Bilateral proptosis due to metastatic Ewing's sarcoma of the orbit : fine needle aspiration cytology (FNAC) and histopathology of a case

AK Khurana; BK Ahluwalia; S Gupta; Choudhry Rajan; BB Arora

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Year : 1992 | Volume : 40 | Issue : 1 | Page : 15-17

Bilateral proptosis due to metastatic Ewing's sarcoma of the orbit : fine needle aspiration cytology (FNAC) and histopathology of a case

AK Khurana¹, BK Ahluwalia¹, S Gupta¹, Choudhry Rajan¹, BB Arora²
¹ Department of Ophthalmology, Medical College and Hospital, Rohtak-124 001, India
² Department of Pathology, Medical College and Hospital, Rohtak-124 001, India

Correspondence Address:
A K Khurana
34/93, Medical Enclave, Rohtak-1240
India

Source of Support: None, Conflict of Interest: None

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PMID: 1464447

Abstract

Bilateral proptosis due to metastatic Ewing's sarcoma is an extremely rare presentation and thus merits reporting. The role of fine needle aspiration cytology in the diagnosis is highlighted.

How to cite this article:
Khurana A K, Ahluwalia B K, Gupta S, Rajan C, Arora B B. Bilateral proptosis due to metastatic Ewing's sarcoma of the orbit : fine needle aspiration cytology (FNAC) and histopathology of a case. Indian J Ophthalmol 1992;40:15-7

How to cite this URL:
Khurana A K, Ahluwalia B K, Gupta S, Rajan C, Arora B B. Bilateral proptosis due to metastatic Ewing's sarcoma of the orbit : fine needle aspiration cytology (FNAC) and histopathology of a case. Indian J Ophthalmol [serial online] 1992 [cited 2021 Mar 3];40:15-7. Available from: https://www.ijo.in/text.asp?1992/40/1/15/24420

Introduction

Ewing's sarcoma is a primary intramedullary malignant neoplasm of bone classically arising from the bones of the limbs, trunk and pelvis^[1],[2].The tumour, common in the first and second decades of life, is highly malignant and inevitably spreads to other bones and lungs^[3], proving fatal within a short span of time. Metastases in the orbit from distant primary sites presenting as proptosis are rare and unilateral, usual situated on the same side as the primary tumour^[4] . This paper describes a case which presented with bilateral proptosis, which retrospectively was diagnosed as metastatic spread to the orbits from a primary Ewing's sarcoma of the right iliac crest.

Case report

A 12 year old male child presented with gradual, painless, progressive bulging of both eyeballs for two months, not associated with defective vision, diplopia, redness of the eyes or any other ocular complaint. The child was suffering from intermittent, mild grade fever accompanied by body aches, loss of weight and appetite for the last six months. On general physical examination, the child was moderately built but poorly nourished, anaemic and emaciated, weighing just 19 kg. There was no jaundice and lymphadenopathy. A diffuse swelling, 5 cm X 3 cm with ill-defined margins and having bony consistency was present in relation to the right iliac crest. On roentgen film, this region showed a destructive process and the consulting orthopaedic surgeon advised biopsy. [Figure - 1] is the photograph taken 15 days after biopsy from the right iliac crest.

Ocular examination revealed bilateral symmetrical non-reducible proptosis with ptosis [Figure - 2]. There was 10^o exotropia with 4^o hypertropia and marked restriction of adduction in the right eye. Visual acuity was 6/6 in both eyes. Fundus examination revealed bilateral early papilloedema. Rest of the ocular examination was unremarkable.

Skiagrams of the orbit and optic foramina were normal. B-scan orbital ultrasonography showed homogenous increased density of retrobulbar tissues. CT scan of the orbits showed evidence of bilateral proptosis associated with high density areas, generalised thickening of orbital soft tissue, appreciable enlargement of extra ocular muscles and thickening of optic nerves [Figure - 3]. No evidence of bony involvement was seen. Haemoglobin was 8.5 G%, W.B.C's 7000 consisting of 60% polymorphs, 35% lymphocytes, 2% monocytes, 2% eosinophils and 1% basophils; bleeding time 1' 50" and clotting time 3' 20".

Histopathology report of biopsy from the right iliac crest:

Histologically, the lesion was densely cellular, consisting of compartments of rounded uniform cells having periodic acid Schiff (PAS) positive cytoplasm depicting presence of glycogen. The nuclei were oval having thin chromatin and usually a single nucleolus. Little, thin, intercellular fibrous strands infiltrating the bony and soft tissues of the biopsy specimen were also seen [Figure - 4].

Fine needle aspiration cytology (FNAC):

The aspiration smears from the retrobulbar space were cellular and consisted of monolayered sheets of cells with indistinct cell borders, with scanty pale cytoplasm which was PAS positive [Figure - 5]. The cellular characteristics of the aspirate were almost identical to that of biopsy from the right iliac crest.

Treatment in the form of radiotherapy of the primary tumour of the right iliac crest along with chemotherapy was instituted. However, the general condition of the child kept on deteriorating and unfortunately he died within six months of the diagnosis of Ewing's sarcoma.

Discussion

Ewing's sarcoma, being an extremely malignant tumour metastasises at a very high rate and also tends to break through cortex of bone of origin into the adjacent tissues. Spread of this tumour into the orbits is most likely through blood. Metastases to orbits are extremely rare in Ewing's sarcoma^[5]. Further, in the etiological list of proptosis in childhood also, metastatic Ewing's sarcoma is not so common. In a large series estimating the causes of proptosis in children, only two patients out of a total of 585 had Ewing's sarcoma^[4]. Most of the patients with metastatic orbital Ewing's sarcoma had unilateral proptosis, generally on the same side as the primary tumour and clinically presented as rapidly developing proptosis with haemorrhage and necrosis as in neuroblastoma^[6],[7]. Mohan and Sen^[8] have reported 2 cases of metastatic Ewing's sarcoma in their series of 91 cases of unilateral proptosis of neoplastic origin. Bilateral proptosis as seen in the present case in extremely rare and to the best of our knowledge has not been reported in the available literature.

The history of painless, progressive, bilateral proptosis together with cachexia and fever off and on accompanied by a constant deterioration of general health suggested the possibility of some neoplastic process. Thus the differential diagnosis in the present case would have been leukaemia, lymphoma or metastatic neuroblastoma. The presence of a painless and non-inflammatory bony swelling over the right iliac crest also suggested the possibility of a primary bony tumour with secondaries in the orbits. The histopathological picture of bone biopsy from right iliac crest [Figure - 4] and aspirate from retrobulbar spaces [Figure - 5] were identical and conclusively confirmed the diagnosis of Ewing's sarcoma of the right iliac crest with metastatic spread to orbits.

Fine needle aspiration cytology is an excellent diagnostic technique and its role in determining the Biology of proptosis cannot be overemphasized^[9],[10]. Moreover, in cases where 'metastases' are suspected, correlation of histological findings, from the primary site of tumour and aspirated material from retrobulbar area, as in the present patient, is extremely useful and invariably diagnostic.

The purpose of reporting this case is to stress that bilateral proptosis due to metastases from Ewing's sarcoma can occur early and may even be the first presenting sign, as seen here. Therefore, all cases with proptosis, especially children require a thorough examination of the skeletal system to rule out a primary bony tumour; not only in unilateral proptosis but in bilateral proptosis too.

References

1.	Ewing J. Diffuse endothelioma of bone. Proc NY Pathol. Soc. 1921, 21 : 17-24.
2.	Jereb B. Ong RL. Mohan M. Caparros B & Excelby P Redefined role of radiation in combined treatment of Ewing s sarcoma. Pediatric. Hematol. Oncol. 1986. 3: 111-118.
3.	Lichtenstein L. Bone tumours. St. Louis, Mosby 1972. PP 256-276.
4.	Crawford JS. ed The eye in childhood. Grune and Stratton. 1983, pp 366-367.
5.	Woodruff G, Thorner P 8. Skarf B. Primary Ewing's sarcoma of the orbit presenting with visual loss Br. J. Ophthalmol. 1988. 72: 786-792.
6.	Coley B, Higinbotham N & Bowdon L Endothelioma of bone (Swing's sarcoma). Ann. Surg.. 19-46, 128 : 533
7.	Albert DM, Rubenstein FA & Scheie HG. Tumour metastasis to the eye: 11. Clinical study in infants and children. Am. J. Ophlhalmol.: 1967; 63: 727
8.	Mohan H & Son DK Unilateral proptosis of neoplastic origin Proc. All India ophthal. Soc. 1970. Vol xxxi : 31-41.
9.	Kennerdell JS. Dekker A and Johnson BL Oribital fine needle aspiration Cytology: The result of its use in 50 patients Neuro-Ophthalmology 1980, 1: 117.
10.	Tarkkanen A, Koivunieme A. Liesmaa M & Nerenmies L Fine needle aspiration biospy in the diagnosis of orbital tumours. Graefe's archives of clinical and experimental Ophthalmology. 1982, 219: 165.