Ординатура / Офтальмология / Английские материалы / Clinical Ophthalmology A Systematic Approach 7th Edition_Kanski, Bowling_2011

kanski 7th

Fig. 1.18 Xanthelasma. (A) Histology shows foamy histiocytes within the dermis; (B) clinical appearance – note corneal arcus

(Courtesy of J Harry – fig. A; M Zatouroff, from Physical Signs in General Medicine, Mosby-Wolfe, 1996 – fig. B)

Neurofibroma

Plexiform neurofibromas typically affect children who also have neurofibromatosis type I (NF1). Solitary neurofibromas typically occur in adults, 25% of whom have NF1.

1 Histology shows proliferation of Schwann cells, fibroblasts and nerve axons (Fig. 1.19A).

2Signs. The tumour typically affects the upper lid and gives rise to a characteristic S-shaped deformity (Fig. 1.19B).

3Treatment of solitary lesions involves simple excision but removal of plexiform lesions may be difficult especially if they are diffuse.

29 / 1137

kanski 7th

Fig. 1.19 Neurofibroma. (A) Histology shows proliferation of Schwann cells, fibroblasts and nerve axons, and wavy collagen fibres; (B) characteristic S-shaped eyelid

(Courtesy of J Harry – fig. A)

Copyright © 2011 Elsevier Inc.All rights reserved. Read our Terms and Conditions of Use and our PrivacyPolicy.

If you find this useful please saythanks in your way: dramroo

30 / 1137

kanski 7th

Malignant tumours

Rare predisposing conditions

Young patients who suffer from one of the following conditions may develop eyelid malignancies:

1Xeroderma pigmentosum is an AR disease characterized by skin damage on exposure to natural sunlight, which gives rise to progressive cutaneous pigmentation abnormalities (Fig. 1.20A). Affected patients have a bird-like facies and a great propensity to the development of basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and melanoma, which may be multiple.

Conjunctival malignancies have also been reported.

2Gorlin–Goltz syndrome (naevoid basal cell carcinoma syndrome) is a rare AD disorder characterized by extensive congenital deformities of the eye, face, bone and CNS. Many patients develop multiple small BCC during the 2nd decade of life (Fig. 1.20B) and are also predisposed to medulloblastoma, breast carcinoma and Hodgkin lymphoma.

3Muir–Torre syndrome is a rare AD condition that predisposes to cutaneous and internal malignancies. Cutaneous tumours include BCC, sebaceous gland carcinoma (SGC) and keratoacanthoma. Colorectal and genitourinary carcinoma is the most common systemic tumour.

4Bazex syndrome is an XLD condition characterized by eczematous and psoriasiform lesions associated with carcinomas of the upper respiratory and digestive tracts. Eyelid BCC may also occur.

5Other predispositions include immunosuppression, prior retinoblastoma and albinism.

Fig. 1.20 Predispositions to eyelid malignancies. (A) Xeroderma pigmentosum; (B) Gorlin–Goltz syndrome

(Courtesy of J Krachmer, M Mannis and E Holland, from Cornea, Mosby 2005 – fig. B)

Basal cell carcinoma

General features

BCC is the most common human malignancy and most frequently affects elderly patients. The most important risk factors are fair skin, inability to tan and chronic exposure to sunlight. Ninety per cent of cases occur in the head and neck and about 10% of these involve the

31 / 1137

kanski 7th

eyelid. BCC is by far the most common malignant eyelid tumour, accounting for 90% of all cases. It most frequently arises from the lower eyelid, followed in relative frequency by the medial canthus, upper eyelid and lateral canthus. The tumour is slow-growing and locally invasive but non-metastasizing. Tumours located near the medial canthus are more prone to invade the orbit and sinuses, are more difficult to manage than those arising elsewhere and carry the greatest risk of recurrence. Tumours that recur following incomplete treatment tend to be more aggressive.

Histology

The tumour arises from the cells that form the basal layer of the epidermis. The cells proliferate downwards (Fig. 1.21A) and characteristically exhibit palisading at the periphery of a tumour lobule of cells (Fig. 1.21B). Squamous differentiation with the production of keratin results in a hyperkeratotic type of BCC. There can also be sebaceous and adenoid differentiation while the growth of elongated strands and islands of cells embedded in a dense fibrous stroma results in a sclerosing (morphoeic) type of tumour.

Fig. 1.21 Basal cell carcinoma. (A) Histology shows downward proliferation of lobules of basal (purple) cells; (B) histology shows palisading of cells at the periphery of a tumour lobule; (C) nodular tumour; (D) rodent ulcer; (E) large rodent ulcer; (F) sclerosing tumour

(Courtesy of J Harry – figs A and B)

Clinical types

The main clinical features of epidermal cell malignancy are ulceration, lack of tenderness, induration, irregular borders and destruction of lid margin architecture.

1Nodular BCC is a shiny, firm, pearly nodule with small dilated blood vessels on its surface. Initially, growth is slow and it may take the tumour 1–2 years to reach a diameter of 0.5 cm (Fig. 1.21C).

2Noduloulcerative BCC (rodent ulcer) has central ulceration, pearly raised rolled edges and dilated and irregular blood vessels (telangiectasis) over its lateral margins (Fig. 1.21D); with time it may erode a large portion of the eyelid (Fig. 1.21E).

3Sclerosing BCC (morphoeic) is less common and may be difficult to diagnose because it infiltrates laterally beneath the epidermis as an indurated plaque (Fig. 1.21F). The margins of the tumour may be impossible to delineate clinically and the lesion tends to be much more extensive on palpation than inspection. On cursory examination a sclerosing BCC may simulate a localized area of chronic blepharitis.

4Other types not usually found on the lid are cystic, adenoid, pigmented and multiple superficial.

Squamous cell carcinoma

General features

SCC is a much less common, but typically more aggressive tumour than BCC with metastasis to regional lymph nodes in about 20% of cases. Careful surveillance of regional lymph nodes is therefore an important aspect of initial management. The tumour may also exhibit perineural spread to the intracranial cavity via the orbit. SCC accounts for 5–10% of eyelid malignancies and may arise de novo or from pre-

32 / 1137

kanski 7th

existing actinic keratosis or carcinoma in situ (Bowen disease, intraepidermal carcinoma; Fig. 1.22). Immunocompromised patients such as those with AIDS or following renal transplants are at increased risk. The tumour has a predilection for the lower eyelid and the lid margin. It occurs most commonly in elderly individuals with a fair complexion and a history of chronic sun exposure. The diagnosis of SCC may be difficult because certain ostensibly benign lesions such as keratoacanthoma and cutaneous horn may reveal histological evidence of invasive SCC at deeper levels of sectioning.

Fig. 1.22 Carcinoma in situ. (A) Histology shows dysplastic changes throughout the thickness of the epidermis, together with hyperkeratosis; (B) red scaling plaque

(Courtesy of L Horton – fig. A; H Frank – fig. B)

Histology

The tumour arises from the squamous cell layer of the epidermis. It is composed of variable-sized groups of atypical epithelial cells with prominent nuclei and abundant eosinophilic cytoplasm within the dermis (Fig. 1.23A). Well-differentiated tumours may show characteristic keratin ‘pearls’ and intercellular bridges (desmosomes).

33 / 1137

kanski 7th

Fig. 1.23 Squamous cell carcinoma. (A) Histology shows acanthotic squamous epitheliumand eosinophilic (pink) islands of dysplastic squamous epitheliumwithin the dermis; (B) nodular tumour with surface keratosis; (C) ulcerating tumour; (D) cutaneous horn

(Courtesy of L Horton – fig. A; A Singh, from Clinical Ophthalmic Oncology, Saunders 2007 – fig. B; H Frank – fig. C; S Farley, T Cole and L Rimmer – fig. D)

Clinical types

The clinical types are variable and there are no pathognomonic characteristics. The tumour may be indistinguishable clinically from a BCC but surface vascularization is usually absent, growth is more rapid and hyperkeratosis is more often present.

1Nodular SCC is characterized by a hyperkeratotic nodule which may develop crusting erosions and fissures (Fig. 1.23B).

2Ulcerating SCC has a red base and sharply defined, indurated and everted borders but pearly margins and telangiectasia are not usually present (Fig. 1.23C).

3Cutaneous horn with underlying invasive SCC (Fig. 1.23D).

Keratoacanthoma

Keratoacanthoma is a rare tumour that usually occurs in fair-skinned individuals with a history of chronic sun exposure. It is found more frequently than would be expected by chance in patients on immunosuppressive therapy. Histopathologically, keratoacanthoma is regarded as part of the spectrum of SCC.

1Histology shows irregular thickened epidermis surrounded by acanthotic squamous epithelium. The sharp transition from the thickened to normal adjacent epidermis is referred to as shoulder formation (Fig. 1.24A); a keratin-filled crater may be seen.

2Signs in chronological order:

•A pink, rapidly growing, hyperkeratotic lesion, often on the lower lid (Fig. 1.24B), which may double or treble in size within weeks (Fig. 1.24C).

•Growth ceases for 2–3 months, after which spontaneous involution occurs.

•During the period of regression a keratin-filled crater may develop (Fig. 1.24D).

•Complete involution may take up to a year and usually leaves an unsightly scar.

3Treatment involves complete surgical excision. Other options include radiotherapy, cryotherapy and topical or intralesional 5- fluorouracil.

34 / 1137

kanski 7th

Fig. 1.24 Keratoacanthoma. (A) Histology shows irregularly thickened eosinophilic epidermis with a keratin-containing cup and well-marked shoulder formation; (B) hyperkeratotic nodule; (C) large tumour; (D) keratin-filled crater during involution

Sebaceous gland carcinoma

General features

Sebaceous gland carcinoma (SGC) is a very rare slowly-growing tumour which most frequently affects the elderly, with a predisposition for females. It usually arises from the meibomian glands, although on occasion it may arise from the glands of Zeis or from sebaceous glands in the caruncle. In contrast to BCC and SCC, the tumour occurs more commonly on the upper eyelid where meibomian glands are more numerous. In about 5% of cases there is simultaneous involvement of both lids on one side, probably due to intraepithelial spread or to spontaneous development of multiple primaries. The clinical diagnosis of SGC is frequently difficult because, in its early stages, external signs of malignancy may be subtle so that the tumour may resemble a chalazion or blepharitis. However, the presence of yellowish material within the tumour is highly suggestive of SGC. Because of frequent difficulties in diagnosis and delay in treatment, the overall mortality rate is about 5–10%. Adverse prognostic features include upper lid involvement, tumour size of 10 mm or more and duration of symptoms of over 6 months. SGC that arises from the glands of Zeis is thought to have a more favourable prognosis.

Histology

The tumour consists of lobules of cells with pale foamy vacuolated lipid-containing cytoplasm and large hyperchromatic nuclei (Fig. 1.25A).

35 / 1137

kanski 7th

Fig. 1.25 Sebaceous gland carcinoma. (A) Histology shows cells with large hyperchromatic nuclei and vacuolated cytoplasm; (B) nodular tumour; (C) spreading tumour; (D) pagetoid spread

(Courtesy of A Garner – fig. A; A Singh, from Clinical Ophthalmic Oncology, Saunders 2007 – fig. B; S Tuft – fig. C; H Frank fig. D)

Clinical types

Although SGC does not have a characteristic clinical appearance it may present with the following:

1Nodular SGC presents as a discrete, hard nodule, most commonly within the upper tarsal plate, that may exhibit yellow discoloration due to the presence of lipid (Fig. 1.25B). Because the lesion may masquerade as a chalazion, it is recommended that any ‘chalazion’ of an unusual consistency should undergo full-thickness resection and histological examination.

2Spreading SGC infiltrates into the dermis and causes a diffuse thickening of the lid margin (Fig. 1.25C) that may result in loss of lashes and be mistaken for ‘chronic blepharitis’. Occasionally the tumour may exhibit multifocal non-contiguous origins.

3Pagetoid spread refers to extension of the tumour within epithelium including the palpebral (Fig. 1.25D), forniceal or bulbar conjunctiva. This may lead to the mistaken diagnosis of an inflammatory condition.

Lentigo maligna and melanoma

Melanoma rarely develops on the eyelids but is potentially lethal. Although pigmentation is a hallmark of skin melanomas, half of lid melanomas are non-pigmented and this may give rise to diagnostic difficulties. Features suggestive of melanoma include recent onset of a pigmented lesion, change in an existing pigmented lesion, irregular margins, asymmetrical shape, colour change or presence of multiple colours, and diameter greater than 6 mm in diameter.

Lentigo maligna

Lentigo maligna (melanoma in situ, intraepidermal melanoma and Hutchinson freckle) is an uncommon condition that develops in sundamaged skin in elderly individuals. Malignant change may occur, with infiltration of the dermis.

1Histology shows intraepidermal proliferation of spindle-shaped atypical melanocytes that replace the basal layer of the epidermis (Fig. 1.26A).

2Signs

•A slowly expanding pigmented macule with an irregular border (Fig. 1.26B).

•Nodular thickening and areas of irregular pigmentation are highly suggestive of malignant transformation (Fig. 1.26C).

3Treatment is usually by excision.

36 / 1137

kanski 7th

Fig. 1.26 Lentigo maligna. (A) Histology shows melanoma cells proliferating within the basal layers of the epidermis; (B) lentigo maligna; (C) melanoma arising from lentigo maligna

(Courtesy of L Horton – fig. A; S Delva – figs B and C)

Melanoma

1Histology shows large atypical melanocytes within the dermis (Fig. 1.27A).

2Signs

aSuperficial spreading melanoma is characterized by a plaque with an irregular outline and variable pigmentation (Fig. 1.27B).

bNodular melanoma is typically a blue-black nodule surrounded by normal skin (Fig. 1.27C).

3Treatment is usually by wide excision and may include local lymph node removal.

37 / 1137

kanski 7th

Fig. 1.27 Melanoma. (A) Histology shows melanoma cells within the dermis; (B) superficial spreading melanoma; (C) nodular melanoma

(Courtesy of J Harry – fig. A)

Merkel cell carcinoma

Merkel cell carcinoma is a fast-growing tumour which typically affects the elderly. Although Merkel cells lie within the epidermis, the tumour appears to arise from the dermis. Its rarity may lead to difficulty in diagnosis and delay in treatment. The tumour is highly malignant and 50% of patients have metastatic spread at presentation.

1Histology shows sheets of cells with scanty cytoplasm, round or oval nuclei and numerous mitotic figures (Fig. 1.28A).

2 Signs. A violaceous, well-demarcated nodule with intact overlying skin, most frequently involving the upper eyelid (Fig. 1.28B). 3 Treatment is by excision, frequently combined with chemotherapy.

38 / 1137