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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 2  |  Page : 203-206

Maxillary Ewing's sarcoma: A report of innocuous lesion with nocuous pathology


1 Private Practice, Bangalore, Karnataka, India
2 Department of Oral Medicine and Radiology, Bangalore Institute of Dental Sciences and Hospital, Bangalore, Karnataka, India
3 Department of Oral Medicine and Radiology, AECS Maaruti College of Dental Sciences, Bangalore, Karnataka, India

Date of Submission24-Sep-2015
Date of Acceptance13-Nov-2016
Date of Web Publication02-Dec-2016

Correspondence Address:
Kavyashree Lokesh
11th Main, Indiranagar, Bangalore - 560 008, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.195140

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   Abstract 

Ewing's sarcoma represents a highly lethal and a rare malignancy, which predominately prevails in the long bones and skeleton. Its occurrence in the oral cavity has been estimated to be 1-2% of the overall reported cases. The common presentation of this malignancy overlaps with that of other malignant lesions affecting the jaws. Early diagnosis and treatment initiation improves the overall prognosis, even though the estimated survival rate is 5 years in approximately 4-7% of the patients following therapy. Treatment in concordance to other bone malignancies such as osteosarcoma and chondrosarcoma has been tried and is known to improve and prolong the survival rate. Here, we report a case of this rare malignancy occurring in the jaws with an unusual presentation as a common dental problem of an unhealed and infected extraction socket.

Keywords: CD99 marker, cytogenetics, Ewing′s sarcoma, PNET


How to cite this article:
Lokesh K, Kannabiran J, Eregowda SM. Maxillary Ewing's sarcoma: A report of innocuous lesion with nocuous pathology. J Indian Acad Oral Med Radiol 2016;28:203-6

How to cite this URL:
Lokesh K, Kannabiran J, Eregowda SM. Maxillary Ewing's sarcoma: A report of innocuous lesion with nocuous pathology. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2021 Apr 13];28:203-6. Available from: https://www.jiaomr.in/text.asp?2016/28/2/203/195140


   Introduction Top


Established with a historical background of approximately 8-10 decades, Ewing's sarcoma was first described by James Ewing in the year 1921. Earlier literature suggests describing Ewing's sarcoma as a malignancy of childhood and adolescence, however, recently, rare reports of its occurrence in the old age group has also been proposed. A well-established epidemiology of the reported cases reveals Ewing's sarcoma as a rare entity accounting for 6% of all malignant bone tumors. Synonymously, a soft tissue counterpart of Ewing's termed as the primitive neuroectodermal tumor (PNET) has also been described by several authors. [1],[2],[3],[4] Its occurrence in the oral cavity is predominantly observed in the posterior mandibular arch and less commonly involving other sites such as the maxilla. [5] Here, we report a case of this rare malignancy involving the maxillary jaw masquerading as a routine dental problem of an unhealed extraction socket.


   Case Report Top


A female patient aged approximately 18 years reporting to the outpatient department presented with persistent pain and unilateral facial swelling of the right side since 20 days following a tooth extraction in relation to the right upper second molar. Patient related the onset of pain and swelling to the tooth extraction, which was undertaken at a private clinic elsewhere due to spontaneous mobility. Patient reported a sudden onset of swelling with rapid progression but with no evidence of regression. Pain as described by the patient was mild-to-moderate in intensity, intermittent in nature, and aggravating on mastication, which could be attributed to the adjacent tooth mobility. Furthermore, related history of breathing difficulties, nasal, ocular, and visual disturbances were not reported.

On examination, a diffuse swelling in the middle third of the face in the anterior maxillary region measuring approximately 4 × 4 cm was observed. The swelling caused fullness of the right side of the face extending to the midline region of the upper lip obliterating the philtrum. The skin overlying the swelling appeared normal, freely movable, with no evidence of secondary changes. Palpatory findings revealed soft, fluctuant swelling, which was nontender with no associated local rise in temperature. Concomitant oral examination revealed a well-circumscribed vestibular swelling localized to the buccal and palatal aspect of 16, 17, and 18 region. The surface of the swelling appeared uneven because of the presence of an extracted tooth socket covered by tissue sloughing necrosis in the region of 18 [Figure 1]. On palpation, the swelling appeared tender, hard in consistency, with biotical expansion extending from 15 to 18 regions. No evidence of bleeding, discharge, or paresthesia was noted.
Figure 1: Intraoral view showing unhealed extraction socket with swelling of the posterior maxilla

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From the patient history and abovementioned clinical findings, a clinical diagnosis of post-extraction complication in terms of osteomyelitis was considered with a possible differential diagnosis of benign and malignant neoplasm. Initial radiographic examination included an intraoral periapical view, maxillary occlusal view [Figure 2], and an extraoral panoramic view [Figure 3], which revealed an ill-defined periapical radiolucency of the right maxillary region extending from 15 to 18 region with irregular and ragged borders, suggestive of a rapid osteolytic lesion with associated root resorption and loss of lamina dura in the region of 15, 16, and 17. Further radiographic examination with paranasal sinus view revealed a complete obliteration and opacification of the right maxillary sinus. Additional investigation with computed tomography revealed an extensive, heterogeneously enhancing solid mass in the right maxilla, extending into the maxillary sinus and infiltrating into the nasal and orbital cavities [Figure 4].
Figure 2: Occlusal view showing radiolucent lesions with ragged and irregular borders of the posterior maxilla

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Figure 3: Orthopantomograph showing irregular radiolucent lesion with ragged borders of the right maxilla

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Figure 4: Reconstructed computed tomography image showing destruction and infiltration into the orbital, nasal, and infratemporal regions

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Based on the radiographic findings, a malignancy, most likely a primary bone tumor, or a lymphoma was suspected and fine-needle aspiration cytology (FNAC) was performed. As a routine investigation, complete blood investigation (hemogram) was advised, which revealed leukocytosis with a raised erythrocyte sedimentation rate (ESR). FNAC findings showed malignant small round cells [Figure 5] suggestive of possible histological differential diagnosis of neuroblastoma, rhabdomyosarcoma, lymphoma, and Ewing's sarcoma. Immunohistochemistry was carried out to confirm the diagnosis which showed strong positivity to the immunohistochemical marker CD99 [Figure 6] confirming the diagnosis of Ewing's sarcoma. Other markers such as cytokeratin, desmin (specific for rhabdomysarcoma), chromogranin (specific for neuroblastoma), and vimentin were also used to rule out other possible small round cell malignancies. As an additional investigation for confirmation, a cytogenetic study was carried out, which showed a translocation between chromosome 11 and 22. Thus, from the above investigatory findings, a confirmatory diagnosis of Ewing's sarcoma of the maxilla was made. Further imaging with total body scintscan and chest X-ray were advised to rule out any occult primary and metastatic sites. The results showed no additional uptake of the dye except in the primary tumor of the maxilla [Figure 7]. Patient underwent chemotherapy as the prime treatment modality with adriamycin and bleomycin for 6 cycles, following which the patient expired.
Figure 5: Histopathological image with hematoxylin and eosin staining showing malignant round cells

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Figure 6: Histopathological image showing malignant round cells with a string positivity to CD99

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Figure 7: Positron emission tomography scan showing uptake of the radioactive dye in the right maxilla

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   Discussion Top


Ewing's sarcoma described by James Ewing is a primary malignant tumor of the bone, arising from the reticular endothelial cells of the bone marrow. [6] It is often described as a rare, aggressive bone tumor accounting to 4-7% of all the primary bone malignancies. Its occurrence in the maxillofacial region, as estimated by Wood et al., is 2-7%, predominantly involving the mandibular ramus. [7] The present report describes the rare occurrence of this tumor in the maxilla with uncommon clinical features evading the aggressiveness of the malignancy. Although literature suggests clinical presentation of the swelling and pain as the classical presenting signs, our present case also had the aforementioned features following tooth mobility and extraction.

Radiological features of irregular lytic lesion are a profound finding in previously reported cases, which was also present in our case. Some cases have also been reported with findings of periosteal reactions such as onion peel and sun spicule or sun ray pattern, which was not evident in our case. [8],[9] Hematological findings often provide a nonspecific diagnostic aid, which in approximately 85% of the cases show leucocytosis and a raise in ESR. Histological findings of atypical round cells is an engraved feature of this malignancy, however, other malignant tumors show similar round cell features on histological sectioning. [10] Immunohistochemistry with markers such as Myc 2 (CD99), cytokeratin, desmin (specific for rhabdomysarcoma), chromogranin (specific for neuroblastoma), and vimentin helps in differentiating the type of round cell malignancy, and is thus the confirmatory investigation to arrive at the diagnosis. Additional studies such as cytogenetic study often support the final diagnosis. Round cell malignancy with a strong positivity to the Myc 2 or CD99 immunohistochemical marker was observed in our case, which was confirmed through cytogenetic sutudy. [11],[12]

Although Ewing's sarcoma is a highly radiosensitive malignancy, it rapidly metastasizes to the bone and lungs. Combined radiotherapy with surgery has, therefore, been an ineffective therapy with an overall survival of 5 years in 4-7% of patients. [13] Bacci et al. investigated combined chemotherapy drugs including vincristine, doxurubucin, cyclophosphomide, actinomycin D, and ifosfamide as standard treatment regimen to improve the survival rate to 5 years in 45-75% of the cases. [14]


   Conclusion Top


Ewing's sarcoma is, thus, a rare malignancy with a fatal prognosis. Early diagnosis with initiation of treatment can improve the overall survival rate. The scarcity of reported cases in literature often evokes a dilemma in the diagnosis of this malignancy and identification of its possible metastasis to distant sites, thus, compromising the overall outcome of therapy. However, with advancing techniques, such as immunohistochemistry and cytogenetic studies, diagnosis of this rare malignancy has become simpler and feasible.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Gupta S, Gupta OP, Mehrotra S, Mehrotra D. Ewing's sarcoma of maxilla: A rare presentation. Quintessence Int 2009;40:135-40.  Back to cited text no. 1
    
2.
Wexler LH, Kacker A, Piro JD, Haddad J Jr, Close LG. Combined modality treatment of Ewing's sarcoma of the maxilla. Head Neck 2003;25:168-78.  Back to cited text no. 2
    
3.
Fielding AF, Lindemeyer R, Wood-Harris J, Hartman MJ. Ewing's sarcoma of the mandible: A combined approach to treatment. J Clin Pediatr Dent 2002;26:409-12.  Back to cited text no. 3
    
4.
Kawabata M, Yoshifuku K, Sagara Y, Kurono Y. Ewing's sarcoma/primitive neuroectodermal tumor occurring in the maxillary sinus. Rhinology 2008;46:75-8.  Back to cited text no. 4
    
5.
Talesh KT, Motamedi MH, Jeihounian M. Ewing's sarcoma of Mandibular condyle: Report of a case. J Oral Maxillofac Surg 2003;61:1216-9.  Back to cited text no. 5
    
6.
Wang CL, Yacobi R, Pharoah M, Thorner P. Ewing's Sarcoma: Metastatic tumor to the jaw. Oral Surg Oral Med Oral Pathol 1991;71:597-602.  Back to cited text no. 6
    
7.
Wood RE, Nortje CJ, Hesseling P, Grotepass F. Ewing's tumor of the jaw Oral Surg Oral Med Oral Pathol 1990;69:120-7.  Back to cited text no. 7
    
8.
Vohra VG. Roentgen manifestations in Ewing's sarcoma, a study of 156 cases. Cancer 1967;20:727-33.  Back to cited text no. 8
    
9.
Goaz PW, White SC. Oral radiology: Principles and interpretation. 3 rd ed. St. Louis: Mosby; 1994.  Back to cited text no. 9
    
10.
Regezi JA, Sciubba JJ. Oral Pathology, Clinical Pathologic Correlations. 3 rd ed. Philadelphia: Saunders; 1999. p. 407-9.  Back to cited text no. 10
    
11.
Ushigome S, Machinami R and Sorensen PH. Ewing sarcoma/Primitive neuroectodermal tumor (PNET). World Health Organization Classification of Tumors': Pathology and Genetics, Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2002. p. 298-300.  Back to cited text no. 11
    
12.
Smith R, Owen LA, Trem DJ, Wong JS, Whangbo JS, Golub TR, et al. Expression profiling of EWAS/FLI identifies NKX2.2 as a critical target gene in Ewing's sarcoma. Cancer Cell 2006;9:405-16.  Back to cited text no. 12
    
13.
Baude-Brogniez A, Baranzelli MC, Ferri J, Piot B, Demaille MC, Lecomte-Houcke M, et al. Ewing's sarcoma of the jaw. The value of multidisciplinary management. Apropos of 4 cases. Rev Stomatol Chir Maxillofac 1999;100:34-7.  Back to cited text no. 13
    
14.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003;348:694-701.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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