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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 1  |  Page : 66-69
Ewing's sarcoma of the maxilla


1 Department of Oral Pathology and Microbiology, Bapuji Dental College and Hospital, Davangere, Hubli - 580030, Karnataka, India
2 Department of Oral and Maxillofacial Surgery, Bapuji Dental College and Hospital, Davangere, Hubli - 580030, Karnataka, India

Click here for correspondence address and email

Date of Submission05-May-2006
Date of Decision01-Mar-2007
Date of Acceptance09-Mar-2007
 

   Abstract 

Ewing's sarcoma is an uncommon malignancy that occurs usually in childhood. In most reported cases, the occurrence of this tumor is more in the mandible than the maxilla. A case of Ewing's sarcoma of the maxilla together with its associated structures is presented and discussed with complete clinical, radiographic, and histological evaluation. Although the prognosis remains poor, an increased rate of survival is now associated with early diagnosis and combined therapy.

Keywords: Ewing′s sarcoma, child, treatment outcome

How to cite this article:
Vikas Prasad B, Ahmed Mujib B R, Bastian T S, David Tauro P. Ewing's sarcoma of the maxilla. Indian J Dent Res 2008;19:66-9

How to cite this URL:
Vikas Prasad B, Ahmed Mujib B R, Bastian T S, David Tauro P. Ewing's sarcoma of the maxilla. Indian J Dent Res [serial online] 2008 [cited 2020 Oct 31];19:66-9. Available from: https://www.ijdr.in/text.asp?2008/19/1/66/38935
James Ewing was the first to describe Ewing's sarcoma, which was named after him in 1921. Ewing's sarcoma, a primary malignant tumor affecting the skeletal system, is now thought to arise from immature reticulum cells or primitive mesenchymal cells of the bone marrow. [1]

This tumor accounts for approximately 4-10% of all malignant neoplasms of the bone, with pelvic girdle and the long bones of the lower extremities being the most common locations. It is an uncommon malignancy, which usually occurs in childhood. [2] Ewing's sarcoma rarely affects the jaws, and the incidence of jaw involvement has been reported as varying from 1% to 10%. [3]

Since the prognosis is poor due to its uncontrolled potential for metastatic spread, early and appropriate intervention is warranted.


   Case Report Top


An 18-year-old male patient reported with a complaint of swelling since 6 months and pain for past 2 months on the left side of the face. He gave a history of trauma (a blunt injury in a road traffic accident) in the same region a year back. Swelling was insidious in onset. Over a period of 6 months, the entire left side of the midface was involved with progressive decrease in left nasal airway competence and reduction in left eye opening [Figure - 1].

The patient's history revealed no systemic problems. Moreover, a general physical examination revealed no significant disorders. A gross facial asymmetry with a well-circumscribed swelling measuring 12 × 8 cm was present on the left midface. There was restricted eye opening with decreased palpebral fissure width and antimongoloid slant in the left eye with moderate proptosis. Visual acuity was present at 1 ft range with normal eye movements.

On palpation, swelling was tender, bony hard in consistency, non-pulsatile and non-compressible with no evidence of secondary changes. Intraorally, swelling and obliteration of left maxillary vestibule was noted, extending from the region of left upper canine to maxillary tuberosity.

Intraoral palpation revealed a lobulated surface with positive fluctuancy posteriorly in the tuberosity region. There was grade-1 mobility of teeth from lateral incisor to second molar in the left quadrant and tenderness throughout the affected region.

Considering the patient's history and clinical observations, a malignant tumor of the maxilla was suspected, possibly an osteosarcoma, chondrosarcoma or a metastatic carcinoma. Following investigations were carried out.

  1. Radiographs and a CT scan
  2. Hematological and biochemical investigations
  3. An incisional intraoral biopsy


Radiographic findings

An OPG revealed a mixed radiolucent and radio-opaque lesion involving maxilla, nasal cavity, and orbitozygomatic region. Lateral nasal wall erosion with septal deviation, elevated orbital floor with reduced orbital volume, clouding of left maxillary antrum, and loss of lamina dura with displacement of roots of few teeth of the affected quadrant were also noted [Figure - 2].

Computed tomography

A preliminary lateral tomogram of the skull was obtained; serial transaxial sections were then performed from the infratemporal fossa to the hard palate employing 5-mm slice thickness with appropriate angulations. Sixty milliliters of Angiograffin 65% was used for contrast enhancement.

Observations

An expansile soft tissue density mass lesion of 64 HV enhancing up to 70 HV was seen in left maxillary sinus with expansion of the wall with thinned-out and eroded bony margins. Postero-lateral and antero-lateral walls of left maxillary sinus showed cortical break and extension into infratemporal, temporal, buccal, and facial tissues. Cortical breach was observed in the superior and medial walls of the antrum with intraorbital and nasal cavity extensions. Erosions of the left zygomatic bone were seen with hypo- and hyper-dense areas.

Impression

CT features were suggestive of an expansile mass with erosive borders in left maxilla (suggestive of malignancy) [Figure - 3].

Hematology report

Hb - 11.6 gm%, TC - 7350 cells/mm 3 , ESR - 16 mm hr -1 , PCV - 35%; bleeding time - 1 min, clotting time - 4 min; DC - neutrophils - 64%, lymphocytes - 33%, eosinophils - 03%, monocytes and basophils - 0%; blood group - AB, Rh type - +ve, HIV - negative (tridot), HBSAg - negative (surface line).

Biochemical investigations

Using a semiautomatic analyzer (Erbachem Pro), the following were investigated: blood sugar, urea, SGOT, SGPT, K, Na and Ca. The above parameters were within the normal range.

Histopathology

Sections were stained using H and E, PAS, and reticulin stains.

The H and E sections revealed solid sheets and masses of small round cells with scanty stroma. Intervening between the tumor cells, few connective tissue septa were seen. The cells revealed faint cytoplasmic membrane with large hyperchromatic nuclei with scanty cytoplasm. In certain areas, the tumor cells were arranged in rosettes perivascularly. Infrequent mitotic figures were also observed. Significant necrotic areas were also seen [Figure - 4].

PAS sections were positive for intracytoplasmic glycogen content [Figure - 5].

Reticulin was positive around vessels and in collagenous septa [Figure - 6].

The typical histologic appearance with the presence of PAS-positive glycogen and the absence of any tumor osteoid was conclusive to diagnose this tumor as Ewing's sarcoma.

Treatment

Primary tumor resection was done by extended maxillectomy, which included hard palate till midline and a part of soft palate, lateral nasal wall with septum and naso-lacrimal apparatus, floor of orbit, zygomatic bone, and arch pterygoid plates.

This was followed by reconstructive procedures. Three weeks post-operatively, the patient was subjected to chemotherapy. Rehabilitation was done later with prosthetic obturator, and reasonable facial harmony was achieved. The follow-up of this case for past 1 year revealed no recurrence till date.


   Discussion Top


Ewing's sarcoma is a rare malignant tumor affecting the skeletal system. This tumor is found to affect children and young adults. Swelling, pain, parasthesia, and loose teeth are frequent symptoms. [3] Unlike the present case, mandible is found to be more commonly affected than the maxilla. Radiographically, it is most often seen as destructive expansile, mottled radiolucent lesion, which may produce a laminated periosteal reaction. [3] Some authors point out that radiographic appearance of "onion skinning" is a characteristic sign of Ewing's lesion of the bone. [4],[5] While it may be true in most of the cases, some dissenting authors have stated that this pattern is either uncommon or exceedingly rare in jaw lesions. [6],[7] In the jaw, an osteolytic radiolucent area with cortical destruction was seen in most reported cases. With respect to teeth, some radiological features were noted, which include periodontal space widening, loss of lamina dura, root resorption, displacement or more commonly destruction of unerupted tooth follicles. [1]

An extraskeletal form of this tumor has been described by Angervall and Enzinger, which is termed Ewing's sarcoma of soft tissues. The ultra structural characteristics of the cells of this tumor studied by Gillespie and his associates proved that these cells are identical to those of the typical Ewing's cells. [8]

Histopathologically, this tumor must be differentiated from other small, round cell tumors, including small cell osteosarcoma and malignant neoplasms like mesenchymal chondrosarcoma, rhabdomyosarcoma, malignant lymphoma, eosinophilic granuloma, neuroectodermal tumors, and metastatic neuroblastoma.

Of these, eosinophilic granuloma, malignant lymphoma, and metastatic neuroblastoma are most easily mistaken histologically for Ewing's sarcoma. Eosinophilic granuloma can be distinguished because of the presence of "histiocytic" features, that is, abundant, indistinct eosinophilic cytoplasm with oval or indented nucleus. Multinucleated cells may be present. Malignant lymphoma contains lymphoid cells, intermixed with round cell components of varying size and cytoplasmic contents. The nuclei of these cells are round or oval with a distinct nuclear membrane, which is sometimes smooth or cleaved. The PAS stain is usually negative, and reticulin stain is often positive.

The cells of metastatic neuroblastoma have a yellow fluorescence when exposed to formalin vapor and are neuron specific and enolase positive. [9]

The prognosis of Ewing's sarcoma is poor because of multiple metastases most commonly to the bone, lung, lymph node, and liver, which may occur within a few months after the onset of the tumor. [10] Recently, some reports of early and prompt diagnosis with combined surgery, radiation, and chemotherapy protocols have raised the five-year survival rate of patients with Ewing's sarcoma from 16% to 74%. [11]


   Conclusion Top


In general, Ewing's sarcoma is a rare malignancy that may affect the facial bones of young individuals. Even with an isolated area of Ewing's sarcoma, the risk of metastasis is so great that it may warrant multiple therapy modalities. In case of suspected cases, an evaluation of the lesion should be carried out using plain films, CTs, MRIs, bone scan, and biopsy. After treatment, it is mandatory to provide suitable prosthesis, so that these young patients lead a quality life.

 
   References Top

1.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. 1  [PUBMED]  
2.Berk R, Heller A, Heller D, Schwartz S, Klein EA. Ewing's sarcoma of the mandible: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:159-62.  Back to cited text no. 2    
3.Arafat A, Ellis GL, Adrian JC. Ewing's sarcoma of the jaws. Oral Surg Oral Med Oral Pathol 1983;55:589-96.  Back to cited text no. 3  [PUBMED]  
4.McCormack LJ, Dockerty MB, Ghormley RH. Ewing's sarcoma. Cancer 1952;5:85-99.  Back to cited text no. 4    
5.Wilmer D. Radiology of Bone tumors and allied disorders. W.B. Saunders: Philadelphia; 1982. p. 2251.  Back to cited text no. 5    
6.Pindborg JJ, Hjorting-Hansen. Atlas of diseases of the jaws. W.B. Saunders: Philadelphia; 1974. p. 36.  Back to cited text no. 6    
7.Worth HM. Principles and practice of oral radiology interpretation. Year Book Medical Publishers: Chicago; 1963. p. 580-1.  Back to cited text no. 7    
8.Angervall L, Enzinger FM. Extra skeletal neoplasm resembling Ewing's sarcoma. Cancer 1975;36:240-51.  Back to cited text no. 8  [PUBMED]  
9.Bacchini P, Marchetti C, Mancini L, Present D, Bertoni F, Stea G. Ewing's sarcoma of the mandible and maxilla. Oral Surg Oral Med Oral Pathol 1986;61:278-83.  Back to cited text no. 9  [PUBMED]  
10.Fielding AF, Lindemeyer R, Harris JW, 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. 10    
11.Hustu HO, Holton C, James D Jr, Pinkel D. Treatment of Ewing's sarcoma with concurrent radiotherapy and chemotherapy. J Pediatr 1968;73:249-51.  Back to cited text no. 11  [PUBMED]  

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Correspondence Address:
B Vikas Prasad
Department of Oral Pathology and Microbiology, Bapuji Dental College and Hospital, Davangere, Hubli - 580030, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.38935

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    Figures

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

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