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Table of Contents
CLINICOPATHOLOGICAL PEARLS
Year : 2019  |  Volume : 2  |  Issue : 3  |  Page : 179-181

Ewing's sarcoma of the sinonasal tract: A histopathologically challenging case


1 Department of Otolaryngology-Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia
2 College of Medicine and Surgery, King Saud University, Riyadh, Saudi Arabia
3 Department of Histopathology, King Fahad Medical City, Riyadh, Saudi Arabia
4 Department of Otolaryngology-Head and Neck Surgery, College of Medicine; College of Medicine and Surgery, King Saud University, Riyadh, Saudi Arabia

Date of Web Publication1-Jul-2019

Correspondence Address:
Saad Abdulrazaq Alsaleh
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, King Saud University, Riyadh 13784
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JNSM.JNSM_82_18

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How to cite this article:
Alim BM, Alsaleh AS, Fatani HA, Alsaleh SA. Ewing's sarcoma of the sinonasal tract: A histopathologically challenging case. J Nat Sci Med 2019;2:179-81

How to cite this URL:
Alim BM, Alsaleh AS, Fatani HA, Alsaleh SA. Ewing's sarcoma of the sinonasal tract: A histopathologically challenging case. J Nat Sci Med [serial online] 2019 [cited 2019 Sep 15];2:179-81. Available from: http://www.jnsmonline.org/text.asp?2019/2/3/179/261952




  Introduction Top


Ewing's sarcoma (ES) is an aggressive yet rare small-blue-round cell malignant tumor that typically affects the long bones of the limbs of a young age group. It has a common skeletal form and a relatively rare extra-skeletal one. It belongs to ES family of tumors (ESFT), which seldom arises in the head-and-neck region and constitutes of about 1%–7% of ESFT cases. Nasal and paranasal sinuses involvement is even rarer with only a few reports published.[1],[2],[3],[4] Diagnosis and management of ES can be challenging, as it has to be differentiated from other tumors that share the features of undifferentiated small round cells such as nonkeratinizing carcinoma, small cell carcinoma, sinonasal undifferentiated carcinoma (SNUC), and so forth.[5] We have presented a case of sinonasal ES in a 62-year-old male who presented with left nasal obstruction and recurrent epistaxis.


  Case Presentation Top


The 62-year-old man who is known to have liver cirrhosis, portal hypertension, and esophageal varices presented himself to the Rhinology clinic at King Saud University Medical City, complaining of left-sided nasal obstruction, left epiphora, and diffuse recurrent left nasal bleeding with no significant similar symptoms on the right side for 2 years. He had two trials of endonasal excision in other countries, which have failed due to excessive bleeding, and a pathological diagnosis could not be established. Nasal endoscopy on the left side showed a large vascular lobular mass originating from the lateral nasal wall with a normal examination on the right side. Orbital examination and cranial nerves were grossly normal. Contrasted computed tomography (CT scan) and magnetic resonance imaging (MRI) of the head and neck showed a large, heterogeneous, and expansile left nasal mass extending to ethmoid sinuses and measuring 1.5 cm × 5 cm × 4 cm in the transverse, anteroposterior, and craniocaudal dimensions, respectively [Figure 1]. Numerous internal linear flow voids noted on infused T1 MRI have indicated a predominantly vascular structure, likely a hemangiopericytoma.
Figure 1: Pre- and post-operative images of a left nasal Ewing's sarcoma. (a) Pre-operative T2 magnetic resonance imaging scan. (b) Pre-operative angiogram showing the internal carotid supply of the neoplasm through the ethmoid vessels. (c) Pre-operative coronal computed tomography scan. (d) Post-operative coronal T2 magnetic resonance imaging scan

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The patient was consented for endoscopic endo-nasal removal of the mass 24–48 h after the embolization of the left sphenopalatine artery (SPA) to lessen the possibility of excessive bleeding due to the vascularity of the tumor and abnormal coagulation secondary to his hepatic disease. Embolization of the left SPA was uncomplicated, and the surgery was scheduled 24 h later. The surgery started with a left lynch incision and endoscopic-assisted ligation of the anterior and posterior ethmoidal arteries to further control all feeding vessels. This was followed by endoscopic excision of the mass, which was delivered en bloc. In addition, it appeared that the tumor existed in the left, middle, and superior turbinates and the ethmoid cavity. To further decrease intraoperative bleeding, coblation technology was used in certain areas to remove the neoplasm. Blood loss was estimated to be <500 ml and the perioperative course was uncomplicated.

What is the histopathological diagnosis?

The initial histopathological review of the specimen was consistent with a SNUC with negative margins. As the clinical course and appearance of the neoplasm were not consistent with a SNUC, the pathology slides were reviewed in another tertiary center, which labeled the mass as a poorly differentiated malignant neoplasm with features suggestive of ES [Figure 2]. The biopsy was sent for immune-histochemical staining and was positive for vimentin, synaptophysin, and CD99. It was negative for S100, CK20, and SBV. The fluorescence in situ hybridization analysis showed EWSR1 gene rearrangement on chromosome 18q11.2 in 100% scored nuclei. Whole body positron emission tomography-computed tomography (PET) scan and MRI of the head and neck were done, revealing spinal metastasis with no residual disease in the original tumor bed. The case was discussed in the tumor board, and a comprehensive decision was made to start the patient on chemotherapy. However, due to the poor general medical condition of the patient, he was unfit for chemotherapy; thus, he was provided with radiotherapy 50 grays\20 fractions to the primary mass and 20 grays\5 fractions to the spinal metastasis.
Figure 2: Histopathology slides of the specimen. (a) CD-99 shows diffuse membranous positivity. (b) Sheets of small, round, and uniform cells with scant and clear cytoplasm

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Follow-up and outcome

The patient was followed up in the Rhinology clinic monthly in the 1st postoperative year and every 3–4 months in the 2nd year. He continued with the Oncology service as well. Endoscopic examination and follow-up imaging revealed no recurrence of the neoplasm to date with only postradiotherapy findings and synechiae. The patient was kept on regular nasal normal saline rinses. A follow-up PET\CT scan showed the partial response of the spinal metastatic lesions to the radiotherapy, for which, the patient is regularly checking up with the radiation oncology team.


  Discussion Top


ES is a rare primary bone tumor that commonly affects the long bones in 35%, pelvis in 24%, chest wall in 20%, and head-and-neck region commonly the maxilla and mandible in only 1%–4% of the population.[6] Microscopically, ES is described as a primitive neuroectodermal tumor, as it shows a cellular pattern of small rounded cells with rounded nuclei surrounded by a scanty eosinophilic cytoplasm.[7] As seen in the above case, the histopathological diagnosis was not straightforward initially. The challenge in the histological diagnosis of ES lies in its similarities with other small round cell tumors as mentioned in the introduction, and a second opinion with regard to histopathology adds a great benefit in starting the appropriate treatment protocol. Kronz et al. found that diagnostic discrepancies in the surgical pathology of the head-and-neck area range from 1%–53% to 17%–60%, respectively, for cytopathology studies that stress on the importance of the second opinion, especially when dealing with uncommon pathologies.[8] In another case, among five adult patients with primary sinonasal ES, two patients had the original diagnoses changed by a second review of the histopathology slides and one of them was originally diagnosed with SNUC similar to what happened in our case.[9]

Immune-histochemical staining aids tremendously in such pathologies. For instance, positivity toward CD99 and vimentin strongly indicate ES, whereas positivity toward vimentin and desmin strongly indicate rhabdomyosarcoma.[4] The majority (80%) of ES is also characterized by gene translocation. That indicates that 5' end portion of the ES gene from chromosome band of 22q12 fuses with 3' end portion of friend leukemia virus integration gene located on chromosome 11q24. The combination of histopathological, immune-histochemical and molecular findings guides the best toward ES diagnosis.[4] However, in our case, the diagnosis was based mainly on immune-histochemical and histological findings.

Treatment of such an uncommon pathology in such a rare location requires a multidisciplinary approach and a thorough discussion in the tumor board. ES is both chemo and radiosensitive.[10],[11] Literature shows a heterogeneity in the treatment of ES among different authors. According to the CESS-86 protocol, neoadjuvant chemotherapy followed by surgery and radiotherapy or chemotherapy is the suggested plan.[12] Other authors suggested chemotherapy to be used as a definitive treatment and in un-resectable tumors with palliative intent.[13] In the adult population, mainly, and in the pediatric population, to some extent, endoscopic endonasal resection of the mass has been associated with the quality of life outcomes and cure rates similar to that achieved with external approaches in the past, and the primary goals of a free margin resection plus minimal postoperative morbidity can be well achieved with endoscopic approach in a properly selected patient and in expert hands.[1],[14] In Whaley et al.'s work on ES treatment in the head-and-neck region, the 5-year-survival rate increases from 20% to 58% when following a multi-disciplinary approach of surgical resection followed by radiotherapy and chemotherapy.[15] Nevertheless, the recurrence either early or late has been seen in about one-third of patients in another review, which can be understood by the nature of the disease itself.[16] In the present case, the tumor board's decision was in accordance with the available literature, yet the patient's medical condition could not allow him to receive chemotherapy. Preoperative embolization is an invaluable part that helps en bloc resection of such vascular masses,[17] and in our case, preoperative embolization plus anterior and posterior ethmoidal artery ligation and coblation has helped in the complete resection of the lesion and in ensuring negative margins.

Clinico-pathological pearls

  1. Comprehensive knowledge of histopathological, immune-histochemical, and molecular biology is vital to guide the clinician to the correct diagnosis of rare pathologies such as ES
  2. Due to the rarity of cases and unavailability of evidence-based statements, management of ES must be individualized to each case
  3. Multidisciplinary team discussions, second opinion of an expert pathologist and performing a full immuno-histochemical panel must be considered
  4. Referral to highly experienced centers should not be delayed.


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.
Lepera D, Volpi L, Facco C, Turri-Zanoni M, Battaglia P, Bernasconi B, et al. Endoscopic treatment of Ewing sarcoma of the sinonasal tract. J Craniofac Surg 2016;27:1001-6.  Back to cited text no. 1
    
2.
Alexiev BA, Tumer Y, Bishop JA. Sinonasal adamantinoma-like Ewing sarcoma: A case report. Pathol Res Pract 2017;213:422-6.  Back to cited text no. 2
    
3.
Woo CG, Lee B, Song JS, Cho KJ. Clinicopathologic features of the non-CNS primary Ewing sarcoma family of tumors in the head and neck region. Appl Immunohistochem Mol Morphol 2018;26:632-9.  Back to cited text no. 3
    
4.
Lin JK, Liang J. Sinonasal ewing sarcoma: A case report and literature review. Perm J 2018;22:17-86.  Back to cited text no. 4
    
5.
Woo CG, Lee B, Song JS, Cho KJ. Clinicopathologic features of the non-CNS primary Ewing sarcoma family of tumors in the head and neck region. Appl Immunohistochem Mol Morphol 2018;9:632-9.  Back to cited text no. 5
    
6.
Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, et al. Prognostic factors in Ewing's tumor of bone: Analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. J Clin Oncol 2000;18:3108-14.  Back to cited text no. 6
    
7.
Yeshvanth SK, Ninan K, Bhandary SK, Lakshinarayana KP, Shetty JK, Makannavar JH, et al. Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Ther 2012;8:142-4.  Back to cited text no. 7
    
8.
Kronz JD, Westra WH. The role of second opinion pathology in the management of lesions of the head and neck. Curr Opin Otolaryngol Head Neck Surg 2005;13:81-4.  Back to cited text no. 8
    
9.
Lombardi D, Mattavelli D, Redaelli De Zinis LO, Accorona R, Morassi ML, Facchetti F, et al. Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease. Head Neck 2017;39:E45-E50.  Back to cited text no. 9
    
10.
Owens C, Abbott LS, Gupta AA. Optimal management of ewing sarcoma family of tumors: Recent developments in systemic therapy. Paediatr Drugs 2013;15:473-92.  Back to cited text no. 10
    
11.
La TH, Meyers PA, Wexler LH, Alektiar KM, Healey JH, Laquaglia MP, et al. Radiation therapy for Ewing's sarcoma: Results from memorial sloan-kettering in the modern era. Int J Radiat Oncol Biol Phys 2006;64:544-50.  Back to cited text no. 11
    
12.
Paulussen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, et al. Localized Ewing tumor of bone: Final results of the cooperative Ewing's sarcoma study CESS 86. J Clin Oncol 2001;19:1818-29.  Back to cited text no. 12
    
13.
Coskun BU, Cinar U, Savk H, Basak T, Dadas B. Isolated maxillary sinus Ewing's sarcoma. Rhinology 2005;43:225-8.  Back to cited text no. 13
    
14.
Castelnuovo P, Lepera D, Turri-Zanoni M, Battaglia P, Bolzoni Villaret A, Bignami M, et al. Quality of life following endoscopic endonasal resection of anterior skull base cancers. J Neurosurg 2013;119:1401-9.  Back to cited text no. 14
    
15.
Whaley JT, Indelicato DJ, Morris CG, Hinerman RW, Amdur RJ, Mendenhall WM, et al. Ewing tumors of the head and neck. Am J Clin Oncol 2010;33:321-6.  Back to cited text no. 15
    
16.
Ahmed AA, Zia H, Wagner L. Therapy resistance mechanisms in Ewing's sarcoma family tumors. Cancer Chemother Pharmacol 2014;73:657-63.  Back to cited text no. 16
    
17.
Satish D, Nanadakumar R, Balasubramanya AM, Mathew N. A rare case of Ewing's sarcoma in the sinonasal tract. Int J Otorhinolaryngol Head Neck Surg 2018;1:304-7.  Back to cited text no. 17
    


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  [Figure 1], [Figure 2]



 

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