Journal of Indian Academy of Oral Medicine and Radiology

: 2015  |  Volume : 27  |  Issue : 3  |  Page : 449--452

Destructive radiolucent lesion involving right maxillary alveolus: Report of a rare case

Manjiri Joshi1, Rajendrasinh S Rathod2, Arpan Shah2, Rahul Thakkur3,  
1 Department of Oral Medicine and Radiology, Manubhai Patel Dental College and Hospital, Vadodara, Gujarat, India
2 Department of Oral Pathology and Microbiology, Manubhai Patel Dental College and Hospital, Vadodara, Gujarat, India
3 Department of Oral and Maxillofacial Surgery, Manubhai Patel Dental College and Hospital, Vadodara, Gujarat, India

Correspondence Address:
Manjiri Joshi
Department of Oral Medicine and Radiology, Manubhai Patel Dental College and Hospital, Vadodara - 390 011, Gujarat


Ewing«SQ»s sarcoma/primitive neuroectodermal tumor (ES/PNET) usually arises in the long bones of extremities. It is uncommon to find ES in the head and neck region, particularly in gnathic bones. It comprises 6-8% of all primary bone malignancies. Only 1% of cases is reported with jaw involvement and has mandibular predilection. Even with early intervention, patients with metastasis have approximately 20% chance of 5-year survival. Here, we report a rare case of ES involving right maxillary alveolus.

How to cite this article:
Joshi M, Rathod RS, Shah A, Thakkur R. Destructive radiolucent lesion involving right maxillary alveolus: Report of a rare case.J Indian Acad Oral Med Radiol 2015;27:449-452

How to cite this URL:
Joshi M, Rathod RS, Shah A, Thakkur R. Destructive radiolucent lesion involving right maxillary alveolus: Report of a rare case. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2022 Dec 5 ];27:449-452
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Ewing's sarcoma (ES) is a rare malignant round cell tumor. James Ewing described this tumor initially in the year 1921. The mean age of occurrence in the head and neck region is 10.9 years. ES generally affects white population and has male predilection (male:female ratio = 1.3-1.5:1). [1] According to the anatomical site of occurrence, it is classified as:

Intraosseous (most common),Extraskeletal (less common), andPeriosteal (rare) types. [2] ES is the most common malignant neoplasm of osseous origin in childhood and adolescent age group. However, irrespective of age, higher frequency of other bone tumors makes it relatively uncommon. It comprises 6-8% of all primary bone malignancies. [3] Even with early intervention, patients with metastasis have approximately 20% chance of 5-year survival. ES usually arises in the long bones of extremities. It is uncommon to find ES in the head and neck region, particularly in gnathic bones. [4],[5] Posterior mandible is the most favored site for jaw tumors. Very few cases of ES arising in maxillary bone are found in literature. [6],[7],[8] Thus, its occurrence in maxilla is uncommon. Here, we present a rare case of ES arising in right maxillary alveolus in an adolescent male.

 Case Report

A 17-year-old boy presented with a painless swelling involving the right midfacial region. History revealed presence of the swelling since 6 months, which gradually increased in size. On clinical examination, roughly spherical swelling of 4 cm diameter was noted involving the right malar region of face. Overlying skin was stretched, but was movable over the swelling. Intraorally, a swelling was observed involving right maxillary alveolus from canine to second molar tooth, with obliteration of adjacent gingivobuccal sulcus. Expansion on palatal aspect of alveolus was also noted. The lesion was covered by normal-appearing mucosa. Lesion was nontender with firm consistency [Figure 1].{Figure 1}

Orthopantomograph (OPG) showed increased trabecular density in the right posterior maxillary area with anterior displacement of root of second premolar and posterior displacement of first molar root. Computed tomography revealed an irregular osteolytic and enhancing soft-tissue density lesion involving right posterior maxillary alveolus, located in premolar and first molar region [Figure 2]. The lesion showed irregular borders. Multiple small radiopacities were noted within the tumor. Erosion of inferolateral wall of the maxillary sinus was evident. Anterolaterally, the lesion was extending in right buccal space [Figure 3]. Based on clinicoradiological features, provisional diagnosis of fibro-osseous lesion was made. The list of differential diagnoses included locally aggressive benign odontogenic tumors, low-grade malignant odontogenic tumors, and sinonasal malignancies arising in adolescent age.{Figure 2}{Figure 3}

After obtaining informed consent from the patient, incisional biopsy was performed under local anesthesia. On microscopic examination, the tissue specimen was found to contain sheets of small round cells infiltrating fibrocollagenous tissue. Areas with tumor cells arranged in alveolar pattern were noted [Figure 4]. The tumor cells showed round hyperchromatic nuclei and scant cytoplasm. Mitotic figures were observed. Occasional bony trabeculae were noted, but osteoid or chondroid stroma was not seen. Immunohistochemistry was performed in a stepwise manner. Epithelial markers such as CK19, CK7, p63, epithelial membrane antigen, and high molecular weight cytokeratins were negative. Among the various markers tested for round cell tumors, Mic-2, synaptophysin, and Fli-1 were positive in tumor cells. Other members of the panel, such as desmin, myogenin, and chromogranin, were negative. The tumor cells showed strong membrane reactivity for CD99 (Mic-2) [Figure 5] and nuclear expression of Fli-1, which was consistent with the diagnosis of ES/primitive neuroectodermal tumor (PNET).{Figure 4}{Figure 5}

It was necessary to exclude any evidence of metastasis. Skeletal radiographic survey was carried out, which revealed no abnormality at other skeletal sites. Right subtotal maxillectomy with adequate mucosal and osseous margins was performed. Patient underwent chemotherapy. He followed uneventful post-treatment course up to 6 months and then lost to follow-up.


ES or ES/PNET is the most common osseous malignancy observed in pediatric and adolescent age group. It was first described by James Ewing in 1921 as a "diffuse endothelioma of bone" or "endothelial myeloma." Since its first description, the tumor had been a subject of controversy regarding its histogenesis. With the versatile tools of immunohistochemistry and cytogenetics, today it is established that ES and PNET represent the histomorphological variants of a malignant tumor arising from primitive neuroectodermal cells. The tumor, which is known as PNET, displays features of neuroectodermal differentiation. ES represents undifferentiated form of the same neoplasm. Both are similar in terms of expression of immunomarkers and cytogenetic abnormalities. The common change observed in karyotypes of both tumors is a balanced translocation t(11;22) (q24;q12). This common cytogenetic abnormality in these entities suggests that both the entities are same in terms of tissue of origin and pathogenesis. [5]

ES/PNET most commonly occurs during the first three decades of life, with majority of cases diagnosed during the second decade. Males are more commonly affected than females. Clinically, majority of the jaw tumors present as enlarging swelling causing facial asymmetry. The lesion may be associated with pain or it may be painless. Adjacent teeth may become loose as a result of permeative growth of the tumor causing destruction of alveolar bone. [7]

Radiologically, jaw tumors present as expansile osteolytic lesions with poorly defined borders and often with cortical destruction and permeation of surrounding soft tissues. Typical laminated periosteal reaction or onion skinning is seen only in tumors arising in bones of extremities. It is not a peculiar feature of gnathic ES/PNET. [5]

Histologically, typical ES/PNET shows marked cellularity with very little stroma. Tumor cells may be arranged in sheets which are divided by fibrous septa. Alveolar and angiomatous arrangements may also be seen. The tumor cells look primitive. They exhibit very little cytoplasm, round to oval hyperchromatic nuclei with smooth outline and dispersed or powdery chromatin. Nucleoli are usually not prominent. Cytoplasmic glycogen can be detected in tumor cells by periodic acid Schiff (PAS) staining. However, it has little diagnostic value. Tumors with more neuroectodermal differentiation may show Homer-Wright rosettes and pseudo-rosettes.

Immunohistochemistry has become an indispensable tool for the diagnosis of ES/PNET. Intense membranous expression of CD99 (MIC-2 or p30/32 protein) is seen in almost all cases. Strong nuclear expression of Fli-1 is also very much suggestive. [9] Fli-1 appears to be integrated chimerically with EWS locus in the typical t(11;22) translocation seen in ES/PNET. [10] Co-expression of other markers of neural differentiation, such as S-100 protein, chromogranin, synaptophysin, Leu-7, or NSE, suggests neuroectodermal differentiation.

ES/PNET has been described as highly malignant and progressively destructive tumor, with propensity for rapid haematogenous metastasis and tendency for recurrence. Rarely, ES/PNET of extremities may show metastasis to jaw bones. [5] In our case, such possibility was excluded by doing skeletal survey. Majority of the cases are treated today by multimodality treatment. The tumor is radiosensitive. However, treatment with radiotherapy is usually avoided considering the complications of radiotherapy. Surgical removal of the localized tumor is recommended. Chemotherapy helps to prevent local relapse. Cytotoxic chemotherapy with multiple drug regimen (Ifosfamide + Etoposide alternating with Vincristine + Doxorubicin + Cyclophosphamide + Dactinomycin) has improved the survival rate by more than 50% for localized tumors. [11] However, prognosis of metastatic tumors is still worse. The most common metastatic sites are bones and lungs.

The primary site of the tumor is considered as the single indicator of prognosis. Other prognostic parameters are tumor stage, size, presence or absence of metastasis, and type of cytogenetic abnormality. [4] Primary jaw tumors show better prognosis than tumors arising in other sites. Among the gnathic tumors, mandibular tumors are associated with relatively favorable prognosis than maxillary ones. [5]


We describe a rare case of ES/PNET of maxillary alveolus. ES/PNET should be included in the differential diagnosis of aggressive looking gnathic lesions of adolescent age group.

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