|Year : 1979 | Volume
| Issue : 1 | Page : 15-17
Alveolar soft part sarcoma of the orbit
PK Mukherjee, S Agrawal
Department of Ophthalmology,Pt. J.N.M. Medical College, Raipur, India
P K Mukherjee
Department of Ophthalmology,Pt. J.N.M. Medical College, Raipur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mukherjee P K, Agrawal S. Alveolar soft part sarcoma of the orbit. Indian J Ophthalmol 1979;27:15-7
Alveolar soft part sarcoma is a name coined to a group of uncommon tumors with a wide distribution all over the bodys,. The same tumor has also been called as malignant granular cell myoblastoma, and malignant non chromaffine para ganglioma. Henderson doubted if these neoplasms are synonymous. Duke Elder however has put them in the same group. So few of these neoplasms have been reported to occur in the orbit that it is unwise to draw any conclusion regarding their clinical behavior except that they are malignant. Only five cases that seem to qualify as orbital example of this neoplasrn have been reported,.
In view of the rare occurrence of the tumor the following case is being reported:
| Case Report|| |
A thirty years old male farmer came to Ophthalmic outpatient department complaining of severe pain, lacrimation, photophobia and loss of vision in the right eye of eight days duration There was no history of trauma to she eye. The patient revealed that he had noticed a diffuse swelling which was painless. Since last two months the swelling had grown rapidly and spread over the right temporal side adjoining the orbit as well. The eye ball had become more and more prominent and fixed. Since previous fifteen days he had been unable to close the eye properly. The eye had become red. Since last eight days the pain, lacrimation, and photophobia, had become unbearable. Vision which was normal previously had come to perception of light only.
The past history and family history were nonsignificant.
On examination the right eye was proptosed, pushed forvard, medially and downwards. The movements of the eye ball were restricted all round. The orbicularis oculi was paralysed. There was a diffuse swelling in the region of the lacrimal gland. The swelling was rubbery in consistency, not fixed to the skin or bone. It's posterior margin could not be defined. The swelling seemed to spill over the orbital margin and extended in the right temporal region wh ch showed diffuse swelling with bone destruction of the outer wall of the orbit. The lower half of cornea was covered by an ulcer extending from the limbus to the upper part of the pupil. One fourth of the anterior chamber was full of hypopyon. The pupil was constricted, irregular and not reacting to light. The vision was limited to preception of light with accurate projection from all sides.
The left eye was normal in all respects. Systemic examination did not show any positive sign.
The case was diagnosed as a malignant tumor of lacrimal gland.
The patient was admitted in the hospital. A scraping from the corneal ulcer was sent for culture and sensitivity to antibiotic. In the mean time the patient was given 100 mg. pethidine i.m. for pain. He was put on injection streptopencillin I gm. i.m. daily. The local treatment consisted of subconjunctival injection of 2.5 lacs of crystalline penicillin, instillation of atropine sulphate eye ointment 1 per cent with neosporin eye ointment, pad and bandage.
The corneal scraping showed pneumococci sensitive to all the antibiotics. Within four days of treatment the hypopyon disappeared but there was no abatement of proptosis, on the contrary the cornea became more exposed and the ulcer was on the verge of perforation. So it was decided to exenterate the orbit. The orbit was exenterated on 19.10.76 under general anaesthesia. The exenterated material contained a firm mass extending from the superior temporal to the apex of the orbit, none of the muscles of the globes were infiltrated but the orbicularis was infiltrated by the tumor. The eye ball and the lacrimal gland were intact.
On histopathological examination the tumor came out to be an alveolar soft part sarcoma [Figure - 1][Figure - 2].
The post operative period was unventful. It was decided to put a skin graft in the exenterated socket which the patient refused and left the hospital against medical advice. The patient has not been seen since then.
| Discussion|| |
Abrikossoff published several cases under the name of granular cell myoblastoma. These benign tumors were eponymed as Abrikossoff's tumors. He cited a prior case from literature and thought that it was the malignant tumour later form of the granular cell myoblastoma. He added cases to this category so the malignant granular cell myoblastoma came to include a heterogenous collection of tumors of variable morphology. A malignant tumor was reported by Christopherson et al in which the cells were arranged in alveolar fashion arround a central space and hence named alveolar soft part sarcoma. The tumor had striking similarity with malignant granular cell myoblastoma. However there were some distinct difference between the two tumors which started the controversy. "If these tumors belonged to one group?" The next chapter emerged-when another neoplasm was reported under the name of malignant non chromaffin paraganglioma. The cellular arrangement had similarity to alveolar soft part sarcoma and organoid variety of malignant granular cell myoblastoma. Thus the present trend is to use the three terminology as synonymous.
So far only five cases have been reported to arise from the orbit,,.
As stated earlier with so scanty material it is difficult to draw clinical features of this rare and elusive tumor. It's multifaceted microscopic appearance is responsible for prevailing confusion regarding the nomenclature and cell origin. Out of five cases reported earlier four died before one year of radical surgery. One survived more than one year. Exenteration of orbit may prolong the life but does not eradicate the tumor.
| Summary|| |
A rare case of alveolar soft part sarcoma of the orbit in a 30 years old male has been reported.
| References|| |
Abrahams, I.W., Fenton, R.H., Vindone, H. 1968. Arch. Ophthal., 79, 185.
Abrikossoff, A.I. 1931. Virchows, Arch. (Pathol. Anat.) 280. 723.
Abrikossoff, A.I. 1926. Virchows, Arch, (Pathol. Anat.) 260, 215.
Ackerman, L.V., Phelps, C.R, 1946. Surgery 20, 511.
Altamirano-Dimas, M., Albores-Saavedra, J. 1966, Arch. Ophthal., 75, 496.
Christopherson, W.M., Foote, F.W. and Stewart F.W. 1952. Cancer 5, 100.
Duke Elder, S., 1974. System of Ophthalmology,
Vol. XIII Part II, P. 1115 Henry Kimpton London,
Henderson, J.W. 1973. Orbital. Tumors, 289, 292,
W.B. Saunders Company, London.
Nirankari, M.S., Greer, C.H. Chadda, M.R., 1963. Brit. J. Ophthal., 47, 357.
Ravich. A. Stout, A.P. and Ravich, R.A. 1945. Ann. Surg. 121, 361.
Reese, A.B. 1963.
Tumors of the Eye Second Ed. P. 449,
Harper & Row Publishers.
Rose, R.C., Miller, T.R., Foote, F.W. (Jr.) 1952. Cancer 5, 112.
Smetana, H.F., Scott, W.F. 1951.
Mill Surg. 109, 303.
[Figure - 1], [Figure - 2]