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There are 2 types of primary glaucoma—primary open-    TABLE 18.1: Tumours and Tumour-like Lesions of the Eye  511
           angle (chronic simple glaucoma) and primary angle-closure  and Adnexal Structures.
           (acute congestive glaucoma). Primary open-angle glaucoma
           is more common type and is usually a genetically-determined  Benign               Malignant
           disease. Primary angle-closure glaucoma occurs due to  I. EYELID
           shallow anterior chamber and hence narrow angle causing    Squamous cell papilloma  Squamous cell carcinoma
           blockage to aqueous outflow.                               Basal cell papilloma   Basal cell carcinoma
              In all types of glaucoma, degenerative changes appear   Sebaceous adenoma      Sebaceous adenocarcinoma
           after some duration and eventually damage to the optic nerve  Naevi               Malignant melanoma
           and retina occurs.                                    II. CONJUNCTIVA-CORNEA
                                                                      Squamous cell papilloma  Squamous cell carcinoma
           PAPILLOEDEMA. Papilloedema is oedema of the optic disc     Pseudoepitheliomatous  Mucoepidermoid carcinoma
           resulting from increased intracranial pressure. This is due  hyperplasia
           to anatomic continuation of the subarachnoid space of the  Naevi                  Malignant melanoma
           brain around the optic nerve so that raised intracranial   Haemangioma
                                                                 III. LACRIMAL GLAND
           pressure is passed onto the optic disc area. In  acute
           papilloedema, there is oedema, congestion and haemorrhage  Pleomorphic adenoma    Carcinoma in pleomorphic
                                                                                             adenoma
           at the optic disc. In chronic papilloedema, there is degene-  IV. ORBIT
           ration of nerve fibres, gliosis and optic atrophy.
                                                                      Glioma                 Malignant glioma
           SJÖGREN’S SYNDROME. Sjögren’s syndrome is charac-          Inflammatory ‘pseudotumour’  Malignant lymphoma
           terised by triad of keratoconjunctivitis sicca, xerostomia (sicca  Meningioma     Rhabdomyosarcoma
           syndrome) and rheumatoid arthritis. The condition occurs  V. INTRAOCULAR
           due to immunologically-mediated destruction of the lacrimal  Naevi                Malignant melanoma
           and salivary glands along- with another autoimmune disease  Neurofibroma          Retinoblastoma
           (Chapter 4).
                                                               is seen more commonly in the upper eyelid (basal cell
           MIKULICZ’S SYNDROME.  This is characterised by
           inflammatory enlargement of lacrimal and salivary glands  carcinoma is seen more frequently in the lower eyelid).  CHAPTER 18
           (Chapter 19). The condition may occur with Sjögren’s  MORPHOLOGIC FEATURES. Grossly, the tumour
           syndrome, or with some diseases like sarcoidosis, leukaemia,  appears as a localised or diffuse swelling of the tarsus, or
           lymphoma and macroglobulinaemia.                      may be in the form of ulcerated or papillomatous tumour
                                                                 at the lid margin.
           TUMOURS AND TUMOUR-LIKE LESIONS                       Microscopically, the tumour may show well-differen-
           The eye and its adnexal structures are the site of a variety of  tiated lobules of tumour cells with sebaceous differen-
           benign and malignant tumours as well as tumour-like lesions.  tiation, or may be poorly-differentiated tumour requiring
           A brief list of such lesions is given in  Table 18.1. The  confirmation by fat stains (Fig. 18.5). These tumours can
           morphology of many of these tumours and tumour-like
           lesions is identical to similar lesions elsewhere in the body.                                             The Eye, ENT and Neck
           However, a few examples peculiar to the eye are described
           below.

           Inflammatory Pseudotumours
           These are a group of inflammatory enlargements, especially
           in the orbit, which clinically look like tumours but surgical
           exploration and pathologic examination fail to reveal any
           evidence of neoplasm.
            MORPHOLOGIC FEATURES. Grossly, these lesions are
            circumscribed and sometimes have fibrous capsule.
            Microscopically, many of the lesions can be placed in well-
            established categories such as tuberculous, syphilitic,
            mycotic, parasitic, foreign-body granuloma etc, while
            others show non-specific histologic appearance having
            abundant fibrous tissue, lymphoid follicles and
            inflammatory infiltrate with prominence of eosinophils.

           Sebaceous Carcinoma
           This is the most frequent tumour of the eyelid next only to basal
           cell carcinoma, although it is very rare tumour elsewhere in  Figure 18.5  Sebaceous carcinoma. The tumour cells are arranged
                                                               as lobules with peripheral basaloid cells and pale cells in the centre. The
           the body. It arises either from the meibomian glands in the  cells have sebaceous differentiation appreciated by foamy, vacuolated
           tarsus or from Zeis’ glands of eyelash follicles. The tumour  cytoplasm.
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