Extraosseous sinonasal tract metastasis of Ewing's sarcoma presenting with sixth cranial nerve palsy

Mousumi Banerjee; Shorya Vardhan Azad; Akshra Pahuja; Arvind Kairo

doi:10.4103/IJO.IJO_2919_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 483-485

Extraosseous sinonasal tract metastasis of Ewing's sarcoma presenting with sixth cranial nerve palsy

Mousumi Banerjee¹, Shorya Vardhan Azad², Akshra Pahuja¹, Arvind Kairo³
¹ Senior Resident, Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India
² Associate Professor, Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India
³ Consultant in Otorhinolaryngology, AIIMS, New Delhi, India

Date of Submission	02-Nov-2022
Date of Acceptance	28-Nov-2022
Date of Web Publication	28-Apr-2023

Correspondence Address:
Shorya Vardhan Azad
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi - 110 029
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2919_22

Abstract

Ewing's sarcoma (ES) is a rare, aggressive, and fatal malignancy of bones and soft tissues that affect children and adolescents. It has a high potential of hematogenous metastasis, with lungs, bones, and bone marrow being the most common sites. Extraskeletal metastasis of Ewing's sarcoma in the sinonasal cavity is rare and usually presents with nasal complaints and periorbital swelling. Diplopia as the presenting complaint of sinonasal tract metastasis of Ewing sarcoma has not been reported in the literature. A high suspicion for metastasis should be kept in mind in patients presenting with atypical ophthalmic findings of Ewing sarcoma and appropriate radio imaging should be procured for early intervention.

Keywords: Diplopia, Ewing sarcoma, extra-skeletal metastasis, sixth nerve palsy

How to cite this article:
Banerjee M, Azad SV, Pahuja A, Kairo A. Extraosseous sinonasal tract metastasis of Ewing's sarcoma presenting with sixth cranial nerve palsy. Indian J Ophthalmol Case Rep 2023;3:483-5

How to cite this URL:
Banerjee M, Azad SV, Pahuja A, Kairo A. Extraosseous sinonasal tract metastasis of Ewing's sarcoma presenting with sixth cranial nerve palsy. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:483-5. Available from: https://www.ijoreports.in/text.asp?2023/3/2/483/374999

Ewing sarcoma (ES) is primarily an intramedullary malignant neoplasm of the bone that predominantly affects children and adolescents, with its peak occurrence being between 10 and 20 years of age.^[1] The most common sites of metastasis include the lungs, bones, and bone marrow.^[2] Only one reported case of extraskeletal metastasis of ES in the sinonasal cavity was found after an extensive literature search that presented with nasal obstruction and periorbital cellulitis.^[3]

Herein, we describe a rare case of extraskeletal sinonasal tract metastasis of ES presenting with atypical ophthalmic findings.

Case Report

A 21-year-old woman presented with sudden onset painless diminution of vision in the left eye (OS) for two days, along with headache and diplopia for seven days. There was no history of ocular trauma or surgical procedures. She was previously diagnosed with ES of the right pelvis two years ago and had undergone chemotherapy and local radiotherapy. She has been on maintenance therapy since the last eight months. Whole-body positron emission tomography and computed tomography (PET- CT) scan performed at initiation of maintenance therapy (eight months back) documented sclerotic changes in the right pelvic bone and healed lesions with no metabolically active lesion in the whole body, thereby negating the possibility of metastasis. There was no history of nausea, projectile vomiting, seizures, epistaxis, paresthesia, or paresis of the left side of the face prior to ocular complaints.

Systemic examination revealed a blood pressure of 110/76 mmHg with a reduced red blood cell (RBC) count of 2.70 mill/mm³ and hemoglobin of 7.9 g/dl.

On ocular examination, best-corrected visual acuity (BCVA) was 6/6 in the right eye (OD) and positive perception of light (PL) with an inaccurate projection of rays (PR) (two quadrants) in OS. Relative afferent pupillary defect (RAPD) and limitation of abduction were noted in OS. Anterior segment examination was within normal limits with an intraocular pressure (IOP) of 12 mmHg in OU. Fundus examination revealed bilateral superficial hemorrhages suggestive of anemic retinopathy with dilated, tortuous veins in OS, raising suspicion of some compressive lesion [Figure 1]a, [Figure 1]b. Optical coherence tomography (OCT) depicted a central macular thickness of 245 μm and superficial location of the retinal hemorrhages in the sub–internal limiting membrane (ILM) nerve fiber layer plane [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d Optic nerve function tests showed preserved color vision with contrast sensitivity of 1.65 on the Pelli-Robson chart in OD.

Figure 1: Ultra-wide field pseudo color imaging (Optos) of the right eye (a) and left eye (b) depicting superficial flame-shaped hemorrhages (white arrow), suggestive of anemic retinopathy and dilated tortuous vein in OS (black arrow)

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Figure 2: OCT of the left eye (a) depicting superficial location of the hemorrhages in sub ILM plane (red arrow) with a central macular thickness of 230 μm (c). Figure (b and d) represents the section of the fundus through which oct images in (a and c) were taken respectively. MRI brain and orbit axial (T1 weighted) and coronal section depicting heterogeneous enhancing mass in bilateral sphenoidal sinus (white arrow) with compression of the optic canal in the left eye with the growing mass (e and f)

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Magnetic resonance imaging (MRI) of the brain and orbit were advised with a high suspicion of a compressive lesion. Polypoidal expansile mucosal thickening with hyperintense signal intensity was noted, involving the bilateral sphenoidal sinuses and right maxillary sinus. Compression of the left optic nerve was appreciated on the axial section of MRI [Figure 2]e, [Figure 2]f.

An urgent otorhinolaryngologist referral was sought for diagnostic endoscopic biopsy performed via the spheno-ethmoidal recess approach using 0° and 30° Hopkins rod sinuscopes. Histopathological examination of the lesion revealed features of malignant small round cell tumors which were immunopositive for MIC-2 (diffuse) and EMA (weak), and negative for SATB2 (overall features consistent with Ewing Sarcoma). [Figure 3] depicts an endoscopic view of the tumor (white in color) in the sphenoidal sinus. Chemotherapy was re-initiated. We could preserve the vision in OD, but OS vision could not be salvaged.

Figure 3: Endoscopic view showing the mass lesion (white in color) in sphenoidal sinus

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Discussion

Metastatic ES within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important for early diagnosis. The common symptoms reported with sinonasal involvement of ES include nasal obstruction, epistaxis, rhinorrhea, periorbital cellulitis, decreased vision, dyschromatopsia, paresthesia, proptosis, and epiphora.^[3],[4],[5] None of these cases presented with solely ophthalmic manifestations, leading to suspicion of metastasis.

Our patient neither had a nasal complaint nor any systemic issues. Based on the presence of abduction limitation and RAPD along with fundus findings in OS, a suspicion of compressive pathology (? metastasis) was considered. The abducent nerve is the most common cranial nerve to be affected by any progressive lesion in the sphenoidal sinus due to its medial location in the cavernous sinus and close proximity to the sphenoidal sinus; this is followed by the optic nerve and oculomotor nerve.^[6] The presence of sphenoidal mucosal thickening in OS close to cranial nerve VI explained the diplopia, depicted by abduction limitation. Compression of the optic nerve and venous drainage by the enlarging metastatic tumor explained the vision loss and tortuosity of veins in OS. MRI is a better modality of evaluating soft tissue involvement and delineating tumor margins; a CT scan in our case would have missed out on the extraskeletal paranasal sinus lesions and might have delayed the diagnosis and intervention.

The Euro Ewing 2012 study protocol suggested the use of PET-CT in ES for initial staging and assessment of tumor burden before initiating chemotherapy and it be repeated after completion of induction therapy for re-assessment of response. Though it is difficult to specify an exact time interval for increased risk of disease metastasis, Leavey et al.^[7] reported most episodes of disease recurrence (80%) within two years of initial diagnosis. A PET-CT was repeated soon after the induction phase in our case, which showed no active lesion in the body. We would like to emphasize that a normal PET-CT at the end of the induction phase does not rule out the chances of metastasis at a later stage in ES due to its aggressive nature.

Causes of anemia in ES are multifactorial. Increased concentration of inflammatory cytokines, such as interleukin (IL)-6 or tumor necrosis factor (TNF) causes a “relative erythropoietin deficiency” that involves inadequate production of erythropoietin for the degree of anemia, and increased resistance of erythropoietic precursors to erythropoietin. It is further aggravated by the cytotoxic effects of the chemotherapeutic agents on the bone marrow. Our patient had initially presented with a hemoglobin of 4.6 g/dl and had received multiple blood transfusions along with chemotherapy. Concern for anemic retinopathy increases with values falling below 6 g/dl, as was in our case.^[8] Severe anemia leads to hypoxic damage to the retina, and hypoxia usually affects the superficial retinal nerve fiber layer more than the deeper retinal vasculature^[8]: this corroborates with the fundus findings in our case.

Vision loss in anemic retinopathy can occur due to hemorrhages, edema, or hard exudates at the macula. It can also occur secondary to disc edema or optic neuropathy, which is usually bilateral.^{[9],[10],[11]} However, we do not believe that anemic retinopathy contributed to the sudden onset vision loss in our case due to lack of involvement of the macula.

Conclusion

Diplopia as a presenting sign of metastasis to the paranasal sinuses has not been reported in the literature. We wish to highlight that even though orbital metastasis is rare, any ophthalmic manifestation in a case of ES should raise suspicion for metastasis in a clinician's mind. An appropriate radio imaging of the orbit should always be procured along with brain imaging.

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

1.	Jiang Y, Ludwig J, Janku F. Targeted therapies for advanced Ewing sarcoma family of tumors. Cancer Treat Rev 2015;41:391-400.
2.	Jereb B, Ong RL, Mohan M, Caparros B, Exelby P. Redefined role of radiation in combined treatment of Ewing's sarcoma. Pediatr Hematol Oncol 1986;3:111-8.
3.	Hayes SM, Jani TN, Rahman SM, Jogai S, Harries PG, Salib RJ. Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma. J Laryngol Otol 2011;125:861-4.
4.	Davido N, Rigolet A, Kerner S, Gruffaz F, Boucher Y. Case of Ewing's sarcoma misdiagnosed as a periapical lesion of maxillary incisor. J Endod 2011;37:259-64.
5.	Vaccani JP, Forte V, de Jong AL, Taylor G. Ewing's sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol 1999;48:209-16.
6.	El Mograbi A, Soudry E. Ocular cranial nerve palsies secondary to sphenoid sinusitis. World J Otorhinolaryngol Head Neck Surg 2017;3:49-53.
7.	Leavey PJ, Mascarenhas L, Marina N, Chen Z, Krailo M, Miser J, et al. Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatr Blood Cancer 2008;51:334-8.
8.	Carraro MC, Rossetti L, Gerli GC. Prevalence of retinopathy in patients with anemia or thrombocytopenia. Eur J Haematol 2001;67:238-44.
9.	Beck RW, Smith CH. Neuro-ophthalmology: A Problem-oriented Approach. Boston: Little, Brown, and Co.; 1988.
10.	Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ, et al. Anemia and papilledema. Am J Ophthalmol 2003;135:437-46.
11.	Imai E, Kunikata H, Udono T, Nakagawa Y, Abe T, Tamai M. Branch retinal artery occlusion: A complication of iron-deficiency anemia in a young adult with a rectal carcinoid. Tohoku J Exp Med 2004;203:141-4.