Malignant orbital diseases in adults

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As with all cancers, orbital malignancy is more common in middle age and in the elderly and may be either primary disease arising in the orbit, secondary to spread from the sinuses, or metastatic from remote sites.

Lacrimal gland carcinoma

Adenoid cystic carcinoma, with a peak incidence in the fourth decade, is the commonest malignancy of the lacrimal gland and accounts for 30% of all epithelial tumours. Other, much rarer, carcinomas include primary adenocarcinoma, muco-epidermoid carcinoma, squamous carcinoma and malignant mixed tumours; malignant mixed tumours arising either within a longstanding pleomorphic adenoma, or within recurrent pleomorphic adenoma after previously incomplete resection.

The diagnosis of lacrimal carcinoma is suggested by an unrelenting, relatively rapid progression of ocular displacement and upper eyelid swelling caused by a non-tender lacrimal gland mass; such patients will often present with a history of less than 6 months (Figure 13.4a). The rapid progression and pain may suggest acute dacryoadenitis, but failure of any inflammatory lesion of the lacrimal gland to clearly improve within a few weeks should prompt further investigation for malignancy.

CT scan shows expansion of the lacrimal gland by a mass that typically moulds to the globe and extends posteriorly alongside the lateral orbital wall, displacing the lateral rectus medially (Figure 13.4b). In more advanced cases there may be erosive changes in the bone of the lacrimal gland fossa, best shown with bone-window settings. Calcification, usually a few flecks, may be present in up to one-third of malignant lacrimal gland tumours.

The tumour should be biopsied through the upper lid skin crease (Chapter 12). Adenoid cystic carcinoma is composed of solid cords of malignant epithelial cells, often with cystic spaces giving a "Swiss cheese" pattern. The tumour infiltrates well beyond the macroscopic boundaries evident at surgery and on radiological imaging, and adenoid cystic carcinoma has a propensity for perineural spread into the cavernous sinus and pterygo-palatine fossa.

The best method of treatment remains controversial, because recurrences of adenoid cystic carcinoma may be very slowly progressive and can become manifest at more than 10 years after primary treatment; it is likely, therefore, that a "cure" can only be countenanced after 20 years without recurrence. These patients probably do best with removal of the tumor bulk (which can often be almost complete) through an anterior orbitotomy and subsequently they should receive 55-60cGy of radiotherapy to both the orbit and the cavernous sinus (Figure 13.4c). Unless frankly invaded by tumour, the cortical bone of the lateral orbital wall should be left undisturbed as any breach is likely to encourage seeding of tumour cells into the cranial diploe, with a slow and relentless fatal recurrence of local disease (Figure 13.4d). Implantation brachytherapy has been

Lacrimal Sac Tumor

Figure 13.4 (a) A patient with left lacrimal gland carcinoma, (b) such tumours typically producing enlarged lacrimal gland moulding to the globe and extending posteriorly alongside the lateral orbital wall, (c) treatment with debulking and fractionated high-dose radiotherapy to the orbit and cavernous sinus produces good disease control with a satisfactory cosmesis, (d) craniofacial resection may encourage seeding of the tumour into the cranial diploe, with subsequent slow and relentless fatal local recurrence.

Figure 13.4 (a) A patient with left lacrimal gland carcinoma, (b) such tumours typically producing enlarged lacrimal gland moulding to the globe and extending posteriorly alongside the lateral orbital wall, (c) treatment with debulking and fractionated high-dose radiotherapy to the orbit and cavernous sinus produces good disease control with a satisfactory cosmesis, (d) craniofacial resection may encourage seeding of the tumour into the cranial diploe, with subsequent slow and relentless fatal local recurrence.

advocated as delivering a high radiation dosage to the tumour bed whilst relatively sparing the globe, but it does not, however, treat the cavernous sinus or pterygo-palatine fossa - to which these tumours often extend by perineural invasion.

There is no reliable evidence to suggest that either exenteration or "super-exenteration" (with removal of the neighbouring orbital bones) leads to a reduction of recurrence rate or a prolongation of life; such procedures are associated with a marked disfigurement in relatively young people. Intra-carotid chemotherapy may have a role as an adjunct to radiotherapy in advanced disease.

Dry eye is inevitable with removal of the lacrimal gland and radiotherapy; frequent topical lubricants, together with occlusion of the lacrimal drainage puncta, are often required. Radiation-induced cataract is common at about two years after treatment.

Tumour recurrence occurs relatively late with adenoid cystic carcinoma and the relatively young person may have many years of useful, symptom free life before regrowth of tumour is evident. Unfortunately most patients will eventually suffer a painful and relentlessly progressive local recurrence of the disease (Figure 13.4d), with a high mortality from intracranial recurrence or metastasis.

Other mesenchymal tumours

Sarcomas of the orbit are extremely rare.The highly malignant osteosarcoma is generally secondary to childhood orbital radiotherapy for retinoblastoma; even with radical clearance, the tumour is almost uniformly fatal by two years after diagnosis. Children may present with metastatic Ewing's sarcoma or Wilm's tumour within the orbit and will require systemic therapy after diagnosis.

Fibrosarcoma is a malignant tumour of fibroblasts, which can arise as a primary lesion (Figure 13.5) or as a secondary tumour from adjacent sinuses or at the site of prior radiotherapy. Wide excision, often with exenteration, is recommended and palliation with radiotherapy and chemotherapy may be beneficial. The prognosis for vision and life is variable, but is best for primary juvenile fibrosarcomas.

Figure 13.5 Fibrosarcoma recurrence at one year after incomplete tumour resection.

Several rare orbital tumours present with a spectrum of disease, from benign to malignant. Malignant fibrous histiocytoma arises from fascia, muscle or other soft tissues and typically presents as a well-defined mass in the supero-nasal quadrant of the orbit. Recurrence of these radio-resistant and chemo-resistant tumours is common, even after wide excision, and the tumour is associated with a significant mortality. Haemangiopericytoma, likewise, has a spectrum of malignancy and should be treated by wide and, if possible, intact resection.

Leiomyosarcoma, a tumour of smooth muscle, and liposarcomas of various degrees of differentiation have been reported to very rarely involve the orbit.

Orbital lymphoma in adults

Lymphocyte recruiting lesions of the orbit display a spectrum from a benign morphology, showing well-organised follicular pattern (so-called "reactive lymphoid hyperplasia"), through the rare "atypical lymphoid hyperplasia" with poorly organised or disrupted follicular formation, to frank malignant lymphoma. With the advent of histochemical stains specific for various particular types of lymphocytes, their precursors and the follicle dendritic cells, it has become evident in recent years that many lesions labelled "atypical lymphoid hyperplasia" are, in fact, lymphomas displaying various degrees of disorganised follicle formation or destruction.

Orbital lymphomas, about 10% of orbital masses, are almost exclusively non-Hodgkin's B-cell lymphomas, the tumours containing a minority of reactive T lymphocytes and a variable degree of follicular organisation. The few reported T-cell lymphomas involving the orbit are in patients with systemic disease, and advanced Burkitt's lymphoma may spread from the neighbouring sinuses. Dependent on the grade of lymphoma, up to about a half of patients presenting with orbital disease will be found to have systemic involvement within six months of presentation.

Orbital lymphomas present as a slowly progressive, painless subconjunctival "salmon patch" (Figure 13.6) or, if deeper in the orbit, with swelling of the eyelids, globe displacement or diplopia. The typical age of presentation is over 50, although younger individuals may develop the more aggressive, high-grade lymphomas.

CT scan typically shows a moderately well defined soft tissue mass moulding around the globe and affecting - but often not destroying the form of - multiple tissues (such as the lacrimal gland or extraocular muscles) (Figure 13.7); bilateral disease is present in about 25% of cases. Calcification in the lesion and changes in the orbital bones are very unusual, although rarely a longstanding lesion may

Figure 13.6 Typical conjunctival lymphoma.

'salmon-patch" mass of

Figure 13.6 Typical conjunctival lymphoma.

'salmon-patch" mass of

Figure 13.7 Lymphoma of the lacrimal gland: (a) moulding around the globe and extending into the surrounding fat and (b) levator/superior rectus muscle complex.

dependent on structural analysis, open biopsy is recommended as it provides a solid piece of tissue with minimal disruption of structure. Fine needle aspiration biopsy will only provide disrupted tissue, with no indication of form. All patients with lymphoid lesions of the orbit should undergo investigation for systemic disease, this including whole-body CT scan and bone-marrow biopsy where the lymphoma is of higher grade (for example, with follicle-centre lymphoma or diffuse large B-cell lymphoma).

Low-grade lymphomas involving the orbit respond well to about 2400cGy fractionated radiotherapy (Figure 13.8), although a slowly progressive lesion in a very frail or moribund patient may be observed. Widespread low-grade systemic disease generally responds well to oral chemotherapy.

Patients with high-grade lymphomas have, however, a much higher chance of systemic

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