|Year : 1990 | Volume
| Issue : 4 | Page : 202-204
Embryonal rhabdomyosarcoma of orbit-A case report
OPS Maurya1, R Patel1, V Thakur1, Rajendra Singh1, Mohan Kumar2
1 Department of Ophthalmology, Institute of Medical Sciences, Banaras Hindu University, Varanas-221 005, India
2 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanas-221 005, India
Department of Ophthalmology, Institute of Medical Sciences, Banaras Hindu University, Varanas-221 005
Source of Support: None, Conflict of Interest: None
Embryonal rhabdomyosarcoma of orbit presenting as a case of rapid proptosis of the right eye is reported in a 4 year old male child. There was no evidence of recurrence during a follow up of 6 months. Rhabdomyosarcoma is one of the most common primary malignant orbital neoplasms of child hood. It usually produces a precipitously progressing unilateral proptosis of sudden onset. It is a highly malignant neoplasm of pleuripotential embryonic mesoderm, which commonly differentiates to form cells similar to rhabdomyoblasts of the foetus. Because of the presence of elongated cells that contain abundance of eosinophilic glycogen rich cytoplasm, it is generally referred to as embryonal form of rhabdomyosarcoma.
|How to cite this article:|
Maurya O, Patel R, Thakur V, Singh R, Kumar M. Embryonal rhabdomyosarcoma of orbit-A case report. Indian J Ophthalmol 1990;38:202-4
|How to cite this URL:|
Maurya O, Patel R, Thakur V, Singh R, Kumar M. Embryonal rhabdomyosarcoma of orbit-A case report. Indian J Ophthalmol [serial online] 1990 [cited 2020 May 31];38:202-4. Available from: http://www.ijo.in/text.asp?1990/38/4/202/24496
| Case report|| |
Sonu, a 4 year old male child was brought to the out patient department with complaints of painless, rapidly progressing protrusion of the right eye ball since 1 month which was not associated with apparent diminution of vision. There was no history of any injury, fever or chronic cough. The prenatal and birth history of the child was uneventful.
The patient was of average build for age aid there was no anaemia or lymphadenopathy. The p tient had no apparent diminution of vision. The righ eyeball was moderately proptosed and deviated down and outwards. There was gross restriction of ocular movements of the right eye in all positions gaze. Both upper and lower eye lids were swollen and bulbar conjunctiva was found to be chemosed. Fundus examination of the right eye revealed slight blurring of the optic disc margins, mild venous dilatation and a normal foveal reflex. Hertel's exophthalmometry reading of the right eye was found to be 22 mm in comparison to the normal left eye of 14 mm.
A non tender globular mass (3 cm x 2 cm) with irregular surface and firm consistency was palpable in the upper nasal quandrant of the right orbit. It was not mobile and nonpulsatile or expansible on coughing r crying. The left eye showed no significant abnormality on clinical examination. (Photo 1).
Complete haemogram, including general blood picture, ESR, urine and stool examination were found to be within normal limits. X-ray of the skull, orbit and optic foramen revealed no abnormality. Computerised tomography revealed an irregularly mixed dense well defined shadow in the retrobulbar space of the right orbit. The optic nerve could not be separated out and the lesion seemed to be adherent to the eye-ball (Photo 2 & 3).
Anterior orbitotomy of right orbit was done, under G.A. and a bilobed globular mass with stalk was excised. The tumour measured 4 1/2 cm x 3 1/2 x 3 cm and weighed 14 grams (Photo 4). The mass was examined histopathologically and diagnosed to be embryonal rhabdomyosarcoma. The patient was then subjected to radiotherapy (Photo 5).
| Discussion|| |
From the clinical point of view, rhabdomyosarcoma is of an adult and infantile form.  The adult rhabdomyosarcoma arises from voluntary muscle and is extremely rare since the fully mature muscle cell is not prone to malignant change. The infantile form is much more common and indeed is the most frequent malignant tumour in the orbit. It is seen at an average age of 6 or 7 years although in our case it has occurred earlier i.e. at 4 years.
From the pathological point of view several types have been described - embryonal sarcoma arising from the embryonic mesenchymal tissue either prospective muscle or undifferentiated tissue capable of heteroplastic differentiation into muscle, non striated embryonal rhabdomyosarcoma and striated embryonal rhabdomyosarcoma.  Reports have stressed a preponderence in males and we also found a male child to be affected.
The mode of presentation of this tumour varies considerably and in our case it presented with swelling in the right medial canthus.
The embryonal rhabdomyosarcoma consists of a loosely knit mass of primitive mesenchymal cells among which pleomorphism is a prominent feature; most of the cells are round, oval or stellate but sometimes spindle shaped. The cytoplasm is usually scanty while some have long ribbons or streamers of eosinophilic cytoplasm, often containing cross-striations. The nucleii are round or oval and are rich in chromatin; mitosis is common. The cells are frequently arranged in a loose syncytium but sometimes in the form of compact masses or in alternating bands of loosely arranged and closely packed cells.(Photo 6 & 7)
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]