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
Archives: Chapters
Treatment of Eyelid Tumors
The treatment of tumors of the eyelid is both complex, and fascinating. The choice of treatment depends upon, the type of tumor (benign, malignant, specific diagnosis),, size, location (upper or lower eyelid, canthus involvement,, deeper involvement to ocular surface or orbit), the, age and systemic health of the patient, and the surgeon’s, preference (see Chapter 2).1, For appropriate management, the basic oncological principles, must be followed:
- Adequate tissue biopsy for diagnosis.,
- Assessment of tumor margins.,
- Evaluation for local and systemic spread.
In Chapter 36 we discuss the clinical features of common, eyelid tumors. Herein we discuss the management,, including surgery, radiation, cryotherapy, PDT, and, chemotherapy (Mind Map 37-1). We also provide a broad, overview of reconstructive techniques following excision, and the prognosis of common eyelid malignancies.
Orbital Neoplasms: Past, Present, and Future
This chapter contains an overview of the evolution of, diagnosis and treatments for orbital tumours.1-4 This leads, to a chapter that contains a rational format summarizing, the pertinent themes of diagnosis and treatment utilizing, present-day information. Lastly, I probe and forecast, our likely future.
Approach to Diagnosis of Orbital Tumors
The orbit and ocular adnexa are susceptible to several, primary and secondary diseases affecting various tissue, types such as osseous, vascular, neural, muscular, and, glandular structures (see Chapters 44 and 45).1-3 These, include primary tumors, infections, and inflammations,, as well as secondary disease from the periorbital paranasal, sinuses, eyelids, and brain., Given the intricate anatomy of the orbit, it is no wonder, that most primary and secondary orbital diseases share, the common symptoms of proptosis, strabismus, and, optic nerve compression. Clearly, diagnosis of orbital, pathologies requires a meticulous approach, leading to, an assessment of urgency and often tissue diagnosis., A comprehensive assessment begins with a thorough, ophthalmic and systemic history, considering the chief, complaint(s), major ophthalmic symptoms, their onset,, and duration (see Chapter 3). This information, coupled, with a meticulous ophthalmic examination and, radiologic imaging, typically forms the bedrock for establishing, a presumptive diagnosis and course of medical or, surgical management.
Diagnosis of Eyelid Tumors
The eyelid is the protective shield and the cosmetic, charm of the human eye (see Chapter 2). It consists of, layers of tissue, each of which can give rise to benign and, malignant tumors. This chapter outlines the epidemiology,, differentiating features, and diagnosis of the most, common benign and malignant eyelid tumors (Mind, maps 36-1 to 36-7)., Eyelid cancer constitutes 5%–10% of all skin cancers, the, 4 most common being BCC, SCC, SGCa, and melanoma, (Fig. 36-1). It is important to note that nearly 82%–98%, of all eyelid tumors are benign.1-3 For example, squamous, papilloma, seborrheic keratosis, keratoacanthoma, and, nevi are common benign epithelial tumors of the eyelid., Benign tumors are also more prevalent in the younger, age groups, e.g., dermoid and epithelial tumors comprise, the majority of childhood adnexal tumors.1 Most eyelid, tumors are visible and easily recognized early by the, patient or the parent., Challenges to diagnosis and management include:,
- Older patients often ignore and delay seeking medical, attention.,
- Involvement of the lid margin and palpebral conjunctiva, go unnoticed.,
- Malignant lesions can simulate benign conditions.,
- The eyelid structures are in proximity with the orbit,, allowing contiguous extension.,
- The lymphatic and vascular supply of the head and, neck allow potential routes of metastasis.,
- Extensive surgery with wide margins can result in, unacceptable functional and cosmetic results.
Eyelid tumors can be classified based on tissue of origin, as benign or malignant (Table 36-1). However, in, clinic it is helpful to divide them into melanocytic or, nonmelanocytic (Mind maps 36-1, 36-2). AJCC staging, provides prognostic TNM information that assists, patient management.1,3
Diagnosis and Management of Benign Orbital Tumors
A majority of orbital lesions are benign.1 The orbit is, a bony structure with one major anterior opening and, several more posteriorly located fissures through which, important nerves and blood vessels travel into and out, of the orbit (see Chapter 2). Therefore, various space-occupying, lesions tend to present with a varying mixture, of similar signs and symptoms of globe displacement,, eyelid swelling, blepharoptosis, limitation in eye movement,, conjunctival chemosis and hyperemia, elevated, intraocular pressure, chorioretinal folds, and optic, nerve compression as well as other cranial neuropathies., 1 Generally, benign orbital lesions tend to be more, slow-growing and, therefore, present with a more gradual, onset or may sometimes even be asymptomatic., Compared with malignant lesions, on imaging, benign, lesions tend to be more well circumscribed, causing bone, remodeling rather than erosion. However, these general, rules have exceptions, and not all lesions can be classified, into benign or malignant based on clinical presentation, and imaging findings. The topics of orbital tumor classification, and differential diagnosis are covered elsewhere, in this book (see Chapter 42). This chapter focuses on the, diagnosis and specific management of common benign, orbital lesions.
Differentiation of Systemic Phakomatoses
Phakomatoses are hereditary, multisystem disorders, characterized by the presence of lesions or tumors of the, skin, eye, and central nervous system (CNS). While their, systemic manifestations and genetic underpinnings are, constant, it is clear that the ophthalmologist can make, the initial diagnosis to help preserve patients’ quality, of life.1-3 This chapter highlights some of the common, genetic and clinical characteristics of phakomatoses, as, well as some of the more interesting associations most, likely seen by the eye care specialist.
Nonpigmented Conjunctival Tumors
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.
Soft Tissue Tumors of the Orbit
Space-occupying lesions and tumors in the orbit may, stem from any normal, aberrant, or heterotopic orbital, tissue. When infectious and inflammatory processes are, excluded, a large variety of orbital tumors remain to be, classified according to their epidemiological aspects,, clinical features, radiological characteristics, biological, behavior, or clinicopathological origins (Mind-map, 40-1). This chapter covers congenital tumors, including, dermoid and epidermoid cysts, dermolipoma, as well as, orbital fibrous, osseous, and cartilaginous tumors and, other rare soft tissue tumors, together with their systemic, implications.
Diagnosis and Management of Malignant Orbital Tumors
Given the confined space of the bony orbit, any neoplasm, of the orbit can present with a varying mixture of similar, signs and symptoms of globe displacement, eyelid, swelling, blepharoptosis, limitation in eye movement,, conjunctival chemosis, hyperemia, elevated intraocular, pressure, chorioretinal folds, and optic nerve compression, as well as other cranial neuropathies.1 Malignant, tumors grow rapidly, with symptoms developing over, months to weeks. Due to the rapid growth and tendency, to involve sensory nerves, pain is a more common (but, not necessary) feature of a malignant orbital tumor.2 On, imaging, malignant lesions tend to be infiltrative, not, respecting the natural boundaries of anatomic compartments, and causing bone erosion and destruction rather, than remodeling. These general rules have exceptions., Not all lesions can be classified into benign or malignant, based on clinical presentation and imaging findings;, therefore, most will require tissue biopsy.3 Treatment, is multidisciplinary, with oncology, radiation oncology,, and other specialties as needed. Staging orbital tumors, with the most recent AJCC TNM classification ensures, improved multidisciplinary communication and patient, care.4 The topics of orbital tumor classification and differential, diagnosis are covered elsewhere in this book (see, Chapters 42 and 43). In the current chapter, we focus, on the diagnosis and specific management of common, malignant orbital neoplasms.