Orbital Malignant Peripheral Nerve Sheath Tumours
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Br J Ophthalmol: first published as 10.1136/bjo.73.9.731 on 1 September 1989. Downloaded from British Journal of Ophthalmology, 1989, 73, 731-738 Orbital malignant peripheral nerve sheath tumours CHRISTOPHER J LYONS,' ALAN A McNAB,l ALEC GARNER,2 AND JOHN E WRIGHT' From the I Orbital clinic, Moorfields Eye Hospital, City Road, London EC] V 2PD, and the 2Department of Pathology, Institute ofOphthalmology, London EC] V 9A T SUMMARY We describe three patients with malignant peripheral nerve tumours in the orbit and review the existing literature on these rare lesions. Malignant peripheral nerve sheath tumours are Sensation was diminished over the distribution of the unusual in any part of the body and very rare in the second division of the right trigeminal nerve. The left orbit, where only 13 cases have previously been globe was normal. Plain anteroposterior skull x-rays described. These tumours can spread rapidly along showed a normal appearance, but undertilted the involved nerve to the middle cranial fossa. They occipitomental tomographic views revealed enlarge- are radioresistant, and total surgical excision offers ment of the right infraorbital canal (Fig. 1). copyright. the only hope of cure. Our experience with three An inferior orbital margin incision revealed patients may help clinicians to recognise these lesions tumour protruding from the infraorbital foramen and and excise them at an early stage. extending beneath the soft tissues of the cheek. The tumour had a firm consistency and a pale grey cut Case reports surface. The orbital periosteum on the floor of the orbit was elevated and a mass over 18 mm in diameter PATIENT 1 was found within an expanded infraorbital canal Four years prior to presentation a man which extended posteriorly into the superior orbital 55-year-old http://bjo.bmj.com/ noted a small lump at the medial end of his right fissure. Biopsy specimens were taken. lower eyelid. This was removed through the palpe- bral conjunctiva but recurred within a month. A Histology histopathological report was not obtained. The tumour was composed of a homogeneous and Over the next two years three further attempts densely packed proliferation of spindle shaped cells were made to remove this lump surgically with either aligned in more or less parallel bundles and sheets. In incomplete removal or early recurrence on each places there was a fascicular pattern with palisading occasion. After three years the lump had extended of nuclei, characteristic of Antoni A morphology of a on September 24, 2021 by guest. Protected into the floor of the orbit, and a further attempt at schwannoma (Fig. 2a, b). Looser myxoid areas of excision was made, by a transantral approach. Antoni B differentiation were not seen. However, Histopathological examination on these occasions small clear spaces or vacuoles were common, and in showed the features of 'a neurofibroma with some instances the tumour cells were grouped round schwannian transformation in certain areas'. There such spaces to create a pseudoglandular appearance. was no evidence of malignancy. Despite tumour-free The reaction for S-100 protein was strongly positive, resection margins the mass recurred in the floor of the virtually all the cells being stained (Fig. 2c). The orbit. tumour cells had prominent and sometimes multiple At presentation the right vision was reduced to nucleoli, were frequently hyperchromatic, and perception of hand movements. The globe was mitotic figures could occasionally be recognised. proptosed 5 mm and displaced inferiorly 6 mm and Scattered giant nuclei were also present. There was laterally 9 mm. There was a marked right relative no clearly defined capsule, and tumour cells afferent pupillary defect with disc swelling, and appeared to be infiltrating the adjacent orbital con- movement of the eye was severely restricted. nective tissue. Some haemosiderin resulting from previous haemorrhage was also seen. Correspondence to Mr C J Lyons, FRCS. Transmission electron microscopy of formalin 731 Br J Ophthalmol: first published as 10.1136/bjo.73.9.731 on 1 September 1989. Downloaded from 732 ChristopherJ Lyons, Alan A McNab, Alec Garner, andJohn E Wright copyright. Fig. 1 Patient 1. An undertilted occipitomental tomogram shows enlargement ofthe infraorbital canal caused by tumour (arrow). fixed tissue revealed cells with grooved or indented left eye and dilated conjunctival blood vessels for nuclei and conspicuous nucleoli. Individual cells eight months. Two months after the onset of these were sometimes linked by complexes resembling symptoms he had noticed that the left globe was desmosomes, and there was incomplete basal lamina protuberant, and a week before presentation his formation surrounding the cytoplasmic membranes vision had deteriorated. http://bjo.bmj.com/ of occasional cells (Fig. 3). Although rare collagen At his first attendance his right visual acuity was 6/5 fibres presented exceptionally prominent cross- while the left corrected acuity was 6/24. There was a striations, long-spacing fibres were not identified. A left relative afferent pupillary defect, and the left prominent rough-surfaced endoplasmic reticulum globe was proptosed 8 mm axially. Ocular move- was observed in a minority of cells. ments were restricted in all directions, especially The patient was referred for neuroradiological and elevation. Facial and corneal sensation was normal. neurosurgical opinion to determine if the tumour was The left conjunctiva and episclera were markedly on September 24, 2021 by guest. Protected resectable. The presence of a large mass in the hypervascular, but there was no pulsation nor any posterior fossa, continuous with the mass in the audible bruit. The disc was swollen. orbital floor, was confirmed. A CT scan (Fig. 4) showed a large, well defined, By this time, the patient had developed severe rounded and homogeneous mass situated superiorly paroxysms of lancinating electric shock-like pain within the intraconal space. This enhanced with involving the whole of the right side of his face and contrast. An intra-arterial digital subtraction angio- numbness affecting the left side of his body. He had gram showed a brisk tumour circulation with early also noticed voice changes and difficulty in swallow- filling by contrast (Fig. 5). The provisional diagnosis ing, and he complained of poor balance and difficulty was haemangiopericytoma. in walking, with a tendency to fall to the right. At lateral orbitotomy a large firm tumour lying Palliative radiotherapy was given. The patient died intraconally was dissected free of adjacent structures within four months. and found to extend to the superior orbital fissure, where it was transacted and its stump subjected PATIENT 2 to diathermy. Postoperatively the visual acuity A 42-year-old man had noticed discomfort round the remained unchanged. One year after the operation, Br J Ophthalmol: first published as 10.1136/bjo.73.9.731 on 1 September 1989. Downloaded from Orbital malignantperipheral nerve sheath tumours 733 nv- ~~rK~Dry-- :.7 Fig. 2 Patient 1. (a) Cells with elongated, wavy nuclei showing a tendency to a palisade alignment; the histology ofthe tumour recalls the Antoni A structure typical of schwannoma. (Haematoxylin and eosin, x 125.) (b) In places the tumour cells have abnormally large, hyperchromatic and pleomorphic nuclei and are suggestive ofmalignancy. (Haematoxylin and eosin, x313.) (c) There is a conspicuous staining reaction for S-I00 protein. (Immunoperoxidase, x 125.) copyright. http://bjo.bmj.com/ there was no evidence of local recurrence or distant Electron microscopy of a separate piece of the metastasis. tumour failed to provide any evidence of basal lamina formation round the cells or of long-spacing collagen, on September 24, 2021 by guest. Protected Histology though the cytoplasmic processes of the cells showed Examination of the tumour revealed a highly cellular considerable interdigitation. neoplasm with a predominance of spindle cell forms. In many areas the cells were grouped in fascicles with PATIENT 3 a rudimentary attempt at a palisade arrangement A 21-year-old man had noticed a progressively (Fig. 6a). Other cells were vacuolated, and there was enlarging painless lump in the medial part of his left widespread focal lymphocytic infiltration. Lymphoid upper eyelid for five years. Two previous attempts at tissue was also prominent in the compressed connec- surgical excision had failed. The visual acuity was 6/6 tive tissue which partly encompassed the tumour. in each eye. The left globe was proptosed by 6 mm Some cells had unusually large, pleomorphic, and and displaced laterally by 8 mm. A large mass of soft, hyperchromatic nuclei, and many had conspicuous almost fluctuant consistency occupied both inner nucleoli, while occasional mitotic figures were also quadrants of the left orbit. There were two surgical seen (Fig. 6b). Staining for S-100 protein was scars overlying the mass in the upper lid. CT scans observed in scattered tumour cells (Fig. 6c). A few (Fig. 7) revealed an extensive soft tissue mass myelinated nerve bundles were present in the tissue extending from the skin anteriorly to the posterior surrounding the tumour. limit of the orbit. The orbit was enlarged, and the Br J Ophthalmol: first published as 10.1136/bjo.73.9.731 on 1 September 1989. Downloaded from 734 ChristopherJ Lyons, Alan A McNab, Alec Garner, andJohn E Wright copyright. Fi.3Paino.XO Trasiso elcrnM&X.irgrp Fig. 3 Patient 1. Transmission electron micrograph showingpart ofa cell with an indented nucleus andprominent rough endoplasmic reticulum. There is a distinct basal lamina next to the cell surface. (Uranyl acetatellead citrate, x 13300). mass displaced the medial rectus, optic nerve, and hyperchromatic and, exceptionally, multiple nuclei globe laterally. The medial wall of the orbit was and readily observed nucleoli (Fig. 8b). A positive http://bjo.bmj.com/ intact. staining response for S-100 protein was obtained in At anterior orbitotomy a large tumour was respect of only a very few cells (Fig. 8c), though the adherent to the orbital septum and trochlea, and the technique served to demonstrate occasional small optic nerve posteriorly.