|Year : 2004 | Volume
| Issue : 1 | Page : 62-64
Locally aggressive orbital fibrous histiocytoma
Mandeep S Bajaj, A Sethi, S Kashyap, Thanikachalam, N Pushker
Oculoplastic and Pediatric Ophthalmology Services, Dr. R.P. Centre for Ophthalmic Sciences, New Delhi, India
Mandeep S Bajaj
Oculoplastic and Pediatric Ophthalmology Services, Dr. R.P. Centre for Ophthalmic Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
We report a rare presentation of a massive, locally aggressive, fibrous histiocytoma of the orbit. The importance of histopathological grading and appropriate management are highlighted.
Keywords: Orbit, fibrous histiocytoma, tumour, proptosis, histopathology
|How to cite this article:|
Bajaj MS, Sethi A, Kashyap S, Thanikachalam, Pushker N. Locally aggressive orbital fibrous histiocytoma. Indian J Ophthalmol 2004;52:62-4
Fibrous histiocytomas (FH) constitute 1% of all orbital tumours, and are the commonest primary mesenchymal tumours of the orbit in adults. Except for a few case series, ,,,most cases of ocular or orbital FHs have been reported sporadically worldwide.,, We report a case of a locally aggressive FH in an adult which resulted in a huge, unilateral, orbital mass.
A 58-year-old male presented with a history of a rapidly increasing mass. It had started 8 years earlier as a small painless, nodule near the left lateral canthus. It was excised, but recurred 2 years later.
The best corrected visual acuity was 6/12 in the right eye and no perception of light in the left eye. A large, multilobulated, soft to firm mass, 13x14cm in size, was observed in the left orbit. The tumour completely filled the socket, with no externally visible eyeball structure [Figure - 1]. The right eye was normal except for cataractous changes in the lens and dry age-related macular degeneration.
The CT Scan demonstrated an ill-defined mass, filling the entire left orbit surrounding the globe and extending anterior to it. The uveo-scleral coats were thickened. There were focal areas of calcification within the mass. The left orbit was mildly enlarged with bowing of the medial wall. No intracranial extension of the mass was seen. Metastatic work up was normal.
An incisional biopsy, measuring 20 x 20 x 10 mm, was performed. Haematoxylin and eosin stained sections revealed spindle-shaped tumour cells arranged in a storiform pattern, showing minimal to moderate degree of cellular pleomorphism, few tumour giant cells and mitotic figures of 5-7 HPF, with a variable admixture of ovoid histiocytic cells and spindle-shaped fibroblasts. The tumour had infiltrating margins with areas of necrosis. A histopathological diagnosis of locally aggressive FH was made. An orbital exenteration was performed under general anaesthesia. The exenterated specimen measured 130 x 120 x 105 mm. The tumour also involved the overlying skin. A small, atrophic globe, at the anterior aspect of the specimen, did not appear invaded by the tumour. The cut section of the mass showed a firm, greyish white, multilobulated tumour. The histopathology of the tumour mass was consistent with the biopsy findings [Figure - 2]a, b. In addition, occasional areas of calcification were seen. The eyelids were infiltrated by the tumour. No recurrence was seen over a 2-year follow up.
The size of locally aggressive FH ranges from 2-8 cm and it is extremely rare for it to present as a gigantic orbital mass, as seen in our case.
The cellular origin of FH is debatable. It is considered of histiocytic or primitive mesenchymal cell origin., It has been histologically graded as benign, locally aggressive and malignant. Benign FHs are usually well encapsulated and tumour cells do not show significant nuclear pleomorphism or prominent nucleoli. Giant cells, if present, have no mitotic figures. A storiform or cartwheel pattern of the spindle shaped cells is characteristic of FH. Locally aggressive FH may be grossly encapsulated, but histologically displays infiltrating margins. No significant nuclear pleomorphism or cellular atypia is observed. They show plumper and more hyperchromatic nuclei than benign lesions. Mitotic figures usually do not exceed one per high power field. Malignant FH shows infiltrating edges with frequent areas of necrosis, with one or more histologic criteria of malignancy.
FHs are frequently confused with such lesions as solitary fibrous tumour, monophasic synovial sarcoma and haemangiopericytoma. Solitary fibrous tumour is composed mainly of spindle cells arranged randomly or in ill-defined fascicles. A characteristic feature is the presence of striking areas of hyalinisation. CD34 staining is found most commonly in solitary fibrous tumour and vimentin staining is almost characteristic of FH. Monophasic synovial sarcoma is composed of spindle-shaped cells arranged in a herringbone pattern. Histiocytic cells were also observed in our case along with spindle-shaped cells. Rarely, the presence of increased vascularity and dilated vascular channels is misinterpreted as hemangiopericytoma. In hemangio-pericytoma the predominant component is sinusoidal blood vessels which are arranged in a typical staghorn or antler-like configuration with focal spindle cell areas as the distinguishing feature.
In view of a huge, potentially malignant recurrent tumour and a non-seeing, mutilated eye, an exenteration was considered appropriate in our patient.
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[Figure - 1], [Figure - 2]