|Year : 1991 | Volume
| Issue : 3 | Page : 129-131
Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcomas with management
MS Boparai, RG Dash
Dept of Ophthalmology, Armed Forces Medical College, Pune, India
M S Boparai
Director Medical Research. Ministry of Defence, 'M' Block, New Delhi - 110001
Source of Support: None, Conflict of Interest: None
Fourteen cases of orbital rhabdomyosarcoma have been studied. Clinical presentation was by way of short history, fast growing proptosis, inflammatory signs and epistaxis. Computerised tomography showed only a mass lesion without bony involvement and was very useful in follow up. Ultrasonography revealed pseudo-encapsulation of the tumour which is postulated as possibly specific of Rhabdomyosarcoma due to its fast growth, cellular content and oedema fluid. A survival rate of 21% over a 3 year period has been achieved by combined mode therapy consisting of surgery, radiation and chemotherapy.
|How to cite this article:|
Boparai M S, Dash R G. Clinical, ultrasonographic and CT evaluation of orbital rhabdomyosarcomas with management. Indian J Ophthalmol 1991;39:129-31
| Introduction|| |
Rhabdomyosarcoma is the most common malignant tumour of mesenchymal origin and also the commonest orbital malignancy in children. It is however a comparatively rare lesion for the general ophthalmologist. It should be suspected whenever one sees a rapidly growing unilateral proptosis in a child or a young adult. A tissue diagnosis is a must and should be aimed at.
There have been individual reports on the presentation and management of this malignancy, but there appears to be no reported series in our country. Over a period of 15 years, we have come across 14 cases of rhabdomyosarcomas. Their clinical evaluation, scanning characteristics and management are being presented.
| Material and methods|| |
A total of 148 cases of unilateral proptosis of neoplastic origin were examined. Each case underwent a proptosis work up which included clinical evaluations, ocular and systemic examination, laboratory work up and relevant classical radiological examination. Selected cases were subjected to computerised axial tomography (CT scan) and ultrasonography (USG). Majority of these cases were referred from other hospitals. Diagnosis was confirmed in all by histopathological examination following biopsy/excision biopsy. Based on these parameters, diagnosis of rhabdomyosarcoma was made in 14 cases. Following confirmation of diagnosis, treatment instituted was radiotherapy to the tune of 5000-6000 rads by cobalt 60 in divided doses both by anterior and lateral fields. Some patients were given chemotherapy additionally.
| Observations|| |
1. Rhabdomyosarcomas constituted 9.4% of total cases of unilateral proptosis of neoplastic origin. The age of these 14 cases is given in [Table - 1]. The youngest patient was 21/2 years and the oldest 19 years.
2. A total of 11 cases presented as fast growing painless proptosis. However, a feeling of tenseness was complained of. There was a history of duration of 8-12 weeks in 7 cases and 4-6 months in the other 4. For various constraints 3 patients sought proper medical advice after a year of the appearance of symptoms.
3. The patients presenting with acute short duration proptosis (11) had so called inflammatory signs by way of gross swelling, colour changes and prominent vessels. [Figure - 1] illustrates one such case. Presentation in 3 cases (age 15-20 years) was by way of slow growing proptosis. The proptosis ranged from 5-15 mm. and there were signs of globe compression and visual loss in 7 patients.
4. Two cases in whom there was intranasal extension in addition to fast growing proptosis had mild bouts of nose block and epistaxis before growth could be detected in the nose.
5. Radiologically only generalised orbital/soft tissue enlargement was detected. In no case was any bony rarefaction seen.
6. Computerised axial tomography showed a mass lesion in 7 cases in whom it was done with paranasal extension in two of them.
7. USG characteristics on 'B' mode were:a. an irregular anterior and
b. multiple low to medium amplitude echoes within the tumour mass
c. well defined posterior and (pseudoencapsulation).
'A mode scanning showed an initial tall spike followed by alternate low and medium amplitude spikes from within the lesion and a terminal tall spike. [Figure - 2] shows the 'B' mode and A mode ultrasound scan.
8. Histopathologically 10 cases were of embryonal type and 4 cases were of pleomorphic type. [Figure - 3] shows a typical embryonal growth. In none of the embryonal types could cross striations be demonstrated. In one case the initial biopsy returned a diagnosis of clear cell carcinoma and was thought to be a secondary deposit from some other primary source. However, in view of rapid growth the case was irradiated and then subjected to surgery. The diagnosis from the excision was an embryonal type of rhabdomyosarcoma.
9. Only 3 patients out of 14 are alive and are alive and are under regular follow up. 2 have been followed up for over 7 years and are free of any local recurrence or systemic involvement. 1 has been followed up for 3 years only and is symptom free. [Table - 2] shows the comparative survival rate following diagnosis and treatment. 8 patients died of local recurrence and metastasis in 6 months to two years in spite of local radiation and chemotherapy. 3 have been lost to follow up and are presumably dead.
| Discussion|| |
The age incidence and clinical presentation of tumours in the study is similar to the findings of Reese (1976), Miller and Dalagar (1974) and Jones (1965). The percentage of 9.4%
(14 cases) amongst 148 cases of unilateral proptosis of neoplastic origin may seem to be a rather high figure. However it has to be kept in mind that these are mostly referred cases and do not reflect incidence. Similar figures were reported earlier by Boparai (1985). Any fast growing proptosis in children should be taken as rhabdomyosarcoma till proved otherwise.
Classical radiology is not of much help but CT scan gives an idea of the total extent of the lesion. USG showed a 'pseudocystic' appearance which is considered characteristic. This is at variance from the findings of Coleman (1977) wherein he found no characteristic appearance on echography. However, Nitin Verma (1965) described the pseudocystic ap pearance and thought it to be due to spindle cells of embryonal rhabdomyosarcoma having abundant cytoplasm, loosely separated by oedema fluid. A repeat CT scan and ultrasonography is of tremendous value in assessing the effectiveness of treatment.
Histologically 10 cases (71%) were found to be of the embryonal type which compared favourably with the figures of Jones et al (67%) and Porterfield and Zimmerman (72%). Diagnosis on biopsy in one case was missed. It might have been due to insufficient or unrepresentative specimen. Adequate material therefore should be taken for biopsy. Cross striations described as one of the features are difficult to demonstrate in the embryonal variety (Hogan and Enterline 1958; Stobbe and Dargeon 1950). In none of our cases it was possible to demonstrate cross striations. Factors like tissue sampling, adequate fixation, slide preparation, proper staining and diligent search may have been the reasons for this.
Treatment modalities and survival rate has varied in various reported series. Jones et al (1965), Porterfield and Zimmerman (1962) and Frayer and Enterline (1959) have weighed heavily in favour of exenteration. In recent years the pendulum has swung markedly in favour of radiation therapy alone or combined with chemotherapy following tissue diagnosis (Cassady 1968; Sagerman 1972). Still others like Heyn (1974) and Kilman (1973) have advocated combined surgery, radiotherapy and chemotherapy. Both these regimes offer longer survival than exenteration alone. Furthermore exenteration, a mutilating surgical procedure is not easily accepted by parents. In this small series a 3 year survival rate of 21% (3 cases) has been achieved by combined surgery, radiation and chemotherapy. 2 patients have been followed up for 7 years and are symptom free. They are however showing long term effects of radiotherapy by way of hypoplastic bones, corneal vascularisation, and cataract formation. This is counterbalanced by the retention .of globe and avoidance of mutilating surgery.
| References|| |
Ira Snow Jones and Frederick A. Jakobiec : Diseases of the Orbit, PP 435-457, Harper and Row, New York 1979.
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[Figure - 1], [Figure - 2], [Figure - 3]
[Table - 1], [Table - 2]