|Year : 2015 | Volume
| Issue : 1 | Page : 15-17
Ewing's sarcoma arising from base of nasal cavity and extending to nasopharynx and oral cavity
Ercan Akbay1, Mehmet Yaldiz2, Cengiz Cevik1, Mehmet Ates1, Tumay Ozgur2
1 Department of Otorhinolaryngology, Faculty of Medical, Mustafa Kemal University, Hatay, Turkey
2 Department of Pathology, Faculty of Medical, Mustafa Kemal University, Hatay, Turkey
|Date of Web Publication||29-Nov-2014|
Department of Otorhinolaryngology, Faculty of Medical, Mustafa Kemal University, Hatay
Source of Support: None, Conflict of Interest: None
Ewing's sarcoma (ES) is a malignant, small cell tumor of skeletal system seen in young adults and children. The prognosis is very poor in this type of tumor, which has a metastasis potential at an early stage. It is rarely seen in head and neck region; mandible is involved relatively frequent while paranasal involvement is very rare. Furthermore, ES arising from base of nasal cavity and hard palate is extremely rare. The aim of this report was to discuss a 14-year-old girl with ES arising from base of nasal cavity and extending to nasopharynx in the light of literature.
Keywords: Ewing′s sarcoma , hard palate, nasal cavity, pediatric patient
|How to cite this article:|
Akbay E, Yaldiz M, Cevik C, Ates M, Ozgur T. Ewing's sarcoma arising from base of nasal cavity and extending to nasopharynx and oral cavity. Sifa Med J 2015;2:15-7
|How to cite this URL:|
Akbay E, Yaldiz M, Cevik C, Ates M, Ozgur T. Ewing's sarcoma arising from base of nasal cavity and extending to nasopharynx and oral cavity. Sifa Med J [serial online] 2015 [cited 2019 Jan 21];2:15-7. Available from: http://www.imjsu.org/text.asp?2015/2/1/15/145802
| Introduction|| |
Ewing's sarcoma (ES) is a malignant, small cell tumor of skeletal system seen in young adults and children. , It is an aggressive tumor with the potential of rapid growth and metastasis.  Also, it is frequently seen within the first three decades of life and it arises from limbs, ribs and pelvic bone. The tumor, which is rarely seen in the head and neck region, involves mandible relatively frequent, while it is very rare in the paranasal region. , Moreover, ES arising from base of nasal cavity and hard palate is extremely rare. , In this report, we will discuss a girl with ES arising from base of nasal cavity.
| Case Report|| |
A 14-year-old girl presented to our clinic with nasal congestion, facial pain and swelling in the palate. Within the past 2 months, she had a weight loss of 7 kg and loss of appetite. A well-defined and hypervascularized (in some areas) mass, which completely filled the right nasal cavity and nasopharynx, and extended to oropharynx and oral cavity through right half of hard palate, was detected on physical examination [Figure 1]a and b. On clinical evaluation, there was no invasion in nasal septum at medial and skull base at superior; however, relationship with sinuses couldn't be assessed.
|Figure 1: Mass appearance arising from base of nasal cavity at the right (a) and expanding to the right side of hard palate (b)|
Click here to view
On contrast computed tomography (CT) scan and magnetic resonance imaging (MRI), a soft tissue mass with lobulated contours was observed that extended to the nasal cavity and ethmoid sinus as well as nasopharyngeal level at posterior and caused erosion and destruction of palatine bone in addition to expansion in right maxillary ostium [Figure 2]a and b.
|Figure 2: A mass causing destruction at hard palate on sagittal CT sections (a). MR image of the mass on the same plane (b)|
Click here to view
Complete blood count, including hemoglobin, neutrophil, lymphocyte, monocyte and basophil values, biochemical studies, including glucose, blood urea nitrogen, SGOT, SGPT, K, Na and Ca, and bleeding and clotting times were found to be within the normal range.
Informed consent was obtained from the patient's guardian for biopsy and the use of patient information, including photographs and medical records for publication. Under local anesthesia, incisional biopsies were performed at hard palate and nasal cavity. On histopathological evaluation, a tumor formation was observed, which consisted of atypical cells with hyperchromatic nucleus and small cytoplasm exhibiting diffuse infiltration as well as nodular pattern in some instances [Figure 3]a. In immunohistochemical study, tumor cells expressed strong membranous positivity with CD99 [Figure 3]b and weak positivity with Vimentin, while there was negative reaction with LCA, CD20, CD3, EMA, Tdt, Myogenin, Desmin and Pancytokeratin. A nodular pattern was observed by reticulin histochemical technique. No material with cytoplasmic PAS positivity was observed with PAS histochemical method.
|Figure 3: (a) Tumor tissue in which oval or round atypical cells groups with hyperchromatic nucleus and narrow cytoplasm and trabecular structures were observed (H and E ×100). (b) Strong positive staining by CD99 (CD99 ×100)|
Click here to view
By these findings, tumor was identified as ES. We recommended surgery followed by chemotherapy; however, the parents declined surgery due to its morbidity and she was referred to chemoradiotherapy.
| Discussion|| |
ES was first described by James Ewing in 1921. , It is more common among men.  ES, a primary malignant small cell tumor of skeletal system, originates from primitive mesenchymal cells of bone marrow or immature reticulum cells, which comprises 4-10% of all malign bone neoplasm. , It can be classified into 3 types according to anatomic localization: intraosseous, extraskeletal and periosteal.  This case was the intraosseous type. This sarcoma is rarely encountered in the head and neck region or mandible.  ES presents with nasal congestion, headache, swelling in face, facial pain, paresthesia and loosening or loss of tooth when it is localized at paranasal region. The clinical findings are similar to other malignant tumors except for rapid growth. Generally, fever, lymphadenopathy, weight loss and anemia can be observed. , However, there was only weight loss, fatigue and loss of appetite in our case. The diagnosis is made by histopathology.
On histopathological evaluation, tumor tissue with oval or round atypical cell groups having hyperchromatic nucleus and narrow cytoplasm in addition to trabecular structures were observed on hematoxylin and eosin staining. In immunohistochemical evaluations, it differs from other small cell tumors by strong positive membranous staining with CD99.
In the diagnosis, neither MRI nor CT alone provides sufficient data; however, they can make significant contributions when used in combination.  The presence of osteolytic lesions accompanied by cortical erosion can be observed in osseous area on radiology. However, this finding isn't specific to ES where similar radiological findings can be observed in histiocytosis X, neuroblastoma, osteogenic sarcoma and osteomyelitis. 
The ES management usually consists of neo-adjuvant chemotherapy followed by surgical resection. , These tumors are also radiosensitive.  Thus, therapy can be supported by adjuvant radiotherapy. MRI can provide information on the assessment of tumor response.  The prognosis is poor in this tumor, as metastasis to bone, lung, liver and lymph nodes occur in a short time. However, it was reported that prognosis is better in ESs localized at cranial areas when compared to other regions.  In recent years, 5-year survival rates have increased from 16% to 74% by early diagnosis and combined therapies, including surgery, chemotherapy and radiotherapy.  Our case was referred to a medical oncology clinic as her parents declined surgical intervention.
In conclusion, ES arising from base of nasal cavity and hard palate is extremely rare. Thus, ES should be kept in mind in malignant tumors detected in young adults and children. It is important that ENT surgeons consider ES in the differential diagnosis of masses observed at nasal and maxillofacial region and they remember to perform radiological and histopathological evaluations as soon as possible since early diagnosis can have a significant effect on prognosis.
| References|| |
Brazão-Silva MT, Fernandes AV, Faria PR, Cardoso SV, Loyola AM. Ewing's sarcoma of the mandible in a young child. Braz Dent J 2010;21:74-9.
Vikas Prasad B, Ahmed Mujib BR, Bastian TS, David Tauro P. Ewing's sarcoma of the maxilla. Indian J Dent Res 2008;19:66-9.
Mukherjee A, Ray JG, Bhattacharya S, Deb T. Ewing's sarcoma of mandible: A case report and review of Indian literature. Contemp Clin Dent 2012;3:494-8.
Gupta S, Gupta OP, Mehrotra S, Mehrotra D. Ewing sarcoma of the maxilla: A rare presentation. Quintessence Int 2009;40:135-40.
Harman M, Kiroðlu F, Kösem M, Unal O. Primary Ewing's sarcoma of the paranasal sinus with intracranial extension: Imaging features. Dentomaxillofac Radiol 2003;32:343-6.
Csokonai LV, Liktor B, Arató G, Helffrich F. Ewing's sarcoma in the nasal cavity. Otolaryngol Head Neck Surg 2001;125:665-7.
Li WY, Li X, Jiang XW. Primary Ewing's sarcoma of nasal cavity: A case report. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2009;44:428-9.
Coskun BU, Cinar U, Savk H, Basak T, Dadas B. Isolated maxillary sinus Ewing's sarcoma. Rhinology 2005;43:225-8.
Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation. Radiographics 2013;33:803-31.
Wexler LH, Kacker A, Piro JD, Haddad J Jr, Close LG. Combined modality treatment of Ewing's sarcoma of the maxilla. Head Neck 2003;25:168-72.
[Figure 1], [Figure 2], [Figure 3]