of the tumor to the point where, in some cases, no tumor can be demonstrated microscopically. This reduction may allow a more nearly complete and total eradication of primary tumor by radiotherapy and lead to a reduction in the late local recurrence rate [8].


This is a systemic disease which is responsive to chemotherapy. The chemotherapy for the primary tumor has to be individualized for every patient. The best results are achieved when chemotherapy is used both before and after definitive radiotherapy and surgical treatment. The first chance is the best chance, recurrent or residual disease is very chemo-resistant. Therefore maximum tolerable doses of combination chemotherapy with supportive agents like G­CSF should be used. For patients with multi focal bone disease, with large pelvic tumors, relapse after initial surgery, high dose regimens - - mega therapy with bone marrow or stem cell support are given.

Ewing's sarcoma cell lines respond in vitro to a combination of tumor necrosis factor alpha and gamma interferon, suggesting a possible field of application for biological response modifiers [8] .

 
   References Top

1.Miller RW. Contrasting epidemiology of childhood osteosarcoma, Ewing Sarcoma and Rhabdomyosarcoma. National Cancer Institute Mono­gram. 1981; 56: 9-14.  Back to cited text no. 1    
2.Ewing J. Diffuse endothelioma of bone. Proc NY Pathol Soc. 1921; 21:17-24.  Back to cited text no. 2    
3.Trigg ME, Glaubiger D. The frequency of isolated CNS involvement in Ewings Sarcoma. Cancer. 1981; 49: 2404.  Back to cited text no. 3    
4.Rosen G, Woller N. Disease free survival in children with Ewing Sar­coma treated with radiation and adjuvant 4 drug sequential chemo­therapy. Cancer. 1974; 33: 84.  Back to cited text no. 4    
5.Marcove RC. Ewing Sarcoma. In the surgery of tumors of bone and cartilage. New York: Grune and Stratton. 1981;120-132.  Back to cited text no. 5    
6.Rosen G, Capparos B, Nirenberg A. Ewing Sarcoma: Ten years experience with adjuvant chemotherapy. Cancer .1981;47:2204-2213.  Back to cited text no. 6    
7.Thomas PRM. Ewing sarcoma. In: Perez CA, Brady LW, editors. Prin­ciples and practice of radiation oncology. Philadelphia: Lippincott Raven Publishers. 1998; 2038-2051.  Back to cited text no. 7    
8.Huvos AG, Marcove RC. Combination chemotherapy (T-6), in the multidisciplinary treatment of Ewing Sarcoma. Natl Cancer Inst mono­gram. 1981; 56: 289-299.  Back to cited text no. 8    

Top
Correspondence Address:
Athmaram M Adisesh
13/482, Ramachandra Nagar, Anantapur - 515 001 (AP)
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5413.36793

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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