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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 11  |  Issue : 3  |  Page : 115-119

Radiation treatment in a rare case of sinonasal ewing's sarcoma: A case report


Department of Advanced Centre for Radiation Oncology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra, India

Date of Submission30-Jun-2020
Date of Acceptance04-Jul-2020
Date of Web Publication29-Sep-2020

Correspondence Address:
Dr. Gopal Pemmaraju
Department of Advanced Centre for Radiation Oncology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jrcr.jrcr_34_20

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  Abstract 


Ewing's sarcoma of the nasal cavity and paranasal sinuses (PNS) is extremely rare and a very few cases have been reported so far.Diagnosing Ewing's sarcoma requires the use of radiological studies, histopathological examination, immunohistochemistry(IHC), and cytogenetic studies.CD 99 or Mic 2 membranous expression on Immunohistochemistry and antibody against FLI 1 are the hallmark in the diagnosis of Ewing's sarcoma.Management of sinonasal Ewing's sarcoma includes surgery,chemotherapy and radiotherapy. Radiation treatment plays an important role in the treatment of Ewing's sarcoma as an adjuvant treatment.
We are reporting a case of a 9 year old female child presented with complaints of epistaxis, nasal obstruction and swelling over right side of the face. Biopsy of the right sino nasal mass was done. Histopathologically the tumour composed of sheets of ovo-sphenoid cells.IHC showed positivity for Mic-2 and p53 protein.PET-CT scan showed an FDG avid uniformly enhancing soft tissue mass around 7.7x3.4x5.8 centimetres involving the nasopharynx, sphenoid bone,sphenoid sinus,ethmoid sinus, maxillary sinus,nasal cavity,infiltration through the optic canal into the right orbit with intracranial extension.Patient underwent Neo-adjuvant chemotherapy with Ewing's Family Tree EFT(Ewing's Family Tree)-2001 protocol and had complete response on assessment.We treated the patient with adjuvant radiation treatment to a dose of 52 Gy in 29 fractions (1.8 Gy/fraction) over 6 week to the pre-chemo tumour bed by VMAT technique..No major acute toxicities were noticed during the course of treatment. Patient needs to be assessed for local control and late toxicities.
A multidisciplinary team approach involving oncosurgery, medical oncology and radiation oncology is very crucial in attaining better outcomes.Radiation as an adjuvant treatment helps us in achieving better local control and disease free survival.Radiation techniques like VMAT,IMRT can deliver treatment with homogenous dose distribution within target and sparing organs at risk.

Keywords: Intensity-modulated radiotherapy, positron-emission tomography, volumetric modulated arc therapy


How to cite this article:
Pemmaraju G, Parab A, Singh A. Radiation treatment in a rare case of sinonasal ewing's sarcoma: A case report. J Radiat Cancer Res 2020;11:115-9

How to cite this URL:
Pemmaraju G, Parab A, Singh A. Radiation treatment in a rare case of sinonasal ewing's sarcoma: A case report. J Radiat Cancer Res [serial online] 2020 [cited 2020 Dec 5];11:115-9. Available from: https://www.journalrcr.org/text.asp?2020/11/3/115/296554




  Introduction Top


A primitive neuroectodermal tumor (PNET) is a malignant poorly differentiated tumor with both neuroectodermal and mesenchymal components. Ewing's sarcoma/PNET belongs to the family of small round cell tumors showing varying degrees of neuroectodermal differentiation. Ewing's sarcoma and PNET show similar translocations and now considered to be the part of “Ewing's family of tumours.” PNET was first described by Arthur Purdy Stout in 1918 as a tumor composed of small round cells arranged in rosettes in the tumor of the ulnar nerve.[1] James Ewing described a tumor composed of undifferentiated cells in the long bones in 1921 and hence being called by the name.

Ewing's sarcoma is the second most common malignant bone tumor after osteosarcoma and accounts for 5%–10% of the primary bone tumors.[2],[3] Ewing's sarcoma most commonly occurs in the long bones or pelvis and less frequently in the soft tissues of the trunk and extremities. Ewing's sarcoma generally arises from the diaphysis or metaphysis of long bones. Ewing's sarcoma of the head and neck accounts for 1%–4% of all the cases. Ewing's sarcoma of the nasal cavity and paranasal sinuses (PNS) is extremely rare and a very few cases have been reported so far.[4],[5] Ewing's sarcoma is seen in the age group of <20 years in about 80% of the cases with the highest incidence in the second decade of life[6] and has a slight male predominance with a male-to-female ratio 1.5:1. The diagnosis of Sinovial Ewing's sarcoma is a challenge where other small round cell neoplasms need to be excluded. Diagnosing Ewing's sarcoma requires the use of radiological studies, histopathological examination, immunohistochemistry (IHC), and cytogenetic studies. CD99 or Mic 2 membranous expression on IHC and antibody against FLI 1, which is centered in the nucleus of the tumor cells, are the hallmarks of the diagnosis of Ewing's sarcoma.[7]

Management of sinonasal Ewing's sarcoma includes surgery, chemotherapy, and radiotherapy. Although Ewing's sarcomas are radiosensitive tumors, the proportion of patients primarily treated with radiation alone declined over the past 30 years. This may be due to late effects of radiation, advances in surgery and reconstruction, and fear of second malignancies. Still, radiation treatment plays an important role in the treatment of Ewing's sarcoma as an adjuvant treatment after surgery or chemotherapy with decreased local recurrence rates and disease-free survival (DFS).


  Case Report Top


A 9-year-old female child presented with complaints of epistaxis, nasal obstruction, and swelling over the right side of the face. Biopsy of the right sinonasal mass was done. Histopathologically, the tumour was composed of sheets of ovo-sphenoidal cells and focal areas of hemopericytoma like architecture. IHC showed diffuse cytoplasmic membrane positivity for Mic-2 and few tumor cells showed a positivity to p53 protein. The tumor shows retained INI 1 protein expression and negativity for CD 31, Bcl-2, SMA, desmin, EMA, S-100 protein, CD56, synaptophysin, and AE1/AE3. MIB-1 labeling index was approximately 25%–30% with the highest proliferation areas. Differential diagnoses were (1) PNET and (2) high-grade (sarcoma like) hemangiopericytoma. In view of diffuse cytoplasmic membrane positivity for Mic-2, the possibility of PNET was favored and molecular evaluation for EWSR 1 gene re-arrangement was advised. Interphase florescent in-situ hybridization (FISH) test revealed positivity for EWSR 1 rearrangement and was consistent with Ewing's sarcoma/PNET.

Positron-emission tomography–computed tomography (PET-CT) scan showed a fluorodeoxyglucose avid uniformly enhancing soft-tissue mass in the nasopharynx, and superiorly, there was breach of the sphenoid bone, with infiltration into the sphenoid sinus with intracranial extension. There was an infiltration of the right ethmoid sinus, infiltration through the optic canal into the extraconal space into the right orbit. Inferiorly it extends into the right nasal cavity with bony breach of the medial wall of the right maxillary sinus with a small component infiltrating the maxillary antrum and abutting the posterior choana [Figure 1] and [Figure 2]. The lesion was around 7.7 cm × 3.4 cm × 5.8 cm in size with maximum standard uptake value of 4.1. Due to the large size and extension, surgical resection was not an option. The patient underwent neoadjuvant chemotherapy with EFT-2001 protocol with drugs.
Figure 1: Computed tomography scan (axial view) showing the extension of the lesion at diagnosis

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Figure 2: Computed tomography scan (sagittal view) showing the extension of the lesion at diagnosis

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Vincristine, Adriamycin, cyclophosphamide, ifosfamide, etoposide, and dactinomycin were administered over 28 weeks. Assessment of PET-CT scan revealed complete response to chemotherapy as per the RECIST 1.1. We treated the patient with adjuvant radiation treatment to a dose of 52 Gy in 29 fractions (1.8 Gy/fraction) over 6 weeks on Halcyon 2.0 with kV cone-beam CT (CBCT) linear accelerator from M/s. Varian, USA, using 6 MV Flattening free filter (FFF) beam after proper immobilization. The treatment planning was done on Eclipse 15.6 version, and the optimized plan was achieved where 95% of the dose was covering 97.5% of the target volume [Figure 3] and [Figure 4] and less dose was given to organs at risk (OAR) [Table 1]. The patient underwent daily CBCT with kV imaging, and postural variations were checked and highly precise radiation treatment was delivered to the patient. No major acute toxicity was noticed during the course of treatment. The patient needs to be assessed for local control and late toxicities.
Figure 3: Radiation planning computed tomography scan (axial) showing dose distribution to prechemo target volume

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Figure 4: Dose volume Histogram

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Table 1: Dose to organs at risk and dose coverage

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  Discussion Top


Ewing's sarcoma is an aggressive malignant tumour mostly seen in long bones of the extremeties and rarely seen in the nasal cavity and paranasal sinuses. Around 20%–30% of cases present with metastasis at diagnosis. Ewing's sarcomas of the head and neck are less prone to have metastasis.[8] Microscopically, Ewing's sarcoma/PNET is a prototype of “small round cell” tumors and composed of sheets of small cells with a high nuclear-to-cytoplasmic ratio with scanty eosinophilic cytoplasm and contains glycogen. IHC studies have shown CD 99, Mic 2, FLI 1, and neuron-specific enolase positivity in Ewing's sarcomas. IHC helps to differentiate Ewing's sarcoma from other small round cell tumors like non-Hodgkin lymphoma, neuroblastoma, rhabdomyosarcoma, mesenchymal chondrosarcoma, retinoblastoma, and desmoplastic small round cell tumors. Molecular genetics with FISH and polymerase chain reaction provide better tools for confirmation of Ewing's sarcoma. Translocation t (11;22)(q24;q12) is seen in around 85% of the cases. There is a fusion of EWS gene on 22q24 with FLI 1 gene on 11q24 resulting in a chimeric fusion EWS-FLI 1. Translocation t (21;12)(22;12) resulting in EWS-ERG fusion is also seen in 10%–15% of the cases.

Sinonasal Ewing's sarcoma patients generally present with nasal obstruction, rhinorrhea, epistaxis, and facial swelling. Radiological and radionuclear imaging, histopathology, IHC, and cytogenetics guide us to diagnose and have some prognostic value. The primary site of the tumor, size, age, and response to chemotherapy are some of the key prognostic factors. Young age, the head and neck as the primary site, and nonmetastatic at the time of diagnosis are some of the factors associated with a good prognosis. The presence of chimeric transcripts in bone marrow even in metastatic Ewing's sarcoma cases has been found to have a poor prognosis.[9]

The treatment of Ewing's sarcoma includes surgery, chemotherapy, and radiation therapy. Radical surgical removal of the tumor with negative margins and proper reconstruction is preferred. Complete resection may not be possible in large and inaccessible tumors. Neoadjuvant chemotherapy reduces the size of the tumor and clears micrometastasis as evident in about 80% of the cases.[10] Surgery should be followed by radiation in cases who underwent marginal resection[11] and poor histological response. Radiation has been advocated as a local control modality even in cases after complete resection or complete response to chemotherapy.[12],[13] Radiation doses in the range of 45–60 Gy have been prescribed in the treatment of Ewing's sarcoma of the sinonasal tract. With the improvement of techniques such as intensity-modulated radiotherapy (IMRT) and volumetric modulated arc therapy (VMAT), there has been a substantial decrease in toxicities and improvement in the local control. VMAT has proved to be comparable to IMRT in achieving dose homogeneity within target volume and in terms of dose to the OAR.[14]


  Conclusion Top


Sinonasal Ewing's sarcoma is very rare. Although the diagnosis is challenging, histopathology, IHC, cytogenetics, and radionuclear imaging guide us in getting accurate results. A multidisciplinary team approach involving oncosurgery, medical oncology, and radiation oncology is very crucial in attaining better outcomes. Radiation as an adjuvant treatment helps us in achieving better local control and DFS. Radiation techniques such as VMAT and IMRT can deliver treatment with homogenous dose distribution within target and sparing OAR. A highly precise treatment delivery is possible with image guidance in modern radiation treatment machines like halcyon that too within a short time and comfort to the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Stout AP. A tumour of the ulnar nerve. Proc NY Pathol 1918;12:2-12.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
    
3.
Whelan J, McTiernan A, Cooper N, Wong YK, Francis M, Vernon S, et al. Incidence and survival of malignant bone sarcomas in England 1979-2007. Int J Cancer 2012;131:E508-17.  Back to cited text no. 3
    
4.
Siegal GP, Oliver WR, Reinus WR, Gilula LA, Foulkes MA, Kissane JM, et al. Primary Ewing's sarcoma involving the bones of the head and neck. Cancer 1987;60:2829-40.  Back to cited text no. 4
    
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Allam A, El-Husseiny G, Khafaga Y, Kandil A, Gray A, Ezzat A, et al. Ewing's sarcoma of the head and neck: A retrospective analysis of 24 cases. Sarcoma 1999;3:11-5.  Back to cited text no. 5
    
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Yeshvant S, Ninan K, Bhandary S, Lakshinarayana KP, Shetty J, Makannavar J. Rare case of extra skeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Ther 2012;8:142-4.  Back to cited text no. 6
    
7.
Desai SS, Jambhekar NA. Pathology of Ewing's sarcoma/PNET: Current opinion and emerging concepts. Indian J Orthop 2010;44:363-8.  Back to cited text no. 7
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8.
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. 8
    
9.
Avigad S, Cohen IJ, Zilberstein J, Liberzon E, Goshen Y, Ash S, et al. The predictive potential of molecular detection in the non metastatic Ewing's family of tumours. Cancer 2004;10:1053-8.  Back to cited text no. 9
    
10.
Hafezi S, Seethala RR, Stelow EB, Mills SE, Leona LT, Duff EM, et al. Ewing's family of tumours of the sinonasal tract and maxillary bone. Head Neck Pathol 2011;5:8-16.  Back to cited text no. 10
    
11.
Lepera D, Volpi L, Facco C, Turri-Zanoni M, Battaglia P, Bernasconi B, Endoscopic treatment of Ewing's sarcoma of the sinonasal tract. J Craniofac Surg 2016;27:1001-6.  Back to cited text no. 11
    
12.
Gradoni P, Giordano D, Oretti G, Fantoni M, Barone A, La Cava S, et al. Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children. Auris Nasus Larynx 2011;38:480-6.  Back to cited text no. 12
    
13.
Iwamoto Y. Diagnosis and treatment of Ewing's sarcoma of the sinonasal tract. J Craniofac Surg 2016;27:1001-6.  Back to cited text no. 13
    
14.
Sankaralingam M, Glegg M, Smith S, James A, Rizwanullah M. Quantitative comparision of volumetric modulated arc therapy and intensity modulated radiotherapy plan quality in sinonasal cancer. J Med Phys 2012;37:8-13.  Back to cited text no. 14
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