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Year : 2000  |  Volume : 10  |  Issue : 4  |  Page : 227-228
Case report: Extraskeletal Ewing's sarcoma of the parotid gland

1 Dept of Radiology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India
2 Dept of Radiotherapy, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India
3 Dept of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012, India

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Keywords: Ewing′s sarcoma, extraskeletal sites, parotid

How to cite this article:
Sandhu M S, Kalra N, Singh D P, Radotra B D, Jain M, Suri S. Case report: Extraskeletal Ewing's sarcoma of the parotid gland. Indian J Radiol Imaging 2000;10:227-8

How to cite this URL:
Sandhu M S, Kalra N, Singh D P, Radotra B D, Jain M, Suri S. Case report: Extraskeletal Ewing's sarcoma of the parotid gland. Indian J Radiol Imaging [serial online] 2000 [cited 2021 Feb 26];10:227-8. Available from:
Extraskeletal Ewing's sarcomas are uncommon soft tissue neoplasms and are rare in the head and neck region. Non-lymphoid soft tissue sarcomas of the major salivary glands are very rare. Many investigator believe that, except for the benign hemangioma in children, most tumors of supporting tissue in the salivary gland regions, although simulating a parotid mass, involve the gland as an extension from a neoplasm form surrounding soft tissues [1],[2]. An extraskeletal, intraparotid Ewing's sarcoma has never been reported before. We report one such case and briefly review ,extraskeletal Ewing's sarcoma.

   Case Report Top

A sixteen-year-old girl presented with a two-month history of the painless swelling in the region of the right parotid gland. It was firm and fixed to the overlying skin in the right preauricular region. There was right facial nerve palsy. No cervical lymph node enlargement was noted. The otorhinolaryngologic examination revealed no other abnormality. A contrast-enhanced CT scan of the region was done. This revealed a well-defined low-density lesion with enhancing margins adjacent to the mandibular condyle replacing the superficial lobe of the right parotid gland [Figure - 1]. There was no calcific focus or evidence of osseous involvement. No lymph nodes were enlarged. CT scan of the chest and ultrasound of the abdomen were abnormal.

As the fine needle cytology investigation was inconclusive and an excision biopsy of the right parotid gland was performed through an extra-oral, periauricular approach. The mass was covered with fibrous capsule. Microscopic examination revealed that large areas of the parotid gland were replaced by numerous round cells. This was suggestive of Ewing's sarcoma [Figure - 2]. Foci of residual acinar tissue were also present.

The patient was started on combination chemotherapy. She also received local radiation therapy to the right parotid region. She subsequently had no evidence of disease at that point of time, and was advised regular follow up. In the fourth year of the follow up, the patient has had recurrence of the disease in the form of nodular swellings in the region of the lateral canthus of the right eye and submandibular region, which have been managed by local radiation and chemotherapy.

   Discussion Top

Sarcomas comprise only 0.6% of all cancers throughout the body and account for only 1.5% of all salivary gland tumors [3]. The existence of these tumors has been debatable with some investigators stating that such lesions represent extensions of soft tissue sarcomas into the gland. In young patients, these tumors occur more often than the more common parenchymal tumors or the conventional soft tissue sarcomas.

Extraskeletal Ewing's sarcoma was first reported by Teff et al in 1969 [4] and the pathological features were first reported by Angervall et al in 1975 [5]. There is no sex predilection for this condition and patients are older; unlike the osseous form where the median age of occurrence is about 20 years. Extraskeletal Ewing's sarcoma has been described in almost all sites where every other sarcoma is found. Distant metastases to the lung are usually found.

To our knowledge, our patient is the first one to present with the parotid gland being the site for the extraskeletal Ewing's sarcoma. Radiological findings of this condition, involving other sites, have been described earlier.

Conventional radiographs are usually normal. A non-specific soft tissue mass without tissue calcification may be seen. When adjacent bone changes are present, histology differentiates it from the osseous form. CT, US and MRI show a well-circumscribed lesion. The mass lesion appears hypoechoic on US and is of low attenuation on CT scan. These are non-specific findings seen with most soft tissue sarcomas [6], and reflect the microscopic findings of necrosis. Variable and moderate post contrast enhancement is seen on CT scan[7]. Spontaneous tumor hemorrhage may occur and may alter the appearances with the above imaging modalities.

The treatment of choice is multimodal, just as it is when Ewing's and extraskeletal Ewing's sarcoma strike other sites. Once the diagnosis is established by way of biopsy, surgery followed by chemotherapy and radiotherapy may offer the best possible aggressive combination. Survival rates with extraskeletal Ewing's sarcoma are still poor and both local recurrences and distant metastases develop frequently. Despite the appreciable rarity of intraparotid extraskeletal Ewing's sarcoma, it is advisable to consider this tumor in the differential diagnosis of a non-calcified soft tissue mass in this region of the body, for the appropriate age group.

   References Top

1.Work WP, Batsakis JG. Classification of salivary gland disease Otolaryngologic Clinics of North America 1977; 10: 287-296.   Back to cited text no. 1    
2.Batsakis JG. Tumors of the head and neck; clinical and pathological consideration. 2 ndsub ed. Baltimore: Williams and Wilkins, 1979; 10.  Back to cited text no. 2    
3.Auclair PL, Langloss JM, Weiss WS et al. Sarcomas and sarcomatoid neoplasms of the major salivary gland regions: a clinicopathologic and immunohistochemical study of 67 cases and review of literature. Cancer 1986; 58:1305-1315.  Back to cited text no. 3    
4.Tefft M, Vawter GF, Mitus A. Paravertebral round cell tumors in children. Radiology 1969; 92:1501-1509.  Back to cited text no. 4    
5.Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing sarcoma. Cancer 1975; 36: 240-251.  Back to cited text no. 5    
6.Golding SJ, Husband JE. The role of computed tomography in the management of soft tissue sarcomas. BJR 1982; 55:740-747.  Back to cited text no. 6    
7.0' Keeffe F, Lorigan JG, Wallace S. Radiological features of extraskeletal Ewing sarcoma. BJR 1990; 63: 456-460.  Back to cited text no. 7    

Correspondence Address:
M S Sandhu
Dept of Radiodiagnosis, PGIMER, Chandigarh 160 012
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2]

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