Nonpigmented conjunctival tumors constitute a broad, spectrum of ocular conditions, spanning benign to malignant, entities.1 Diagnosis of these tumors often hinges, on their clinical characteristics, and in many instances,, cytologic or histopathologic evaluation becomes indispensable, (see Chapters 5 and 6).2 The management, approach frequently depends on both the diagnosis and, the extent of invasion., While most conjunctival tumors have epithelial or melanocytic, origins, they also originate from vascular,, fibrous, neural, histiocytic, myogenic, myxoid, lipomatous,, and lymphoid components (Mind map 38-1). The, most commonly diagnosed malignant conjunctival cancers, are squamous carcinoma, malignant melanoma, and, lymphoma (Fig. 38-1)., Further, conjunctival tumors are also classified into, 3 categories: benign, premalignant, and malignant., It is noteworthy that the 8th edition of the AJCC Cancer, Staging System discourages the use of premalignant, classifications for both melanocytic and squamous, intraepithelial neoplasia, now classified as stage Tis (in, situ, see Chapter 8).3 Such complexities necessitate a, thorough and thoughtful chapter dedicated to the diagnosis, and management of nonpigmented conjunctival, tumors.
Chapter Keyword: dysplasia
Pigmented Conjunctival Tumors
Pigmentation of the conjunctiva is common and may be, benign or malignant. The spectrum of conjunctival pigmented, tumors is broad, ranging from benign acquired, melanosis and conjunctival nevi to more sinister variants, such as melanoma in situ and invasive conjunctival, melanoma.1,2, Conjunctival melanomas can originate spontaneously, from a pre-existing nevus or from primary acquired, melanosis (PAM).33 It is important to note that the 8th, edition AJCC Cancer Staging Manual replaced the term, “primary acquired melanosis with atypia” with “melanoma, in situ.” This was because the former was easily, mistaken for being benign and the latter was consistent, with the nonocular epithelial melanoma nomenclature.1, Further, the AJCC-OOTF advises using PAM only as a, clinical description, as biopsy remains essential for its, differentiation from conjunctival melanoma in situ., Epidemiology of conjunctival melanoma suggests it, most commonly presents in one eye in middle-aged, fairskinned, individuals.3,4 While conjunctival melanoma can, occur in all races, it must be differentiated from naturally, occurring, bilateral, lifelong pigment on the conjunctiva,, termed racial or congenital melanosis.5 Conjunctival, melanomas are rare, comprising approximately 2% of, all ocular tumors and 5% of ocular melanomas.6,7 Nevertheless,, its incidence is increasing and is associated with, a considerable tumor-related risk of mortality.8, However, several pilot studies now suggest that systemic, and advanced local conjunctival melanoma may be treatable, with immunotherapy.9-11, This chapter aims to provide an in-depth understanding, of the varying presentations of pigmented conjunctival, tumors, their differentiation, and appropriate management, (Mind map 39-1). Given the reported recurrence, rates of 12% to 50% after treatment and an overall incidence, of metastasis reaching 26%, there is need for, life-long follow-up and improved treatment options.12,13