|Year : 2013 | Volume
| Issue : 1 | Page : 46-48
Ewing's sarcoma of hand: An unusual case
Maruti Kambali1, Gudi S Narayana1, Nazeer B Shaikh1, H Priyamargavi2, Vijay HS Anand1, Praneeth Revuri1, Rahul Jayaram1
1 Department of Orthopaedics, Sri Devaraj URS Medical College, Kolar, Karnataka, India
2 Department of Anesthesiology, Sri Devaraj URS Medical College, Kolar, Karnataka, India
|Date of Web Publication||17-Apr-2013|
Department of Orthopaedics, Sri Devaraj Urs Medical College, Kolar, Karnataka
Source of Support: None, Conflict of Interest: None
Ewing's sarcoma is an uncommon malignant tumor of bone that usually occurs during the first two decades of life. It is most common between the ages of 10 and 15 years. Although it may develop in any bone, the most frequent sites are the femur, ilium, and tibia. It is uncommon in the upper extremity, except in the humerus and it is rare in the hand. We are reporting the case of a 19-year-old girl who had Ewing's sarcoma in the second metacarpal of the left hand 12 years ago and now presented with Ewing's sarcoma in distal left radius with metastasis to brain, liver, and lungs.
Keywords: Ewing′s sarcoma, fibular grafting, Gy-gray, metacarpal, transverse sinodura
|How to cite this article:|
Kambali M, Narayana GS, Shaikh NB, Priyamargavi H, Anand VH, Revuri P, Jayaram R. Ewing's sarcoma of hand: An unusual case. Int J Health Allied Sci 2013;2:46-8
|How to cite this URL:|
Kambali M, Narayana GS, Shaikh NB, Priyamargavi H, Anand VH, Revuri P, Jayaram R. Ewing's sarcoma of hand: An unusual case. Int J Health Allied Sci [serial online] 2013 [cited 2019 Oct 22];2:46-8. Available from: http://www.ijhas.in/text.asp?2013/2/1/46/110563
| Introduction|| |
Ewing's sarcoma originates rarely in the bones of the hand. Patients rarely survive more than 5 years. We report a case of primary Ewing's sarcoma of the second metacarpal of the left hand in a female child surviving for 12 years, now involving distal left radius with metastasis to brain, liver, and lungs.
| Case Report|| |
A 19-year-old girl presented 12 years ago with history of fall, sustaining injury to the left hand and noticed a swelling over dorsum of hand, in due course of time swelling gradually increased in size and occasionally associated with pain. It was diagnosed to be Ewing's sarcoma of left second metacarpal elsewhere and treated with excision of second metacarpal and ipsilateral fibular grafting [Figure 1]a and b.
|Figure 1: (a and b) Clinical and X-ray of the left hand showing excised second metacarpal with healed fibular graft. (c) Clinical picture showing operated scar at the temporoparietal region.|
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The patient was symptom free for 7 years. Later she developed headache, vomiting, and blurring of vision without any neurological deficits. The patient was evaluated and diagnosed to have high grade extradural tumor in the right temporoparietal region for which she underwent right temporoparietal craniotomy and decompression and diagnosed as sinodural Ewing's sarcoma [Figure 1]c. The postoperative period was uneventful except for pseudomeningocele, which healed with conservative management. Partial skull/brain radiotherapy of 54 Gy in 27 fractions was given.
At present, the patient presented with pain and swelling over distal third left forearm. Pain is insidious in onset, gradually progressive dull aching in nature. She also complained of cough since 3 months, which was nonproductive,intermittent with no hemoptysis/dyspnea.
Examination revealed fusiform swelling, tenderness, and irregularity over distal left radius [Figure 2]a. Full range of movements of wrist, forearm, and fingers were present. On auscultation, breath sounds over the left inframammary and infra-axillary region were decreased with occasional crepitations. Radiographs of the forearm [Figure 2]b showed a permeative destructive lesion with sclerosis, a small soft tissue mass of the distal shaft of radius.
|Figure 2: (a) Clinical picture showing fusiform swelling over distal radius. (b) Radiographs of the forearm AP view showed a permeative destructive lesion with sclerosis, a small soft tissue mass of the distal shaft of radius. (c) CT abdomen and thorax show a large heterogenous opacity in the left hemithorax mostly pleural deposits. (d) Histopathology showing small round‑like cells that predominate in densely packed sheets|
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CT abdomen and thorax show a large heterogeneous opacity in the left hemithorax mostly pleural deposits [Figure 2]c and [Figure 3]b.
|Figure 3: (a) Clinical picture of distal radius postradiotherapy. (b and c) CT chest and chest X‑ray after radiotherapy showing decreased opacity.|
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FNAC of swelling over the left forearm revealed small round cell tumor, i.e., Ewing's sarcoma/primitive neuroectodermal tumor [Figure 2]d. Later, bone marrow biopsy confirmed the diagnosis as Ewing's sarcoma.
The patient was treated with palliative radiotherapy to the left forearm and left lower chest with 40 Gy/20 fractions/4 weeks. She showed improvement in her general conditions, left radius, and chest [Figure 3]a-c.
| Discussion|| |
Ewing sarcoma accounts for approximately 9% of all primary malignant tumors of bone.  In a report from the Intergroup Ewing's Sarcoma Study, only one of 303 patients had a lesion of the hand, indicating a 0.3% incidence of occurrence. 
Pritchard et al. found no difference in the rates of survival for 5 years or longer between patients who had a lesion in the upper or lower extremity, or with a lesion in the distal part or the proximal part of the extremity. The survival rates were distinctly higher (40% to 50%) when treatment included surgery combined with other modalities. 
Ewing's sarcoma of the hand has a favorable prognosis up to 64% and reports suggest disease-free survival for 2 years or more. Death was due to metastases within 21 months following diagnosis. 
The first case of fifth metacarpal involvement was reported by Bhansali and Desai in 1963 and later in isolated reports; 10 cases were reported in the next 19 years. The variable radiological appearance often makes early diagnosis of Ewing's sarcoma difficult in 24.3% cases. 
Ewing's sarcoma of bone must be distinguished from other poorly differentiated malignant round-cell tumors of bone, including rhabdomyosarcoma, , small-cell osteosarcoma,  mesenchymal chondrosarcoma, ,, lymphoma, leukemia, and especially neuroblastoma and neuroectodermal tumor of bone.  All of the lesions share some of the histological features of Ewing sarcoma. Morphological and cytogenetic studies can help in making the diagnosis. Typically, the cells of Ewing sarcoma are closely packed and poorly differentiated and they have scant cytoplasm that contains glycogen and indistinct cell membranes. Vimentin, a mesenchymal intermediate filament, and cytokeratin have been identified in Ewing sarcoma. The differentiation between Ewing sarcoma and primitive neuroectodermal tumor of bone is imprecise, since the histogenous and the morphological characteristics of these two tumors overlap somewhat. , In our patient, the tumor's cytological characteristics, the absence of Homer-Wright rosettes and of histochemically demonstrable neurofilaments, desmin, and neuron-specific enolase (using a highly specific monoclonal antibody) in the cells, as well as the presence of vimentin, cytokeratin, and cytoplasmic glycogen, all supported the diagnosis of Ewing sarcoma.
| Conclusion|| |
Our patient is surviving for more than 3 years after developing secondaries in right transverse sinodura and 10 years after being diagnosed as Ewing's sarcoma of second metacarpal. At present, she has secondaries in the left lung, liver, and left radius. She is active, attending college, and keeping in a fairly good general condition.
In spite of its multifaceted presentation, Ewing's tumor is an interesting variety of malignant tumor; every case should be assessed and evaluated thoroughly.
| References|| |
|1.||Dryer RF, Buckwalter JA, Flatt AE, Bonfiglio M. Ewing's sarcoma of the hand. J Hand Surg 1979;4:372-4. |
|2.||Kissane JM, Askin FB, Foulkes M, Stratton LB, Shirley SF. Ewing's sarcoma of bone. Clinicopathologic aspects of 303 cases from the intergroup ewing's sarcoma study. Hum Pathol 1983;14:773-9. |
|3.||Pritchard DJ, Dahlin DC, Dauphine RT, Taylor WF, Beabout JW. Ewing's sarcoma a clinicopathological and statistical analysis of patients surviving five years or longer. J Bone Joint Surg 1975;57:10-6. |
|4.||Lacey SH, Danish EH, Thompson GH, Joyce MJ. Ewing's sarcoma of proximal phalnyx: A case report. J Bone Joint Surg 1975;69:6. |
|5.||Bhansali SK, Desai PB. Ewing's Sarcoma: Observation on 107 cases. J Bone Joint Surg 1963;45:541-53. |
|6.||Llombart-Bosch A, Lacombe MJ, Contesso G, Peydro-Olaya A. Small round blue cell sarcoma of bone mimicking atypical ewing's sarcoma with neuroectodermal features: An analysis of five cases with immunohistochemical and electron microscopic support. Cancer 1987;60:1570-82. |
|7.||Yunis EJ. Ewing's sarcoma and related small round cell neoplasms in children. Am J SurgPathol 1986;10:54-62. |
|8.||Sim FH, Unni KK, Beabout JW, Dahlin DC. Osteosarcoma with small cells simulating Ewing's tumor. J Bone Joint Surg 1979;61:207-15. |
|9.||Schajowicz F. Ewing's sarcoma in tumors and tumor-like lesions of bone and joints. New York: Springer; 1981. p. 425 |
|10.||Jaffe R, Agostini RM, Santamaria M, Medina J, Yunis EJ, Goodman M, et al. The neuroectodermal tumor of bone. Am J Surg Pathol 1984;8:885-98. |
[Figure 1], [Figure 2], [Figure 3]