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 Table of Contents  
Year : 2022  |  Volume : 34  |  Issue : 2  |  Page : 234-236

Ewing's sarcoma of the maxilla in an adolescent male – A rare case report

Department of Oral Medicine and Radiology, I.T.S Dental College and Hospital, 47, Knowledge Park III, Greater Noida, Uttar Pradesh, India

Date of Submission27-Jun-2021
Date of Decision28-May-2022
Date of Acceptance29-May-2022
Date of Web Publication22-Jun-2022

Correspondence Address:
Mansimranjit Kaur Uppal
I.T.S Dental College and Hospital, 47, Knowledge Park III, Greater Noida, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaomr.jiaomr_177_21

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Ewing's sarcoma (ES) is an atypical malignant neoplasm of long bones rarely affecting the head and neck region. Here, we report a case of a 13-year-old male patient with a chief complaint of an unhealed socket and swelling in the right mid-facial region, following an extraction. Intraoral examination revealed an unhealed socket with respect to permanent maxillary right second molar region. Advanced imaging such as cone-beam computed tomography (CBCT) revealed an aggressive, expansive, osteolytic lesion involving the right posterior maxillary alveolus, extending into the maxillary sinus. Histopathology and tumor markers such as the cluster of differentiation (CD)99 were deterrent in the diagnosis.

Keywords: CBCT, CD99, Ewing's sarcoma, maxilla, round cell tumor

How to cite this article:
Sharma ML, Srivastava S, Uppal MK, Martolia G. Ewing's sarcoma of the maxilla in an adolescent male – A rare case report. J Indian Acad Oral Med Radiol 2022;34:234-6

How to cite this URL:
Sharma ML, Srivastava S, Uppal MK, Martolia G. Ewing's sarcoma of the maxilla in an adolescent male – A rare case report. J Indian Acad Oral Med Radiol [serial online] 2022 [cited 2023 Jan 31];34:234-6. Available from: http://www.jiaomr.in/text.asp?2022/34/2/234/347914

   Introduction Top

James Ewing first described Ewing's sarcoma (ES) in 1921 as a rare malignant round cell tumor comprising 4–7% of all the primary neoplasms of the bone.[1] It is an aggressive form of childhood malignancy comprising the peripheral primitive neuroectodermal tumor, neuroepithelioma, and Askin's tumor.[2] It is rarely seen before the age of 5 and after 30 years.[1] Its occurrence in the maxillofacial region is estimated to be 1–4%, with mandibular involvement being predominant (69%) and maxilla approximately 28%.[3] Since the maxillary presentation of this disease is a rarity, we present this case report so that the oral physicians are well versed with the clinico-radiological signs of this disease leading to a prompt diagnosis.

   Case Report Top

A 13-year-old male patient reported a chief complaint of unilateral facial swelling of the right mid-facial region for one week following extraction in the upper right back tooth region. History revealed the onset of swelling with mild pain after the tooth extraction due to spontaneous tooth mobility, which showed no signs of regression even after anti-microbial therapy.

   Clinical Description Top

On examination, a solitary, diffuse, extraoral swelling was observed over the right middle third of the face. An intraoral examination revealed an unhealed socket with respect to #17 with the marginal gingiva's erythematous, irregular, granular proliferation on the buccal and palatal aspects. [Figure 1]a. Based on the findings, a provisional diagnosis of osteomyelitis with a differential diagnosis of a granulomatous disease was arrived at.
Figure 1: (a) An ulcero-proliferative lesion on the right maxilla in #17 and #18 (b) IOPAR showing missing #17, complete alveolar bone loss distal to #16 and around the developing #18, giving it a floating tooth appearance

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   Diagnostic Assessment Top

The radiographic examination included an intraoral periapical radiograph (IOPAR), which revealed missing #17, complete alveolar bone loss distal to #16 and around the developing #18, giving it a 'floating tooth appearance' [Figure 1]b.

CBCT of the maxilla revealed an ill-defined, expansile, osteolytic lesion involving the right posterior maxillary alveolus, extending superiorly into the maxillary sinus and posteriorly involving the lateral pterygoid plate [Figure 2]a,[Figure 2]b,[Figure 2]c.
Figure 2: CBCT sections showing (a) An ill-defined, expansile, osteolytic lesion. (b) Involving the right posterior maxillary alveolus. (c) Extending into the maxillary sinus. (d) PET scan showing uptake of the radioactive dye in the right maxilla. (e) Histopathologic section showing sheets of small round cells with large nuclei, scanty cytoplasm, and stroma (Hematoxylin and eosin (H&E) stain, 10x magnification)

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A whole-body contrast-enhanced positron emission tomography-computed tomography (PET-CT) scan revealed anatomical and metabolic regression of metabolically active malignant right maxillary lesion with the standardized uptake of FDG (fluorodeoxyglucose) [Figure 2]d.

A radiographic diagnosis of intraosseous malignancy with differential diagnosis of juvenile ossifying fibroma, eosinophilic granuloma, and lymphoma was given.

After written informed consent, intraoral biopsy was taken which revealed multiple fragments focally lined by keratinized hyperplastic stratified epithelium, displaying ulceration with edematous granulation tissue and round nuclei, suggestive of a malignant round cell tumor [Figure 2]e.

Immunohistochemistry showed strong positivity for vimentin, the cluster of differentiation (CD)99, and FLI-1 (Friend leukemia integration-1), which confirmed the diagnosis of ES.

   Therapeutic Intervention Top

The treatment plan consisted of a combination of chemotherapy followed by five cycles of external beam radiotherapy. Subsequently, another five cycles of adjuvant chemotherapy were administrated for two months.

   Follow-Up Top

The patient visited after six months and was found to be asymptomatic [Figure 3]a and [Figure 3]b.
Figure 3: Follow-up (a) clinical picture of the patient after six months showing normal mucosa (b) CBCT scan revealing evidence of new trabeculae formation

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   Discussion Top

ES is a primary malignant tumor of the bone which presents as a group of small, round, uniform cells with round, lightly stippled nuclei of neuroectodermal origin.[1] The most common presentation in the maxilla is a painful swelling at the involved site, loose teeth, and paraesthesia of the jaws. In this case, both swelling and loose teeth were present.

Radiographically, it represents a mixed, ill-defined, osteolytic lesion.[1] The characteristic 'sunburst' or laminar periosteal “onion skin” reaction, a common radiological feature of ES involving long bones, is rarely seen in jaws.[4],[5] In concurrence with the literature, cone-beam computed tomography (CBCT) revealed an ill-defined, expansile, osteolytic lesion involving the right posterior maxillary alveolus extending into the maxillary sinus. Destruction of buccal and labial cortical plates was appreciated. PET-CT showed uptake of the radioactive dye in the right maxilla.

Special investigations such as genomics have been applied for isolating Ewing Sarcoma RNA Binding Protein-1 (EWSR1)/Friend Leukemia Integration-1 (FLI1) fusion gene translocation, which is positive in approximately 85% of the cases.[6]

In this case, a combination of chemotherapy and radiotherapy was administered. The prognostic factors for the disease are tumor size, primary tumor site, and metastasis.[7] In 2010, as reported by Sari et al.,[8] the 5-year event-free survival rate was 40%, and the 5-year overall survival rate was 47% for patients <18 years. The last systematic review conducted by Margaix-Muñoz et al.[3] in 2017, for 54 years, revealed only 71 case reports of ES in the oral cavity, out of which one-third of the cases occurred in the maxilla.[3]

   Conclusion Top

Ewing's sarcoma is an aggressive tumor that affects mainly children and young adolescents. Therefore, a dentist should have sound knowledge about its clinical and radiological presentation to improve the prognosis of the disease. Extraction should never be carried for a mobile, permanent tooth without understanding the nature of the underlying disease. This disease has a low survival rate; hence, it severely affects the patient's physical, social, psychological, and financial status.

This case lacks the long-term follow-up of the patient for assessment of the quality-of-life post-radiotherapy and chemotherapy administered to the patient, along with any instances of recurrence of the disease.

Ewing's sarcoma is a rare tumor entity that can mimic various head and neck tumors. Hence, a meticulous and holistic examination is the key to early diagnosis, ensuring a good prognosis for this aggressive malignancy.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Mukherjee A, Ray JG, Bhattacharya S, Deb T. Ewing's sarcoma of the mandible: A case report and review of Indian literature. Contemp Clin Dent 2012;3:494-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
Yogesh TL, Shetty A, Keswani H, Rishi D. Aggressive high-grade Ewing's sarcoma of the maxilla: A rare case report. J Oral Maxillofac Pathol 2018;22(Suppl S1):48-53.  Back to cited text no. 2
Margaix-Muñoz M, Bagán J, Poveda-Roda R. Ewing sarcoma of the oral cavity. A review. J Clin Exp Dent 2017;9:e294-301.  Back to cited text no. 3
Poorly defined radiolucencies. In: Langlais RP, Langland OE, Nortjé CJ. Diagnostic Imaging of the Jaws. 1st ed. Malvern: Williams & Wilkins; 1994. p. 414-7.  Back to cited text no. 4
Lokesh K, Kannabiran J, Eregowda SM. Maxillary Ewing's sarcoma: A report of the benign lesion with nocuous pathology. J Indian Acad Oral Med Radiol 2016;28:203-6s.  Back to cited text no. 5
  [Full text]  
Zöllner SK, Amatruda JF, Bauer S, Collaud S, de Álava E, DuBois SG, et al. Ewing sarcoma-diagnosis, treatment, clinical challenges, and future perspectives. J Clin Med 2021;10:1-59.  Back to cited text no. 6
Bosma SE, Ayu O, Fiocco M, Gelderblom H, Dijkstra PDS. Prognostic factors for survival in Ewing sarcoma: A systematic review. Surg Oncol 2018;27:603-10.  Back to cited text no. 7
Sari N, Toğral G, Cetindağ MF, Güngör BS, Ilhan IE. Treatment results of the Ewing sarcoma of bone and prognostic factors. Pediatr Blood Cancer 2010;54:19-24.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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