|Year : 1988 | Volume
| Issue : 3 | Page : 116-119
Malignant fibrous histiocytoma of the orbit
SM Betharia, Roopa Arora, Sushil Kumar
R.P. Centre for Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi-110 029, India
S M Betharia
R.P. Centre for Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi-110 029
Source of Support: None, Conflict of Interest: None
Two cases of malignant fibrous histiocytoma of the orbit are reported. Histopathological examination of the orbital biopsy specimen obtained after orbitotomy in one case and exenteration in another showed pleomorphic neoplastic cells arranged in storiform pattern. The tumour cells were composed of hyperchromatic nuclei with prominent nucleoli and admixtures of fibroblasts. Postoperative radiotherapy and chemotherapy were given. There was no evidence of recurrence two years later.
|How to cite this article:|
Betharia S M, Arora R, Kumar S. Malignant fibrous histiocytoma of the orbit. Indian J Ophthalmol 1988;36:116-9
| Introduction|| |
Kaufman and Stout  first described histiocytic tumors in children where the tumours with large contents of fibrous tissue were called as fibrous xanthomas and those with little fibrous tissue were designated as histocytomas. The concept of the tissue cell possessing the pleuri-potential, tissue forming properties as seen with histiocytes was relatively new at that time. Ozzello  later on performed tissue culture studies of these tumour variants and found that with the passage of time the cells took on the features of a fibroblast. Stout and Lattes  in an effort to standardize the profuse and diverse nomenclature proposed a classification in which the fibrous histocytoma was used as the rubric for several benign and malignant tumors such as fibroxanthoma, dermatofibroma, sclerosing haemangioma, storiform fibrous xanthoma, epithelial sarcoma and others. O'Brien and Stout  first described this tumour with orbital involvement. Russman  reported another case with primary orbital involvement. Zimmerman  commented on the histopathology of orbital fibrous histiocytoma but did not include any case protocols. Isolated cases with orbital involvement were reported by Vogel , Jakobie and Jones , Rodrigues et al , Biedner and Rothkoff  as well as 6 cases by Jokobiec  et al. In this communication we are reporting clinical and histopathological observations in 2 cases.
| Case Reports|| |
Case 1: A 19 years old male presented with a painless and progressive protrusion of the left eye ball of 6 months duration.
The visual acuity in both the eyes was 6/6. On exophthalmometry a proptosis of 9 mm was noted in the left eye [Figure - 1]. There was no limitation of ocular movements and the proptosis was irreducible. Fundus examination revealed an early papilloedema.
Routine investigations were within normal limits. X-ray left orbit showed haziness whereas x-ray skull, optic formina and chest were normal. Ultrasonography showed a fairly well defined mass with echoes of high attenuation value and low internal reflectivity in the central space. CAT Scan revealed a well circumscribed oval mass with smooth outline in the central space which was obscuring the optic nerve. There was no intracranial extension. Lateral orbitotomy was performed [Figure - 2] and the tumour mass was removed. microscopic. examination revealed a well encapsulated mass of firm consistency measuring 5 ems x 4 cms x 4 cms. On sectioning, the mass was found to he compact in nature. It was sent for histopathological examination. Patient was later sent for radiotherapy and chemotherapy.
Case 2 : A 16 years old male presented with gradual progressive painless protrusion of the left eye for the last 6 months. There was no history of diplopia, trauma or headache. On examination the right eye was normal. The visual acuity in the left eye was 6/24 when he first. came to the casualty. There was a soft tissue mass in the left lower orbital region pushing the globe up and nasally. [Figure - 3]. Ocular movements were restricted in all directions. Pupil of the left eye was sluggishly reacting. Fundus examination of the left eye revealed papilloedema.
Investigations included ultrasonography, x-ray orbit and CAT scan X-ray orbit showed soft tissue swelling and CAT scan revealed a soft tissue mass filling the entire orbital region but there was no intracranial extension. Ultrasonography showed low amplitude spikes in a diffuse ill defined lesion with poor sound transmission. On the fourth day of performing a biopsy the tumour mass suddenly showed an increase in size upto 7" x 7" with continous bleeding. Patient was given 15 units of whole blood. Finally a complete exenteration was performed [Figure - 4]. The mass which was irregular and friable was sent for histopathological examination. Postoperatively the patient was given radiation therapy of 5000 rads over 5 weeks.
Microscopic examination of the tumours showed extreme cellular variability but constituted principally of spindle shaped fibroblasts arranged in a storiform pattern. These cells displayed oval or elongated hyperchromatic nuclei, prominent nucleoli and increased mitotic activity. The other types of cells seen were monoculear histiocytes and large bizzare tumour giant cells which were multinucleated with eosinophilic cytoplasm. These findings were suggestive of pleomorphic variety of. malignant fibrous histiocytoma. However, there was no area of necrosis seen See [Figure - 5][Figure - 6].
| Discussion|| |
The fibrohistocytic tumours which exhibit either histiocytic pattern or fibroblastic pattern or more commonly a mixed composition, usually arise in the muscles and deep facia. They can be either benign or malignant. Those tumours which are present superficially, are usually small in size, whereas those arising in the deeper plane are large in size. They can have diverse locations like extremities, nose, paranasal sinuses, orbit, larynx, meninges, lungs, vagina, ovary and submandibular glands. These tumours comprise about 10 to 20% of all sarcomas. 
These classification of fibrous histiocytomas prepared by WHO  includes several benign and malignant soft tissue tumours see [Table 1]. This group of tumours shows considerable variation in cytological features, from spindle cell patterns, characteristic of fibroblast like cells to epithelioid sarcoma, usually involving the extremities
Though these tumours are described in the 5th to 7th decade of life and more commonly in males, our patients were in the second decade. The malignant fibrous histiocytomas have four histological types namely myxoid, inflammatory, pleomorphic and angiomatoid varieties. The myxoid variety has a better prognosis whereas inflammatory type is more aggressive in its course. The angiomatoid type is the least commonly seen.
Fu and associates  described the electron microscopic feature and tissue cultures of four malignant fibrous histiocytomas "involving the thigh, palm, groin and anterior pectoral region. Tissue culture revealed that foamy vacuolated cells were the major constituent. Others have proposed that the fibroblast like cells are most likely derived from histiocytes and are facultative fibroblasts. In one of our patients the tumour was located in the deep posterior part of the orbit whereas in 3 of the 5 benign orbital histiocytomas Jakobiec and associates noted that the lesions were located in the upper inner orbit.
Although the management of fibrous histiocytoma is surgical , radiation therapy has been used as an adjunct to surgery. Soule and Enriquez  reported 65% five year survival for malignant fibrous histiocytoma and 38% ten year survival using a combined surgical, radiation and chemotherapeutic approach. Our patients appeared healthy without any evidence of local recurrence or systemic metastasis after two years of follow up.
The differential diagnosis of malignant fibrous histiocytoma includes leiomyosarcoma, angiosarcoma, neurofibrosarcoma and pleomorphic liposarcoma. Our patients showed no evidence of atypical proliferation of smooth muscle, blood vessels, nerves or adipose tissue. Malignant fibrous histiocytoma can be differentiated from its benign counterpart by the presence of cellular pleomorphism, infiltration of tumour margins and mitotic activity. Kempson and Kyriakos [l5] noted that large tumours (more than 5 to 8 cms in diameter) with infiltrating margins had a greater tendency for recurrence and metastasis than tumours less than 3 cm in diameter. Smaller tumour size indicates a better prognosis. However. careful follow up examinations for possible metastasis are essential.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]