Systemic associations of eyelid tumors

1 Overview of Eyelid Tumors Usha Singh and Raghavendra Rao Kolavali Overview and Epidemiology Eyelid is a common place for skin cancer to occur and constitute 5–10% of all skin cancers. Eyelid neoplasms comprise a variety of benign and malignant growths (Table 1.1). Significant majority of these growths are benign in nature and constitute 82–98% of all neoplasms (Table 1.2). There is wide, racial, and probable geographical variation reported in the incidence of the various eyelid tumors. Eyelid malignancies vary in distribution and presentation. The most common malignant eyelid tumor in western literature is basal cell carcinoma (BCC) comprising 86–91% incidence among the Caucasians [7, 12]. However, in one of the largest series from China and India this incidence is much lower, consequently sebaceous gland carcinoma (SGC) constitutes 32% of all eyelid tumors [6, 13]. In studies from Asian countries [2, 14, 15] it is the sebaceous gland carcinoma which constitutes the majority (67–77%). The mean age for benign tumor is lower than that of malignant tumors. U. Singh (*) Department of Ophthalmology, Advanced Eye Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India R. R. Kolavali Department of Ophthalmology, Post Graduate Institute of Medical Education and Research, Chandigarh, India © The Author(s) 2019 S. S. Chaugule et al. (eds.), Surgical Ophthalmic Oncology, https://doi.org/10.1007/978-3-030-18757-6_1 Epithelial tumor and dermoid cysts are the most common eyelid tumor in children [16]. Malignant eyelid tumor in children is extremely rare. When it presents, is usually a part of a systemic process, genetic defects or following radiation treatment [17, 18]. Merkel cell carcinomas (MCC) of the eyelid are rare neuroendocrine tumor constituting 5–20% of the head and neck tumor, predominantly in Caucasians [19]. Classification of Eyelid Tumors Eyelid tumors can arise from various histological layers eyelid is composed of. Eyelid tumors are classified as benign or malignant or according to the tissue or cell of origin (Tables 1.2, 1.3 and 1.4). They can be subdivided into non-melanocytic and melanocytic tumors. Benign epithelial proliferations such as squamous papilloma, pseudoepitheliomatous hyperplasia, seborrheic keratosis, keratoacanthoma cysts and nevi are common. Among the malignant, BCC (Figs. 1.1 and 1.2) is the most common in Caucasians and SGC among the Asians (Fig. 1.3), followed by squamous cell carcinoma (SCC) and malignant melanoma (MM) (Figs. 1.4, 1.5, 1.6, 1.7 and 1.8). The large majority of BCC (93%) was seen in 71% of females [2] SGC has predilection for the upper lid [20]. Merkel cell cancer has higher prevalence in men. Primary malignant melanomas of the eyelid skin are rare and account for 0.2–13% of all reported 3 4 Table 1.1 Regional incidence of benign and malignant eyelid neoplasms in the epidemiological studies Mean age years (gender preponderance) 227 (5) 72.5 12.5% 12 (10.2%) 178 (56.8) 39 (39) 1097 (31.2) 61 (f) 53.1 (f) 18 – – 894 (18) 206 (24.1) 390 (46) 594 (40) – >60 (m) 6% 100 22 (1.5%) 63.9 (m) 68.6 (m) 1995–2015 4521 4521 2. 3. Chang CH, Taiwan, 2003 [2] Toshida H, Japan, 2012 [3] 1994–1998 1993–2007 144 118 129 118 4. 5. 6. Sihota, India 1996 [4] Rathod A, India, 2015 [5] Ni Z, China, 1996 [6] 1982–1992 2007–2009 1953–1992 313 100 3510 1989–2007 2004–2007 1984–1989 1980–1982 2000–2010 2008–2012 7. 8. 9. 10. 11. 12. Western literature Deprez, Switzerland, 2009 [7] Paul S, USA, 2011 [8] Mclean, AFIP, USA, 1994 [9] Font, USA, 2006 [10] Middle East Bagheri, Tehran, Iran, 2013 [11] Gundogan FC, Turkey, 2015 [12] 4294 (95.0) 126 (87.5) 106 (89.8) 55.4 -(f) 47.8 (f) nil ns 313 100 3510 135 (43.1) 61 (61) 2413 (68.7) ns 37.02 na ns 1 4981 855 846 1474 4981 855 456 NOS 4087 (82) 649 (75.9) 456 (54) 880 (60) – <60 (nil) – 182 1502 (1541) 182 1541 82 1424 (92.4) 46.4 (f) 50.08 (f) ns 58.59 na – U. Singh and R. R. Kolavali Study period 1. Author, country, year Asian studies Huang, Taiwan, 2015 [1] Mean age years Premalignant Malignant (gender Total number of Biopsy proven (%) patients tumors Benign (%) preponderance) % 1 Overview of Eyelid Tumors 5 Table 1.2 Eyelid tumors originating from epidermis Subtypes Non-melanocytic Benign Squamous cell papilloma Seborrheic keratosis Premalignant Actinic(solar) keratosis Intraepithelial neoplasia Inverted follicular keratosis Sebaceous nevus (of Jadassohn) Xeroderma pigmentosa Reactive hyperplasia (pseudoepitheliomatous hyperplasia) Melanocytic Ephelis or freckles Lentigo simplex Keratoacanthoma Congenital dysplastic nevus Lentigo maligna (melanotic freckle of Hutchinson) Solar Lentigo Junctional nevus Intradermal nevus Compound nevus Spitz nevus Balloon cell nevus Blue nevus Cellular blue nevus Oculodermal nevus of Ota Eyelid tumors arising from adnexal and cystic lesions Sebaceous gland Sebaceous gland hyperplasia tumors Sebaceous gland adenoma Syringoma Sweat gland and lacrimal gland tumors Papillary syringadenoma Eccrine spiradenoma Eccrine acrospiroma Eyelid tumors arising from hair follicle Trichoepithelioma Trichofolliculoma/trichoadenoma Trichilemmoma Pilomatrixoma (calcifying epithelioma of Malherbe) Other cystic lesions Epidermal inclusion cyst Sebaceous cyst Retention cyst Eccrine hidrocystoma Apocrine hidrocystoma Trichilemmal cyst Other benign cystic lesion – Malignant Basal cell carcinoma Squamous cell carcinoma Mucoepidermoid carcinoma Keratoacanthoma Melanoma arising from nevi Melanoma arising in lentigo maligna Melanoma arising de novo Sebaceous gland carcinoma Sweat gland (eccrine) adenocarcinoma Mucinous sweat gland adenocarcinoma Apocrine gland adenocarcinoma Adenoid cystic carcinoma Porocarcinoma Carcinoma of hair follicles 6 U. Singh and R. R. Kolavali Table 1.3 Fibrous, fibrohistiocystic, and muscular eyelid tumors Fibrous Fibrous histiocytic Benign Fibroma Keloid Nodular fasciitis Proliferative fasciitis Fibromatosis Xanthelasma Xanthoma Intermediate Dermatofibroma Dermatofibrosarcoma protuberans Angiomatoid fibrous histiocytoma Xanthogranuloma Fibrous histiocytoma Juvenile xanthogranuloma Necrotic xanthogranuloma Reticulohistiocytoma Smooth muscle Skeletal muscle Atypical fibroxanthoma Benign Leiomyoma Angiomyoma Rhabdomyoma Malignant Fibrosarcoma Congenital fibrosarcoma Malignant fibrous histiocytoma Malignant giant cell fibrous histiocytoma Malignant fibroxanthoma Malignant Leiomyosarcoma Rhabdomyosarcoma Table 1.4 Eyelid tumors arising from vascular, perivascular, neural, lipomatous, cartilage, bone lymphoid tumors, hamartomas, and choristomas Vascular Perivascular Neural Lipomatous Cartilage Lymphoid Hamartomas and choristomas Others Benign Nevus flammeus (port wine stain) Papillary endothelial hyperplasia Capillary hemangioma Cavernous hemangioma Venous hemangioma Epithelioid hemangioma (angiolymphoid hyperplasia) Arteriovenous malformation Lymphangioma Hemangiopericytoma Glomus tumor Traumatic neuroma Neurofibroma Plexiform neurofibroma Schwannoma (neurilemoma) Neuroglial choristoma Lipoma Hibernoma Chondroma Osteoma Benign lymphoid hyperplasia Plasmacytoma Dermoid cyst Phakomatous choristoma Ectopic lacrimal gland Myxoma Malignant Angiosarcoma Lymphangiosarcoma Kaposi’s sarcoma Malignant hemangiopericytoma Malignant glomus tumor Malignant peripheral nerve sheath tumor Merkel cell tumor Liposarcoma Chondrosarcoma Mesenchymal chondrosarcoma Osteosarcoma Lymphoma Leukemic infiltration 1 Overview of Eyelid Tumors 7 a b c d Fig. 1.1 (a) Basal cell carcinoma involving the lower lid. (b) Ulcerative basal cell carcinoma involving the medial canthus. (c) Basal cell carcinoma involving the upper lid with central necrotic area. (d) Morpheaform type of basal cell carcinoma involving the lower lid Fig. 1.2 Extensive basal cell carcinoma involving both medial canthi, nose and cheek Fig. 1.3 Sebaceous gland carcinoma misdiagnosed as chalazion and surgically intervened 8 U. Singh and R. R. Kolavali Fig. 1.4 Malignant melanoma involving lower lid and conjunctiva Fig. 1.5 Extensive malignant melanoma involving both the eyelids in a patient with xeroderma pigmentosa a cases [2, 7]. They occur 20 years later than other non-melanoma tumor and have 2.6 times predilection for the lower lid. Eyelids can also be involved by secondary and metastatic lesions. All primary carcinomas of the eyelid can be classified based on their clinical and histological presentation using the TNM [tumor, nodes (lymph), metastasis] by AJCC (8th Ed) classification system [21]. TNM staging describes the size of tumor, number and location of regional lymph nodes which have malignant cells in them and whether the malignant cells have spread or metastasized to another part of the body. The TNM classification of eyelid carcinomas reflects both morbidity and mortality risks in order to provide useful guidelines for patient management. Fig. 1.6 Rapidly growing squamous cell carcinoma of the eyelid and extending to the orbit b Fig. 1.7 (a) Kissing nevus in a young adolescent girl. (b) Nevi involving the upper lid in a young adult with a history of recent growth. (c) Nevus involving the lid margin in a young adult. (d) Keratoacanthoma 1 Overview of Eyelid Tumors 9 c d Fig. 1.7 (continued) a b c Fig. 1.8 (a) Squamous papilloma of the lower eyelid. (b) Lymphangioma diffusely involving the lids and orbit. (c) Extensive Xanthelesma involving all four lids 10 References 1. Huang YY, Liang WY, Tsai CC, Kao SC, Yu WK, Kau HC, et al. 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