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MUSCULOSKELETAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 403-405
Ewing's sarcoma of the small bones of hands and feet


Dept of Radiology and Orthopaedics, AL-RAZI Hospital, Kuwait

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Keywords: Ewing′s Sarcoma, Malignant bone tumour

How to cite this article:
Mohan V, Gupta R P. Ewing's sarcoma of the small bones of hands and feet. Indian J Radiol Imaging 2002;12:403-5

How to cite this URL:
Mohan V, Gupta R P. Ewing's sarcoma of the small bones of hands and feet. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 8];12:403-5. Available from: http://www.ijri.org/text.asp?2002/12/3/403/28494
Ewing's Sarcoma, is defined as a highly malignant anaplastic tumor, found mostly in children and can involve any bone, though bones of the pelvis and lower extremity constitute the majority [1],[2],[3]. This tumor is stated to comprise approximately 6% of the total malignant bone tumours, but occurs very rarely in the small bones of hands and feet, where the reported incidence is not more than 0.3% [4].

We report here one case each involving the 1st phalanx of right index finger and 1st metatarsal bone of the left foot.


   Case Reports Top


Case 1: A four year old girl presented with pain and swelling of her right index finger of two months duration. The pain was severe from the beginning followed by swelling which started at the base of the index finger and grew rapidly to cause swelling of whole of the finger. There was no history of local trauma but the parents did complain of low grade fever and malaise of the child. She had been treated with antibiotics and analgesics for more than two weeks before seeking orthopaedic consultation.

General physical examination showed an ill looking child. Local examination revealed swelling of whole right index finger, more so in the region of 1st and 2nd phalanges. It was firm to hard in consistency with raised local temperature and restriction of interphalangeal joint movement.

Roentgen examination of right hand revealed expansile destructive lesion involving whole of the 1st phalanx with some areas of sclerosis and accompanied with huge soft tissue swelling. [Figure - 1]. A clinico-radiological diagnosis of osteomyelitis was made. However, in view of the rapid progress of the disease, no significant response to the antibiotic therapy, big size of the swelling and its firm to hard consistency, possibility of its being primary malignant bone tumour was kept in the differential diagnosis. Bone scan revealed increased local tracer up-take. Chest roentgenogram and skeletal survey did not reveal any other lesion. Laboratory investigations revealed Hb 10 gm%, total leucocytic count of 12000 and sedimentation rate of 35mm. Fine needle aspiration cytology revealed the diagnosis of Primary Ewing's Sarcoma. Surgical excision of the tumour was undertaken following by chemotherapy. However the child died after 8 months of the diagnosis due to pulmonary metastasis

Case 2: A ten year old boy was admitted in the surgical services with the chief complaints of pain and a pulsatile swelling on the dorsum of his left foot of about 4 months duration. The complaints followed a minor local trauma and the swelling progressed rapidly. The initial radiograph was reported normal. The pain was of shooting type and present all the time and he was unable to walk without support at the time of admission. General physical examination revealed the child to be anaemic. Temperature was normal. Two small, but tender lymphnodes were noted in the left inguinal region. Local examination of the foot revealed a big round soft tissue swelling on the dorsum of the foot which was firm in consistency and was pulsatile. Local temperature was raised. A clinical diagnosis of post-traumatic aneurysm or an arterio-venous fistula was made and an angiogram was requested.

Plain radiograph of the left foot revealed gross expansion, flattening and destruction of the 1st metatarsal bone with areas of new bone formation and a huge soft tissue mass, causing pressure effect and sclerosis of the adjacent 2nd metatarsal bone [Figure - 2]. Angiogram revealed neo-vascularity and abnormal tumour vessels, suggesting the diagnosis of a highly malignant primary bone tumour [Figure - 3] Considering the young age of the patient, the clinical findings and the roentgen observations, the diagnosis of Primary Ewing's Sarcoma of the 1st metatarsal bone was made. Bone Scan revealed very high tracer up-take in the affected bone, but no other abnormal foci in the rest of the skeleton. C.T. Scan of the chest was normal. Laboratory investigations revealed Hb of 9 gm% and erythrocyte sedimentation rate of 44mm. Biopsy confirmed the diagnosis of Ewing's Sarcoma. [Figure - 4] and [Figure - 5]. Surgical treatment was refused by the patients parents and then he was taken away against the medical advise for some local treatment. He was not brought for follow up.


   Discussion Top


Primary malignant bone tumours of all types are rare in the acral portions of the extremities. Besser et al [5] reviewed 300 tumours of the hands, but not a single one was of Ewing's Sarcoma, Lacey etal [6] could find only 13 cases of Ewing's Sarcoma of hand bones on review of the literature and added one of their cases to make it 14 cases till 1987. In the hand bones, the affection of metacarpals is more common than of the phalanges [6],[7],[8]. Recently, the youngest patient, a 5 months's old infant having tumour of phalanx was reported by Strege etal [9]. The affection of the foot bones appears to be extremely uncommon as not many cases have been found reported [10],[11].

As with tumours at other locations, pain and swelling are the two most common presenting complaints, which may tend to be progressive [8]. Low grade fever is common and these patients may have elevated erythrocyte sedimentation rate and leucocytosis. Few patients do give history of previous local trauma and the symptoms may vary from few weeks to many months [4],[7].

Roentgenographic changes may include permeative destructive lesion of the affected bone with areas of sclerosis and layers of periosteal reaction which may produce the so called "onion skin" appearance, said to be characteristic of Ewing's Sarcoma [7]. However, a finding which has not been given much importance is that in the short bones of the hands and feet, the tumour may cause expansion and flattening of the affected bone producing a cystic type of appearance as seen in both of our cases [3]. Pritchard [12] and Dick etal [8] have also noted similar changes and we consider these findings to be of great significance if the bony lesion is accompanied with a huge soft tissue swelling. The size of the swelling and its consistency helps it to be differentiated from conditions like tuberculous dactylitis, osteomyelitis and lesions caused by Histiocytosis. Local clinical examination and increased vascularity of the lesion also helps in suspecting the diagnosis of malignancy. Increased vascularity causing abnormal pulsations in the soft tissue swelling in our 2nd case had led to the suspicion of it being a post-traumatic aneurysm.

The treatment of Ewing's Sarcoma continues to be controversial [13]. Recent reports have emphasized the role of surgical extirpation and adjunctive chemotherapy, especially in the absence of metastatic disease [13],[14]. In the hand and foot bones, resection removes the tumour with radical margins, preserves the function and the results are cosmetically satisfactory [15].

 
   References Top

1.Sherman R. S. and Soong KY, Ewing's Sarcoma, Its Roentegen Classification and Diagnosis. Radiology 1956, 66:529-535.  Back to cited text no. 1    
2.Potdar G.G. Ewing's Tumour. Clinical Radiology 1979, 222:528-533  Back to cited text no. 2    
3.Coombs R. J.: Zeiss J, Paley K. J and Kini J, Case report 802. Skeletal Radiology 1998, 22: 460-463  Back to cited text no. 3    
4.Kissane J. M, Askin F. S, Foulkes M, Stration LB and Shireley S.F. Ewing's Sarcoma of the Bone, Clinico-Pathologic Aspects of 3 0 3 Cases For The Intergroup Ewing's Sarcoma Study. Hum. Pathol, 1983, 14: 773-779.  Back to cited text no. 4    
5.Besser E, Roessner A, Brug E, Erlemann R. Timm. C and Grundmann, Bone Tumours of the Hand. Arch. Orthop, trauma, Surg 1987, 106: 241-247  Back to cited text no. 5    
6.Lacey S. H. Danish E. H, Thompson G. H and Joyce MJ. Ewing's Sarcoma of Proximal Phalanx of Finger. Journ. B. Joint, Surg 1987, 69 A: 931- 933.  Back to cited text no. 6    
7.Clifford RH and Kelly APJr: Primary Malignant. Tumours of the Hand. Plastic, Reconst, Surg, 1955, 15: 227-232.  Back to cited text no. 7    
8.Dick HM, Francis K. C. and Johnston A.D. Ewing's Sarcoma of The Hand Journ. Bone Joint Surg. 1971, 534: 345-348  Back to cited text no. 8    
9.Strege DW, Hanel DP, Vogler C and Schajowics F. Ewing's Sarcoma in a Phalanx of an Infant. Journ Bone Joint Surg. 1989, 714: 1262 - 1265.  Back to cited text no. 9    
10.Schajowics F: Tumour and Tumour Like Lesions of Bone and Joints. Springer, New York, 1981.  Back to cited text no. 10    
11.Shirley S. K. Askin F. B, Gilula LA. Vietti J. J, Thomas PR Seigal G.P, Reinus WR, Kissane J.M. and Nesbit ME. Ewing's Sarcoma of Hands and Feet. Journ, Clin, Oncology, 1985, 3: 686-697  Back to cited text no. 11    
12.Pritchard D. J.: Small Round Cell Tumours. Ortho. Clinic. North America 1987, 20: 367-370  Back to cited text no. 12    
13.Rosen G, Caparros B. Nirenberg A Ewing's Sarcoma. Ten Years experience with Adjuvant chemotherapy. Cancer 1981, 47: 2204-2213.  Back to cited text no. 13    
14.Razek A, Peres CA, Tefft M: Intergroup Ewing's, Sarcoma. Local Control Related to Radiation Dose, Volume and Site of Primary Lesion in Ewing's Sarcoma, Cancer 1980, 46: 516-521.  Back to cited text no. 14    
15.Steichen J. B. and Idler R. S.: Results of Central, Ray Resection without Bony Transposition. Journ, Hand Surg. 1986, 11A: 466-474.  Back to cited text no. 15    

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Correspondence Address:
V Mohan
Dept of Radio-Diagnosis, Acharya Shri Chander College. of Medical Sciences, Sidhra, Jammu. Post Box No: 75, H.P.O., Gandhi Nagar, Jammu 180004 India

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

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