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Overview of Eyelid Tumors

  • Usha Singh
  • Raghavendra Rao Kolavali
Open Access
Chapter

Abstract

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. Significant majority of these growths are benign in nature and constitute 82–98% of all neoplasms. 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 (Deprez and Uffer, Am J Dermatopathol 31:256–62, 2009; Gundogan et al., Asian Pac J Cancer Prev 16:4265–9, 2015). However, in one of the largest series from China and India where this incidence is much lower, consequently sebaceous gland carcinoma (SGC) constitutes 32% of all eyelid tumors (Ni, Zhonghua Yan KeZaZhi 32:435–7, 1996; Kale et al., Indian J Plast Surg 45:22–8, 2012). In studies from Asian countries (Prabha et al., Sch J App Med Sci 3:2165–8, 2015; Ho et al., Hong Kong Med J 19:150–5, 2013; Chang et al., Kaohsiung J Med Sci 19:549–54, 2003), 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. Epithelial tumor and dermoid cysts are the most common eyelid tumor in children (Hsu and Lin, Ophthalmologica 218:274–7, 2004). 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 (Al-Buloushi et al., Eye 19:1313–4, 2005; Nerad and Whitaker, Ophthalmology 106:723–9, 1988). Merkel cell carcinomas (MCC) of the eyelid are rare neuroendocrine tumor constituting 5–20% of the head and neck tumor, predominantly in Caucasians (Lemos et al., J Am Acad Dermatol 63:751–61, 2010).

Keywords

Cancer in the eyelid Eyelid cancer Basal cell carcinoma in the eyelid Sebaceous gland carcinoma in eyelids Squamous cell carcinoma of the eyelid Kissing nevus in the eyelid Squamous papilloma of the lower eyelid Xanthelesma in eyelids 

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. 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].
Table 1.1

Regional incidence of benign and malignant eyelid neoplasms in the epidemiological studies

 

Author, country, year

Study period

Total number of patients

Biopsy proven tumors

Benign (%)

Mean age years (gender preponderance)

Premalignant %

Malignant (%)

Mean age years (gender preponderance)

 

Asian studies

1.

Huang, Taiwan, 2015 [1]

1995–2015

4521

4521

4294 (95.0)

55.4

 

227 (5)

72.5

2.

Chang CH, Taiwan, 2003 [2]

1994–1998

144

129

126 (87.5)

-(f)

nil

12.5%

61 (f)

3.

Toshida H, Japan, 2012 [3]

1993–2007

118

118

106 (89.8)

47.8 (f)

ns

12 (10.2%)

53.1 (f)

4.

Sihota, India 1996 [4]

1982–1992

313

313

135 (43.1)

ns

ns

178 (56.8)

ns

5.

Rathod A, India, 2015 [5]

2007–2009

100

100

61 (61)

37.02

1

39 (39)

58.59

6.

Ni Z, China, 1996 [6]

1953–1992

3510

3510

2413 (68.7)

na

 

1097 (31.2)

na

 

Western literature

7.

Deprez, Switzerland, 2009 [7]

1989–2007

4981

4981

4087 (82)

 

894 (18)

8.

Paul S, USA, 2011 [8]

2004–2007

855

855

649 (75.9)

<60 (nil)

18

206 (24.1)

>60 (m)

9.

Mclean, AFIP, USA, 1994 [9]

1984–1989

846

456

456 (54)

390 (46)

 

10.

Font, USA, 2006 [10]

1980–1982

1474

NOS

880 (60)

 

594 (40)

 

Middle East

11.

Bagheri, Tehran, Iran, 2013 [11]

2000–2010

182

182

82

46.4 (f)

 

100

63.9 (m)

12.

Gundogan FC, Turkey, 2015 [12]

2008–2012

1502 (1541)

1541

1424 (92.4)

50.08 (f)

6%

22 (1.5%)

68.6 (m)

Table 1.2

Eyelid tumors originating from epidermis

Subtypes

Benign

Premalignant

Malignant

Non-melanocytic

Squamous cell papilloma

Actinic(solar) keratosis

Basal cell carcinoma

Seborrheic keratosis

Intraepithelial neoplasia

Squamous cell carcinoma

Inverted follicular keratosis

Sebaceous nevus (of Jadassohn)

Mucoepidermoid carcinoma

Reactive hyperplasia (pseudoepitheliomatous hyperplasia)

Xeroderma pigmentosa

Keratoacanthoma

 

Keratoacanthoma

Melanocytic

Ephelis or freckles

Congenital dysplastic nevus

 

Lentigo simplex

Lentigo maligna (melanotic freckle of Hutchinson)

Melanoma arising from nevi

Solar Lentigo

Melanoma arising in lentigo maligna

Junctional nevus

Melanoma arising de novo

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 tumors

Sebaceous gland hyperplasia

Sebaceous gland carcinoma

Sebaceous gland adenoma

Sweat gland and lacrimal gland tumors

Syringoma

Sweat gland (eccrine) adenocarcinoma

Papillary syringadenoma

Mucinous sweat gland adenocarcinoma

Eccrine spiradenoma

Apocrine gland adenocarcinoma

Eccrine acrospiroma

Adenoid cystic carcinoma

Porocarcinoma

Eyelid tumors arising from hair follicle

 

Trichoepithelioma

 

Carcinoma of hair follicles

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

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 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.
Table 1.3

Fibrous, fibrohistiocystic, and muscular eyelid tumors

 

Benign

Intermediate

Malignant

Fibrous

Fibroma

 

Fibrosarcoma

Keloid

Congenital fibrosarcoma

Nodular fasciitis

Proliferative fasciitis

Fibromatosis

Fibrous histiocytic

Xanthelasma

Malignant fibrous histiocytoma

Xanthoma

Atypical fibroxanthoma

Malignant giant cell fibrous histiocytoma

Dermatofibroma

Dermatofibrosarcoma protuberans

Malignant fibroxanthoma

Xanthogranuloma

Angiomatoid fibrous histiocytoma

Fibrous histiocytoma

Juvenile xanthogranuloma

Necrotic xanthogranuloma

Reticulohistiocytoma

 

Benign

Malignant

Smooth muscle

Leiomyoma

Leiomyosarcoma

Angiomyoma

Skeletal muscle

Rhabdomyoma

Rhabdomyosarcoma

Table 1.4

Eyelid tumors arising from vascular, perivascular, neural, lipomatous, cartilage, bone lymphoid tumors, hamartomas, and choristomas

 

Benign

Malignant

Vascular

Nevus flammeus (port wine stain)

Angiosarcoma

Papillary endothelial hyperplasia

Lymphangiosarcoma

Capillary hemangioma

Kaposi’s sarcoma

Cavernous hemangioma

Venous hemangioma

Epithelioid hemangioma (angiolymphoid hyperplasia)

Arteriovenous malformation

Lymphangioma

Perivascular

Hemangiopericytoma

Malignant hemangiopericytoma

Glomus tumor

Malignant glomus tumor

Neural

Traumatic neuroma

Malignant peripheral nerve sheath tumor

Neurofibroma

Merkel cell tumor

Plexiform neurofibroma

Schwannoma (neurilemoma)

Neuroglial choristoma

Lipomatous

Lipoma

Liposarcoma

Hibernoma

Cartilage

Chondroma

Chondrosarcoma

Osteoma

Mesenchymal chondrosarcoma

Osteosarcoma

Lymphoid

Benign lymphoid hyperplasia

Lymphoma

Plasmacytoma

Leukemic infiltration

Hamartomas and choristomas

Dermoid cyst

 

Phakomatous choristoma

Ectopic lacrimal gland

Others

Myxoma

 
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

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

Fig. 1.6

Rapidly growing squamous cell carcinoma of the eyelid and extending to the orbit

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

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

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.

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Authors and Affiliations

  • Usha Singh
    • 1
  • Raghavendra Rao Kolavali
    • 2
  1. 1.Department of OphthalmologyAdvanced Eye Center, Post Graduate Institute of Medical Education and ResearchChandigarhIndia
  2. 2.Department of OphthalmologyPost Graduate Institute of Medical Education and ResearchChandigarhIndia

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