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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 142-144

Rare case of extraskeletal Ewings sarcoma of the sinonasal tract


1 Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, India
2 Department of Radiology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, India
3 Department of ENT, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore, India

Date of Web Publication19-Apr-2012

Correspondence Address:
Sunil Kumar Yeshvanth
Associate Professor of Pathology, Department of Pathology, K S Hegde Medical Academy, Deralakatte, Mangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.95197

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 > Abstract 

Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.

Keywords: Ewings sarcoma, extraskeletal, sinonasal tract, small round cell tumor


How to cite this article:
Yeshvanth SK, Ninan K, Bhandary SK, Lakshinarayana KH, Shetty JK, Makannavar JH. Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Can Res Ther 2012;8:142-4

How to cite this URL:
Yeshvanth SK, Ninan K, Bhandary SK, Lakshinarayana KH, Shetty JK, Makannavar JH. Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Can Res Ther [serial online] 2012 [cited 2020 Jul 9];8:142-4. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/142/95197


 > Introduction Top


Ewings sarcoma (ES) is a highly malignant small round cell tumor having both skeletal and extraskeletal forms. [1],[2],[3] The skeletal forms are more frequent and occur in long bones of the extremities. The extraskeletal form usually occur in the soft tissue of lower extremities, paravertebral tissues, chest wall, retroperitonium and rarely in the head and neck region. [4],[5] Extraskeletal ES occurring in the sinonasal tract is very rare and only few case reports have been published in world literature. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy combined with surgery and radiotherapy. Although small round cell tumors of the sinonasal tract pose significant diagnostic challenges, majority can be diagnosed by light microscopy. However, some cases need ancillary methods like immunohistochemistry and molecular genetics for definitive diagnosis. Therefore, it is important to know the morphological features of ES, which help in accurate diagnosis and appropriate management of the patient.


 > Case Report Top


A 29-year-old female came with right nasal obstruction and intermittent episodes of epistaxis for one year. Nasal examination revealed a fleshy pedunculated mass in the right middle meatus. Computerized tomography (CT) revealed soft tissue density lesion in the right nasal cavity, maxillary, frontal, ethmoidal and bilateral sphenoid sinuses with bony erosions of medial and posterior wall of the right maxillary sinus [Figure 1]a. Hematological and biochemical investigations were within normal limits. Polypectomy was performed and histopathology revealed respiratory epithelium with subepithelial diffuse proliferation of small round cells having round nuclei, fine chromatin, scanty clear or eosinophilic cytoplasm and indistinct cytoplasmic borders [Figure 1]b and c. Periodic acid Schiff (PAS) stain showed strong intracytoplasmic block positivity [Figure 1]d. Immunohistochemistry was positive for CD99 [Figure 2] and FLI1; however, neuron specific enolase (NSE), cytokeratin, HMB 45, desmin, CD45, myo D1 and chromogranin were negative. Hence, final diagnosis of extraskeletal sinonasal ES was made. The patient was treated with 14 cycles of chemotherapy (vincristine, adriamycin, cyclophosphamide and dexamethasone), combined with surgery (endoscopic excision) and radiotherapy. So, on one-year follow up, patient is symptomatically better with complete recovery.
Figure 1: (a) Computerized tomography-coronal view showing heterogeneously enhancing soft tissue lesion involving the right maxillary, ethmoidal, frontal and right nasal cavity with destruction of medial wall of right maxillary sinus. (b) Microphotograph showing psuedostratifi ed columnar ciliated epithelium, diffuse proliferation of small round cells in the stroma with areas of hemorrhage. (c) Higher magnifi cation showing small round nuclei, fi ne chromatin, scanty clear or eosinophilic cytoplasm and indistinct cytoplasmic borders. (d) Periodic acid Schiff stain showing strong intracytoplasmic positivity.

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Figure 2: Immunopositivity for CD99 (x 10X)

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 > Discussion Top


Ewings sarcoma / primitive neuroectodermal tumor (PNET) are closely related family of small round cell sarcomas with varying degrees of neuroectodermal differentiation. This was first described by James Ewing, an American pathologist in 1921. [2] PNETs show neuroectodermal differentiation, whereas ES lack them as assessed by light microscopy, immunohistochemistry, and electron microscopy. Nearly 80% of patients are younger than 20 years with peak incidence in the second decade. There is male preponderance with bone being the primary site in 60% of cases. [1],[2] Majority of the patients have t(11;22)(q24;q12), i.e, fusion between the 5' end of the EWS gene from chromosome band 22q12 with the 3' portion of the 11q24 FLI1 gene, a member of the ETS family of transcription factors. This EWS/ETS fusion protein blocks the differentiation of pluripotent marrow stromal cells. Rest 10-15% of the cases have t(21;22)(q22;q12) fusing EWS to a closely related ETS gene, ERG from chromosome band 21q22. In less than 1% of cases, t(7;22), t(17;22), t(2;22) and inv(22) have been found that give rise to fusions between EWS and the ETS genes like ETV1, E1AF, FEV, and ZSG, respectively. Mutations associated with P53 or P16/p14 ARF have high aggressive behavior and poor chemotherapeutic response. [5] In our case, based on the histological diagnosis with immunohistochemistry confirmation, the patient was treated with chemotherapy, combined with surgery and radiotherapy. The patient symptomatically improved and with one year of follow up, there is no recurrence or metastasis. Although request was made for cytogenetics, the patient was not willing for it. Microscopically, these tumors show uniform small round cells with round nuclei containing fine chromatin, scanty clear or eosinophilic cytoplasm with indistinct cytoplasmic borders containing PAS positive intracytoplasmic glycogen granules. The differential diagnosis in the sinonasal tract include wide array of small round cell tumors like olfactory neuroblastoma, undifferentiated carcinoma, sinonasal melanoma, embryonal rhabdomyosarcoma, lymphoma, acute leukemia, mesenchymal chondrosarcoma, small cell osteosarcoma and small cell neuroendocrine carcinoma. [1],[3],[6] Olfactory neuroblastoma is confined to cribriform plate, superior turbinate, and superior one third of the nasal septum having uniform small round cells arranged in nests and sheets having sparse cytoplasm, round nuclei, punctuate chromatin with inconspicuous nucleoli against the background of neurofibrillary material. Homer wright rosettes are usually found and are NSE and synaptophysin positive. [1],[3] Hence this tumor was ruled out due to its location, presence of PAS positive intracytoplasmic granules, strong CD 99 positivity, negativity for NSE, absence of rosettes and neurofibrillary material. Undifferentiated carcinoma has medium-sized to polygonal tumor cells, with large ovoid nuclei with prominent nucleoli and distinct cytoplasmic borders with individual and comedo type of necrosis. Immunohistochemically, they show positivity for cytokeratin and are CD99 negative. Sinonasal melanoma was ruled out due to absence of prominent eosinophilic nucleoli, absence of melanin pigmentation and negativity for HMB45. Embryonal rhabdomyosarcoma are common in children and microscopically show small round cells with small nucleoli distributed in the subepithelial layer (cambium layer) with presence of rhabdomyoblasts (strap cells) and immunoreactivity for Myo D1. Lymphomas especially lymphoblastic type can be confused with ES and it was ruled out by absence of single prominent nucleoli and CD45 negativity. Leukemic infiltration was ruled out since there were no blasts in the marrow and tumor cells showed CD34 negativity. Mesenchymal chondrosarcoma and small cell osteosarcoma were ruled out due to absence of either cartilage or osteoid tissue and most importantly FLI1 immunopositivity. Although, CD99 is not specific for ES/PNET, combination with other markers like FLI1, HNK1 and CAV1 gives more accurate diagnosis and helps to avoid erroneous diagnosis. [6] The effective treatment plan for ES includes combined surgical excision and modern chemotherapy/ radiotherapy which has increased the five-year survival rate from 20% to 58%. [1],[6] Chemotherapy reduces the tumor size and clear micrometastasis which is present in 80% of cases. [5],[6] European intergroup co-operative ES study protocol recommends 14 cycles of etoposide, vincristine, actinomycin D, ifosfamide and adriamycin. [1],[6] According to this protocol, chemotherapy is repeated every three weeks (1 cycle) and in each cycle either adriamycin or actinomycin D is used alternatively. In case of insufficient bone marrow recovery (white blood cell count <2.0 ×10 9 /l or platelets <80 × 10 9 /l), the next cycle of chemotherapy is postponed and granulocyte-colony stimulating factor (G-CSF) was added to subsequent cycles. Chemotherapy was interrupted for surgery which includes wide excision of the tumor after four to six cycles followed by radiotherapy. If histological examination of a radically resected tumor revealed more than 10% of viable tumor cells, radiotherapy was also administered postoperatively. [2] Responses to chemotherapy were defined as progressive disease (any new lesion or increase in tumor size), stable disease (<50% decrease in tumor size), partial response (>50% tumor size reduction) and complete response (no viable tumor cells in resected specimen). [2] Important prognostic factors include age of the patient, stage, anatomic location and size of the tumor. Patients younger than 15, with axial and sinonasal tract disease, have a better outcome. The five-year survival is 55% without metastases and it reduces to 22% with it. [1] Pediatric studies have also reported a better prognosis for patients with lung metastases as compared with those of bone metastases. [2] Progressive or recurrent disease has a poor outcome, despite aggressive therapy including high dose chemotherapy and peripheral stem cell transplantation. [2]

We hereby conclude that although wide spectrum of sinonasal small round cell tumors cause significant diagnostic challenges, careful evaluation and sound knowledge of light microscopic features with the help of ancillary techniques like immunohistochemistry and cytogenetics will help for accurate diagnosis and appropriate management of the patient. [3],[6]


 > Acknowledgement Top


Dr. Padmapriya. Associate Professor of Pathology, KMC Manipal.

Dr. Harish S Permi. Associate Professor of Pathology, Kshema.

 
 > References Top

1.Iriz A, Albayrak L, Eryilmaz A. Extraskeletal primary Ewing's sarcoma of the nasal cavity. Int J Pediatr Otorhinolaryngol 2007;2:194-7.   Back to cited text no. 1
    
2.Smorenburg CH, van Groeningen CJ, Meijer OW, Visser M, Boven E. Ewing's sarcoma and primitive neuroectodermal tumour in adults: Single-centre experience in The Netherlands. Neth J Med 2007;65:132-6.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Iezzoni JC, Mills SE. Undifferentiated small round cell tumors of the sinonasal tract: Differential diagnosis update. Am J Clin Pathol 2005;124:110-21.  Back to cited text no. 3
    
4.Klein EA, Anzil AP, Mezzacappa P, Borderon M, Ho V. Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma. Cancer 1992;70:423-31.  Back to cited text no. 4
[PUBMED]    
5.Gupta S, Gupta OP, Mehrotra S, Mehrotra D. Ewing sarcoma of the maxilla: A rare presentation. Quintessence Int 2009;40:135-40.  Back to cited text no. 5
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6.Hafezi S, Seethala RR, Stelow EB, Mills SE, Leong IT, MacDuff E, et al. Ewing's family of tumors of the sinonasal tract and maxillary bone. Head Neck Pathol 2011;5:8-16.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2]


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