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

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Fig. 11.81 Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome. (A) Circinate pattern of hard exudates surrounding the disc. There is also venous irregularity and obscuration of the optic nerve head; (B) FA shows multiple aneurysms at arteriolar bifurcations and marked variation in arteriolar calibre

(Courtesy of J Donald Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. A; RF Spaide, from Diseases of the Retina and Vitreous, WB Saunders 1999 – fig. B)

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Chapter 12 – Ocular Tumours

BENIGN EPIBULBAR TUMOURS 476 Conjunctival naevus 476 Conjunctival papilloma 476 Dermoid 476

Dermolipoma 479 Pyogenic granuloma 480

Conjunctival epithelial melanosis  480

Miscellaneous tumours 481

MALIGNANT AND PREMALIGNANT EPIBULBAR TUMOURS 481 Primary acquired melanosis 481

Melanoma 482

Ocular surface squamous neoplasia  483

Lymphoproliferative lesions 486 Kaposi sarcoma 486

IRIS TUMOURS 486 Iris naevus 486 Iris melanoma 486

Metastatic tumours 488 Miscellaneous tumours  488

IRIS CYSTS 488

Primary 488

Secondary  492

CILIARY BODY TUMOURS 492 Ciliary body melanoma  492 Medulloepithelioma 495

TUMOURS OF THE CHOROID 496 Choroidal naevus 496 Choroidal melanoma 496

Circumscribed choroidal haemangioma  504

Diffuse choroidal haemangioma 504 Optic disc melanocytoma 504 Choroidal osteoma 506

Metastatic tumours 506

NEURAL RETINAL TUMOURS 510 Retinoblastoma

 510

Astrocytoma 517

VASCULAR RETINAL TUMOURS 520 Capillary haemangioma 520 Cavernous haemangioma

 523

Racemose haemangioma  523

Vasoproliferative tumour 524

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PRIMARY INTRAOCULAR LYMPHOMA 525

TUMOURS OF THE RETINAL PIGMENT EPITHELIUM 527 Typical congenital hypertrophy of the RPE 527 Atypical congenital hypertrophy of the RPE

 527

Combined hamartoma of the retina and RPE  527

Congenital hamartoma of the RPE 528

PARANEOPLASTIC SYNDROMES 529

Bilateral diffuse uveal melanocytic proliferation  529

Cancer-associated retinopathy 531 Melanoma-associated retinopathy 531

Benign epibulbar tumours

Conjunctival naevus

A conjunctival naevus is the most common melanocytic conjunctival tumour. The overall risk of malignant transformation is about 1%.

1Histology is similar to cutaneous naevi except that there is no dermis so that subepithelial and stromal replaces dermal in the nomenclature.

aJunctional naevi are uncommon and are characterized by nests of naevus cells at the epithelial–subepithelial junction (Fig. 12.1A).

bCompound naevi are characterized by the presence of naevus cells at the epithelial–subepithelial junction and within the subepithelial stroma, with downward proliferation of surface epithelium containing goblet cells (Fig. 12.1B).

cSubepithelial lesions are confined entirely beneath the epithelium.

2Presentation is usually during the 1st–2nd decades.

3Signs

•Solitary, unilateral, discrete, slightly elevated, pigmented intraepithelial bulbar lesion of variable size, most frequently in the juxtalimbal area (Fig. 12.2C).

•The extent of pigmentation is variable and some may be virtually non-pigmented (Fig. 12.2D).

•Cystic spaces within the naevus are frequent (Fig. 12.2E).

•The second most frequent locations are the plica and caruncle (Fig. 12.2F).

•In children and adolescents the lesion may become pink and congested.

4Signs of potential malignancy

•An unusual site such as palpebral or forniceal conjunctiva.

•Prominent feeder vessels.

•Sudden growth or increase in pigmentation.

•Development after the 2nd decade.

5Treatment by excision is indicated mainly for cosmetic reasons. Less common indications include irritation and suspicion of malignant transformation.

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Fig. 12.1 Conjunctival naevus. (A) Histology of a junctional naevus shows nests of naevus cells at the epithelial/subepithelial junction; (B) histology of a compound naevus shows naevus cells at the epithelial/subepithelial junction and within the stroma, and downward proliferation of surface epitheliumcontaining goblet cells (evident as clear spaces); (C) pigmented juxtalimbal naevus; (D) lightly pigmented juxtalimbal naevus; (E) naevus with cystic spaces; (F) naevus involving the caruncle

(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A; J Harry – fig. B)

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Fig. 12.2 Conjunctival papilloma. (A) Histology shows irregular proliferation of stratified squamous epitheliumcontaining goblet cells, overlying a fibrovascular core;

(B) small juxtalimbal and forniceal papillomas; (C) papillomas involving the plica and caruncle; (D) confluent papillomas; (E) large multiple papillomas interfering with eyelid closure; (F) large sessile papilloma encroaching onto the cornea

(Courtesy of J Harry – fig. A; U Raina – fig. B; R Bates – figs D and E)

Conjunctival papilloma

Conjunctival papillomas in childhood are caused by infection with human papillomavirus (types 6, 11 and 16) by mother-to-infant transmission at birth through an infected vagina. Adult papillomas are not infectious.

1Histology shows a fibrovascular core covered by irregular proliferation of non-keratinized, stratified squamous epithelium containing goblet cells (Fig. 12.2A).

2Signs

•Sessile or pedunculated lesions most frequently located in the juxtalimbal area, fornix (Fig. 12.2B) or the caruncle (Fig. 12.2C).

•The lesions are usually solitary but may be multiple and occasionally bilateral, and may become confluent (Fig. 12.2D).

•Large pedunculated lesions may cause irritation and interfere with lid closure (Fig. 12.2E).

•Occasionally they may involve a large area and encroach onto the cornea (Fig. 12.2F).

3Treatment of small lesions may not be required because they often resolve spontaneously. Large lesions are treated by excision and cryotherapy to the base and surrounding area. Treatment options for recurrences include subconjunctival alpha-interferon, carbon dioxide laser vaporization, topical mitomycin C and oral cimetidine (Tagamet).

Dermoid

Diagnosis

1Histology shows a solid mass of collagenous tissue containing dermal elements covered by stratified squamous epithelium (Fig. 12.3A).

2 Presentation is in early childhood.

3Signs

•Smooth, soft, yellowish, subconjunctival masses most frequently located at the inferotemporal limbus and showing protruding hair (Fig. 12.3B).

•Occasionally the lesions are very large and may virtually encircle the limbus (complex dermoid – Fig. 12.3C).

4Treatment is indicated for cosmetic reasons, chronic irritation, dellen formation and amblyopia from astigmatism or involvement of

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the visual axis. Small lesions can be excised although large lesions may require lamellar keratosclerectomy.

Fig. 12.3 Dermoid. (A) Histology shows a solid mass of collagenous tissue containing dermal elements and covered by stratified squamous epithelium; (B) typical dermoid with protruding hair; (C) complex choristoma; (D) dermoids in a patient with Goldenhar syndrome

(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A)

Systemic associations

Systemic associations include Goldenhar syndrome (see below), and less commonly Treacher Collins syndrome (see Ch. 1) and naevus sebaceus of Jadassohn (see below).

1Goldenhar syndrome (oculoauriculovertebral spectrum) is usually sporadic. It is thought that hemifacial microsomia and Goldenhar syndrome are part of the same spectrum of anomaly.

aSystemic features

•Hypoplasia of the malar, maxillary and mandibular regions (Fig. 12.3D).

•Macrostomia and microtia.

•Preauricular and facial skin tags.

•Some patients manifest severe asymmetrical facial clefting.

•Hemivertebrae, usually cervical.

•Incidence of mental handicap increases with presence of microphthalmos.

•Other features include cardiac, renal and CNS anomalies.

bOcular features, apart from dermoids, include upper lid notching or coloboma (Fig. 3.15D), microphthalmos and disc coloboma.

2Linear naevus sebaceus of Jadassohn

aSystemic features, apart from dermoids, include warty or scaly cutaneous lesions, infantile spasms, CNS anomalies and developmental delay.

bOcular features, apart from dermoids, include ptosis, cloudy cornea, lid colobomas, fundus colobomas and microphthalmos.

Dermolipoma

1 Histology is similar to solid dermoids but also shows fatty tissue. 2 Presentation is in adult life although the lesion is congenital.

3Signs. Soft, movable, yellowish-tan subconjunctival mass located near the outer canthus (Fig. 12.4A). The surface is usually keratinized and may have hairs, reflecting its origin from ectopic skin. Occasionally the lesion may extend into the orbit or anteriorly towards the limbus.

4Treatment should be avoided because surgery may be complicated by scarring, ptosis, dry eye and ocular motility problems. However, if they are particularly unsightly, then debulking the anterior portion may improve cosmesis without compromising ocular

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motility.

5Differential diagnosis

•Orbital fat prolapse, which has a normal conjunctival surface. It is also more mobile and softer (Fig. 12.4B) and can be reposited into the orbit with pressure.

•Orbital lobe of the lacrimal gland and lymphoma.

Fig. 12.4 (A) Dermolipoma; (B) orbital fat prolapse for comparison

(Courtesy of A Pearson)

Pyogenic granuloma

1Pathogenesis. A pyogenic granuloma is a fibrovascular proliferation in response to a tissue insult involving the conjunctiva such as surgery, trauma and less frequently inflammation. Spontaneous lesions are rare.

2Histology shows granulation tissue, chronic inflammation and proliferation of small blood vessels, similar to a cutaneous pyogenic granuloma. The term pyogenic granuloma is a misnomer because the lesion is neither pyogenic nor granulomatous.

3Presentation is a few weeks after surgery for chalazion, strabismus or enucleation.

4Signs. A fast-growing, pink, fleshy, vascularized conjunctival mass near the conjunctival wound (Fig. 12.5A and B) that bleeds easily.

5Treatment with topical steroids is usually successful; resistant cases require excision.

6 Differential diagnosis includes suture granuloma, vascular tumour, and Tenon granuloma or cyst.

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Fig. 12.5 Pyogenic granuloma. (A) Following excision of pterygium; (B) following removal of a meibomian cyst

Conjunctival epithelial melanosis

Conjunctival (racial) epithelial melanosis is a benign condition due to increased melanin production. It is often seen in dark-skinned individuals. Both eyes are affected but the intensity may be asymmetrical.

1 Presentation is during the first few years of life. The melanosis becomes static by early adulthood.

2Signs

•Areas of flat, patchy, brownish pigmentation scattered throughout the conjunctiva but more intense at the limbus (Fig. 12.6A).

•The lesions may be more intense around the perforating branches of the anterior ciliary vessels or around an intrascleral nerve as it enters the sclera (Axenfeld loop – Fig. 12.6B).

•With the slit lamp the pigmentation is seen to be within the epithelium and therefore moves freely over the surface of the globe.

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Fig. 12.6 Epithelial (racial) melanosis. (A) Juxtalimbal involvement; (B) Axenfeld loops

Miscellaneous tumours

1Episcleral haemangioma or telangiectasia may be associated with Sturge–Weber syndrome (Fig. 12.7A).

2Reactive pseudoepitheliomatous hyperplasia is a rapidly-growing, white, hyperkeratotic, juxtalimbal nodule which develops secondary to irritation (Fig. 12.7B).

3Benign hereditary intraepithelial dyskeratosis is a rare bilateral juxtalimbal hyperplastic translucent lesion with dilated vessels (Fig. 12.7C).

4Melanocytoma is a rare, congenital, black, slowly-growing lesion which cannot be moved freely over the globe (Fig. 12.7D).

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Fig. 12.7 Miscellaneous benign conjunctival tumours. (A) Haemangioma; (B) reactive pseudoepitheliomatous hyperplasia; (C) hereditary intraepithelial dyskeratosis;

(D) melanocytoma

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