|Year : 1980 | Volume
| Issue : 2 | Page : 101-103
A Basu, Amitava Das
Department of Ophthalmology, R.G. Kar Medical College, Calcutta, India
Deptt. of Ophthalmology Medical College, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Basu A, Das A. "Orbital haemangiopericytoma". Indian J Ophthalmol 1980;28:101-3
Haemangiopericytoma is a very rare tumour of the orbit. It is classified together with hemangioendothelioma, leiomyoma and leiomyosarcoma, under the term angiosarcoma. Though it is classified under angiosarcoma still it may be benign or malignant. In 12 to 57% of cases it is malignant. Histological differentiation between the benign and malignant forms is practically impossible. It is infiltrative and, if not completely excised, it may recur. It may invade the orbital bones, nose, sinuses and anterior cranial fossa or enter the cranial cavity and prove fatal. The tumour may appear at any age from infancy onwards but usually before 50 years. There is no sex predilection. Stout reported 285 cases of haemangiopericytoma all over the body of which 14% metastasized. Treatment must be complete excision, involving exenteration, if that is necessary. But extreme vascularity may make it difficult. Malignant haemangiopericytoma are known to respond in some degree to irradiation8. Enzinger and Smith reported a 5 year survival rate of 70% among 93 patients with haemangiopericytoma by post operative irradiation. Del Ragato and Spjut are of opinion that radiotherapy is not an alternative to surgery, but adjuvant radiotherapy is indicated. Kent is of opinion that they are radiosensitive. Duke Elder is also of opinion that post operative irradiation, in judicial doses, after wide excision may inhibit recurrences.
| Case report|| |
A male, Hindu patient of 40 years was admitted with the complaints of gradually increasing proptosing of the right eye for last 6 months and of dimness of vision in the same eye for last two months. On local examination of the right eye there was a retrobulbar mass pushing the right eye downward and laterally. The mass extended from behind the globe to outside the orbit and under the right upper lid pushing it forward on its medial and upper segment. There was restriction of movements of the eyeball in those two segments. The size of the visible mass was 2" by 12 ". The mass was loculated. Skin over the mass was normal, with slight vascular markings, movable of normal colour and temperature. Eyeball could not be pushed back. There was no change of size of the mass with change of posture of the head. No bruit was heard over the region. There was circumcorneal injection and cornea was ulcerated due to exposure [Figure - 1]a.
His vision in the right eye could not be checked due to corneal ulcer. His vision in the left eye was normal. His personal and family history was non suggestive. On general examination he was of normal built and nutrition, but a little anaemic. Cranial nerves, motor functions and reflexes were normal. X-Ray of orbit and other investigations did not show any abnormality. Corneal ulcer healed with atropine and soframycin ointment locally, lateral tarsorrhaphy and pressure bandage.
The growth was removed under general anaesthesia. One incision was given under the upper eye lid through conjunctiva and orbital septum extending from superior rectus to medial rectus muscles, then by finger and gauze dissection the growth was separated from the surrounding structures and a semi solid, spongy feeling mass of about 2 inches by 1 inch was removed [Figure - 2]a. I here was excessive bleeding during operation making the operation very difficult. Bleeding points were crushed and some were ligatured. I.V. plasma was started in the operation theatre. Antibiotics were given. There was severe oedema of the face next day which subsided within few days, otherwise the recovery was uneventful [Figure - 1] b.
The mass removed was not smooth surfaced. There was slight oozing from the surface of the growth which stopped by itself very shortly. The capsule of the growth was torn in some places. Histological examination revealed it to be haemangiopericytoma [Figure - 2]b.
Histological report runs as "Section shows numerous vessels lined by normal endothelium separated by tumour cells in the connective tissue cellar. Tumour cells are round, ovoid or fusiform with prominent vesicular nuclei. In some places the cells are arranged in whorls. Reticulin stain indicated reticulin fibres encompassing each of small group of cells outside the vessel wall."
| Discussion|| |
Haemangiopericytoma are a group of tumours characterised by a rich vasculature and occurring wherever there are capillaries. They consist of endothelial channels surrounded by proliferation of contractile pericytes, which are round, ovoid or fusiform, vaguely defined, pale staining cells with prominent, vesicular nuclei. The cells are surrounded by reticulin fibres, the whole being enclosed within a capsule of collagenous fibres and reticulin. The pericytes ate contractile yet have no myofibrils and they are assumed to have relationships to smooth muscle cells. Pericytes lie outside the reticulum sheath of the capillary wall and are differentiated from hemangioendotheliomas, in which the proliferating endothelial cells lie inside the reticulum sheath. Fibrosis or cartilaginous differentiation may occur.
| Summary|| |
Investigations did not suggest any abnormality in a case of proptosis. On exploration of the orbit a vascular mass of about 2" x 1" was removed, which on histological examination proved to be haemangiopericytoma.
| Acknowledgement|| |
1. Our thanks are due to Prof. K.K. Banerji, Principle cum Supdt., R. G. Kar Medical College and hospitals, Calcutta, for permission to report the case.
2. Our sincerest thanks to Prof. K.P. Sengupta, Director, I.P.G.M.E. & R , Calcutta, for the diagnosis and microphotographs.
| References|| |
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Stout, A.P. : 1949, Quoted by Duke Elder.
Murphy, W.T. , 1967, Radiation Therapy, 2nd. Edn., page 908, Saunders, London.
Enzinger, F.M. and Smith, B.H., 1976. Hum. Pathol, 7 : 61.
Del Regato, J.A. & Spjut, H.J. 1977, Cancer, 5th Edn. page 931, C.V.Mosby Co., St. Louis.
Kent, 1957, Amer. J. Roentgen., Quoted by Duke Elder.
Francois, J. & Hanssens, M. 1962, Ann. Oculist (Paris), Quoted by Duke Elder.
[Figure - 1], [Figure - 2]