|Year : 1980 | Volume
| Issue : 1 | Page : 27-29
PM Chaudhuri1, A Sen1, SC Sen1, KP Sen Gupta2, S Chakravorthy2
1 Department of Ophthalmology, Institute of Post Graduate Medical Education & Research Calcutta, India
2 Department of Pathology, Institute of Post Graduate Medical Education & Research Calcutta, India
P M Chaudhuri
Department of Ophthalmology, Institute of Post Graduate Medical Education & Research Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chaudhuri P M, Sen A, Sen S C, Sen Gupta K P, Chakravorthy S. Rhabdomyosarcoma. Indian J Ophthalmol 1980;28:27-9
|How to cite this URL:|
Chaudhuri P M, Sen A, Sen S C, Sen Gupta K P, Chakravorthy S. Rhabdomyosarcoma. Indian J Ophthalmol [serial online] 1980 [cited 2020 Oct 21];28:27-9. Available from: https://www.ijo.in/text.asp?1980/28/1/27/31043
Rhabdomyosarcomas are extremely malignant tumours of myogenic origin, and are considered to be the most common primary malignant neoplasm of the orbit in children,.
Rhabdomyosarcoma need not always arise in muscle, rather it might originate from the embryonal tissue or from the mesenchymal tissue with the potency for aberrent differentiation of muscle fibres. Rhabdomyosarcoma infiltrate and metastasize through blood and lymphatic channels. Most common sites for metastasis are lung, bone and liver. They may spread to lymphnodes in only 10% of cases. Bilateral orbital metastasis is an extremely rare condition.
Rhabdomyosarcomas have been classified into the embryonal, alveolar, pleomorphic and mixed types. The alveolar rhabdomyosarcoma involving the orbit being extremely rate., Orbital Rhabdomyosarcomas are observed twice more frequently in males than in females. A few cases have been reported as occuring in siblings. This tumour has also been reported to arise following radiation therapy for retinoblastoma.
The purpose of this paper is to record an academically interesting as well as a rare case of orbital metastasis of rhabdomyosarcoma.
| Case report|| |
A 9-years old Hindu male was admitted in the ophthalmic department of Seth Sukhlal Karnani Memorial Hospital and Post Graduate Medical Education And Research, Calcutta with bilateral proptosis and exposure keratitis.
A rapidly increasing swelling was noticed on the right calf muscle about 12 weeks back. It was associated with irregular rise of temperature.
After 6 weeks, multiple painful swellings developed over various parts of his scalp and forehead with progressive protrusion of both the eyes. There was no complaint regarding visual loss for first 10 weeks but for the last two weeks he was having blurred vision and photophobia.
On examination, the patient was pale and emaciated. He looked very ill and had marked anorexia. There was a diffuse hard swelling involving the whole of right calf muscle. Right inguinal lymph nodes were enlarged, stony hard and matted. Swellings over the scalp and forehead were of varying sizes and were very tender and hard.
Liver and spleen were not palpable. The chest was clear and clinically there was no evidence of intracranial involvement.
The right eye was proptosed about 40 mm. and left eye about 38 mm. The eyelids were oedematous, conjunctiva very much congested and chemosed. There was sloughing corneal ulcer in the right eye and exposure keratitis in the left eye [Figure - 1]. Vision in the right eye was reduced to perception of light only and that in the left eye to finger counting was present upto 3 meters. Movements of the eye ball were restricted in all directions, the tension was normal in both eyes and fundus oculi could be seen with difficulty and no significant abnormality was noted.
X-ray of lower extremities did not show bony involvement. Skiagraphy of chest, skull and abdomen showed nothing abnormal. X-ray of orbit showed marked soft tissue density, without any bony involvement of the orbit.
Sections from the tumour tissue removed from the calf muscle showed alveolar pattern of the fibro-vascular stroma enclosing the neoplastic cells. The cells, were variable in size and shape with acidophilic cytoplasm and hyperchromatic nucleus exhibiting numerous mitosis. A few racquet cells, strap cells and occasional multinucleated giant cells were also discernible. Myofibrils and cross-striations were not demonstrable [Figure - 2][Figure - 3]. Tissue obtained by left orbital exploration as well as that of inguinal lymph node revealed metastatic growth of the above description.
| Discussion|| |
Rhabdomyosarcoma was first described by Weber. Orbital Rhabdomyosarcoma was first reported by Bayer. Only in recent years its high incidence has been appreciated. To the knowledge of the authors, this is the first case ever reported of alveolar rhabdomyosarcoma of calf muscle causing bilateral orbital metastasis. A considerable number of rhabdomyosarcoma primarily in orbit have been reported, but only a few cases of secondary orbital metastasis have been reported so far.
Kirk and Zimmerman reported a case of rhabdomysarcoma of the orbit of a girl of 9-years old who had nephrectomy done for an embryonic renal tumour that exhibited areas of rhabdomyosarcomatous differentiation at 14 months of age. After proper treatment she showed no incidence of recurrence at the age of 21 years.
Another probable secondary rhabdomyosarcoma was reported by Stephen et al. A 78-years old white man was reported with pericardial effusion. Cytological examination of the pericardial fluid revealed a typical cells suggesting malignancy. Later a thoracentesis and pericardiotomy were done, but histological examination did not show any malignancy. After one month he developed rapidly progressing proptosis. Exenteration was done. Histopathological examination showed pleomorphic rhabdomysarcoma of the orbit.
Jones et al observed 62 cases of ocular rhabdomyosarcoma. Of these few cases also had tumour masses in the nose in which cases the initial symptoms were associated with nasal obstruction. Though some considered these cases to be examples of rhabdomyosarcoma primary in the orbit, there are some who believe orbits are secondarily involved, the primary being situated in nasopharynx,.
Henderson studied 10 cases of orbital rhabdomyosarcoma, 5 cases of which were classified as secondary. This shows a very high incidence of the type. Since in our case the patient developed the tumour in this right calf muscle first, followed by bilateral proptosis after six weeks, we considered this case to be a bilateral metastasis of rhabdomyosarcoma primary being in the right calf muscle. The time lag and bilateral involvement of orbit is strongly suggestive of secondary involvement of orbits.
| Summary|| |
A case of bilateral secondary orbital deposits from the primary alveolar type of rhabdomyosarcoma of right calf muscle in a male child of 9 years is reported and the literature is briefly reviewed.
| References|| |
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Weber 1854. quoted by Jones et al 1966.
[Figure - 1], [Figure - 2], [Figure - 3]