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LETTER TO EDITOR
Year : 2019  |  Volume : 18  |  Issue : 3  |  Page : 177-179  

Large extraskeletal ewing sarcoma of the hand: A rare entity


1 Department of Radiology, Subharti Medical College, Meerut, Uttar Pradesh, India
2 Department of Pediatrics, Subharti Medical College, Meerut, Uttar Pradesh, India

Date of Web Publication14-Aug-2019

Correspondence Address:
Dr. Sonal Saran
Flat No. 6, Swami Shraddhanand Bhawan, Subharti University, Meerut - 250 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aam.aam_55_18

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How to cite this article:
Saran S, Kharbanda A, Malik S. Large extraskeletal ewing sarcoma of the hand: A rare entity. Ann Afr Med 2019;18:177-9

How to cite this URL:
Saran S, Kharbanda A, Malik S. Large extraskeletal ewing sarcoma of the hand: A rare entity. Ann Afr Med [serial online] 2019 [cited 2019 Aug 18];18:177-9. Available from: http://www.annalsafrmed.org/text.asp?2019/18/3/177/264319



Sir,

A 17-year-old male patient presented with a huge mass in the right hand. The mass was first noticed 6 months back; since then, the lesion slowly increased in size with superimposed ulcerative changes over the skin. Systemic examination of the patient was normal. The lesion was located in the 4th web space and measured approximately 11 cm × 11 cm × 10 cm in size [Figure 1]. Conventional radiograph of the hand was performed in anteroposterior and oblique views which showed large soft tissue density mass lesion in the 4th web space with lobulated margins. No obvious destruction of the bones was seen [Figure 2]. The patient was then subjected to ultrasonography which showed large heteroechoic mass lesion with internal hyperechoic foci casting distal acoustic shadowing suggestive of calcification. On color Doppler examination, the lesion demonstrated increased internal vascularity [Figure 3]. Magnetic resonance imaging (MRI) of the hand was also performed with intravenous gadolinium contrast agent. On MRI, the lesion was seen in the 4th web space, completely surrounding the 5th digit. The lesion was relatively isointense to adjacent musculature on T1W sequence and relatively hyperintense on T2W and short-tau inversion recovery sequence. T1W sequence after intravenous contrast injection showed intensely enhancing mass lesion with scattered nonenhancing necrotic foci [Figure 4]. The patient was then evaluated for metastasis which was negative. Wide excision of the mass was planned considering the cosmetic deformity and imaging features suggesting malignancy [Figure 5]. The mass was removed along with the medial three digits and was sent for histopathological examination. Histopathological sections showed unencapsulated tumor tissue composed of sheets and lobules of small round tumor cells separated by thick and thin fibrovascular stroma. Cells had vesicular to finely granular chromatin consistent with small round cell tumor which was later proved to be Ewing's sarcoma on immunohistochemistry [Figure 6].
Figure 1: (a and b) Photograph of the right hand of the patient showing large mass lesion with fungating growth pattern and prominent vessels

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Figure 2: Conventional radiograph of the hand in anteroposterior (a) and oblique (b) views showing large soft tissue density mass lesion in the 4th web space with lobulated margins. No obvious destruction of the bones is seen

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Figure 3: (a) Ultrasonographic examination of the hand showing heteroechoic lesion with internal hyperechoic foci casting distal acoustic shadowing suggestive of internal calcification. (b) On color Doppler examination, the lesion demonstrated increased internal vascularity

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Figure 4: (a) Coronal T1W sequence showing the mass located in the 4th web space and completely surrounding the 5th digit. The lesion is relatively isointense to adjacent musculature. (b) Axial T2W sequence showing the mass appearing relatively hyperintense. (c) Coronal short-tau inversion recovery sequence showing the mass located in the 4th web space and completely surrounding the 5th digit. The lesion is hyperintense with respect to adjacent musculature. (d) Axial T1W sequence after intravenous contrast injection showing intensely enhancing mass lesion with scattered nonenhancing necrotic foci

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Figure 5: (a and b) Wide excision of the mass was performed with medial three fingers

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Figure 6: (a and b) Sections show unencapsulated tumor tissue composed of sheets and lobules of small round tumor cells separated by thick and thin fibrovascular stroma. Cells have vesicular to finely granular chromatin consistent with small round cell tumor which was later proved to be Ewing's sarcoma on immunohistochemistry

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Skeletal/extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, and Askin tumors together form the Ewing sarcoma family of tumors with a characteristic karyotype abnormality involving translocation between chromosome 11 and 22 in common.[1] Histologically, these tumors demonstrate crowded sheets of small round blue cells or lobules of such cells divided by a small amount of fibrous stroma.[2]

Angervall and Enzinger first described the extraskeletal location of Ewing sarcoma in 1975.[3] Askin et al. described a series of extraskeletal Ewing sarcoma involving the chest wall and referred them as Askin tumors.[4] Peak incidence occurs in the second decade of life with slight male predominance. Clinically, the patient presents with large, rapidly growing soft tissue mass. The mass is generally solitary and can be superficial or deep. Upper extremity accounts for only 3% of cases of extraskeletal Ewing sarcoma.[5] There are very few case reports describing extraskeletal Ewing sarcoma of the hand.[6],[7]

The radiological evaluation includes conventional radiography followed by ultrasonography, computed tomography (CT), and MRI. Conventional radiographic findings are nonspecific and include generally a large soft tissue mass. Calcification in the mass is not frequently identified, being reported in only 25% of cases. As the tumor is quite aggressive, adjacent bone erosion and cortical penetration with or without periosteal reaction can be seen in up to 40% of cases. On ultrasonography, large lesions are difficult to evaluate due to small field of view; however, internal characteristics of the lesion include heterogeneous echotexture with cystic areas likely representing the presence of necrosis and/or hemorrhage. On power Doppler examination, the lesion often demonstrates increased vascularity.[8]

CT with contrast administration can be performed to evaluate the complete extent of the lesion by reformatting it in different planes. CT also shows exquisite bony details and helps to identify any subtle bone erosion or periosteal reaction. Low attenuation areas show necrosis and areas of hyperdensity show hemorrhage. MRI on the other hand shows the extension of the lesion in soft tissue planes and helps to evaluate the internal characteristic of the lesion. Lesion outline with respect to adjacent soft tissue structures is better evaluated with the help of MRI as compared to CT. On MRI, the mass generally shows heterogeneous signal intensity with predominantly increased signal intensity on T2W sequence and decreased signal intensity on T1W sequence as compared to adjacent muscles. Areas of hemorrhage show signal intensity depending on the stage of degradation of blood products. On susceptibility-weighted sequence, both calcification and hemorrhage will demonstrate foci of blooming.[8]

Postcontrast images generally show uniform homogeneous enhancement of solid component and peripheral enhancement of the cystic component. Evaluation of invasion into adjacent neurovascular structures and metastatic workup completes the radiological examination which thereby helps the surgeon before performing the aggressive surgical resection.[8]

Treatment of extraskeletal Ewing sarcoma includes neoadjuvant chemotherapy followed by aggressive surgical excision followed with/without radiotherapy. Postoperative follow-up MRI with contrast administration can detect local recurrence as a soft tissue mass with almost similar signal intensity as the operated lesion.[9],[10]

Extraskeletal Ewing sarcoma is a very rare tumor of the hand. Early diagnosis and treatment is necessary to prevent regional and distant spread of this very aggressive neoplasm. There are very few case reports describing extraskeletal Ewing sarcoma of the hand, and more literature is needed to establish a protocol for early clinical, radiological, and histopathological diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Aurias A, Rimbaut C, Buffe D, Zucker JM, Mazabraud A. Translocation involving chromosome 22 in Ewing's sarcoma. A cytogenetic study of four fresh tumors. Cancer Genet Cytogenet 1984;12:21-5.  Back to cited text no. 1
    
2.
Fletcher CD, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC; 2002.  Back to cited text no. 2
    
3.
Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975;36:240-51.  Back to cited text no. 3
    
4.
Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: A distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979;43:2438-51.  Back to cited text no. 4
    
5.
Eggli KD, Quiogue T, Moser RP Jr. Ewing's sarcoma. Radiol Clin North Am 1993;31:325-37.  Back to cited text no. 5
    
6.
Daw JL, Wiedrich TA, Bauer BS. Congenital primitive neuroectodermal tumor of the hand: A case report. J Hand Surg Am 1997;22:743-6.  Back to cited text no. 6
    
7.
Erdmann D, Brown RE, Rumbolo PM. Congenital neuroepithelioma in an infant hand. J Hand Surg Br 1996;21:117-20.  Back to cited text no. 7
    
8.
Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ, et al. From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation. Radiographics 2013;33:803-31.  Back to cited text no. 8
    
9.
Marina NM, Etcubanas E, Parham DM, Bowman LC, Green A. Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer 1989;64:1952-60.  Back to cited text no. 9
    
10.
Fleegler EJ. An approach to soft tissue sarcomas of the hand and upper limb. J Hand Surg Br 1994;19:411-9.  Back to cited text no. 10
    


    Figures

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



 

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