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Malignant tumours Flashcards

1
Q

What is xeroderma pigmentosa?

A

Autosomal recessive condition

Results in skin damage on exposure to natural sunlight which gives rise to progressive pigmentation abnormalities

Patients have a propensity for BCC, SCC, Melanomas and conjunctival malignancies have also been reported

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2
Q

What is Gorlin-Gotz syndrome

A

Autosomal dominant

Characterised by extensive congenital abnormalities of the eye, CNS, bone, and face

Many patients develop multiple small BCC during the 2nd decade of life

They are predisposed to medulloblastoma, breast carcinoma and hodgkin lymphoma

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3
Q

What is Muir-Torre syndrome

A

Rare AD condition

Predisposes to cutaneous and internal malignancies

Cutaneous tumours include BCC, Sebaceous gland carcinoma and keratocanthoma

Most common systemic malignancies includes colorectal and genitourinary carcinoma

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4
Q

What is bazex syndrome

A

X linked dominant syndrome
Characterised by ezematous and psoriasiform lesions
Associated with carcinomas of the upper respiratory and digestive tracts
Eyelid BCC may also occur

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5
Q

What conditions predispose to ocular malignancy?

A 
Xeroderma Pigmentosa
Gorlin Gotz
Meri-Torre syndrome
Bazex syndrome
Prior retinoblastoma
Albinism
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6
Q

Who does BCC affect?

A

Most common human tumour
Most frequently affects elderly patients
Most common malignant eyelid tumour accounting for 90% of cases

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7
Q

What are the risk factors for BCC?

A

Fair skin
Inability to tan
Chronic exposure to sunlight

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8
Q

Where do BCCs occur?

A

90% are in the head an neck
10% of these involve the eyelid

Frequently arises in order:-

Lower lid
Medial canthus
Upper lid
Lateral canthus

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9
Q

What is the growth of BCC like?

A

Slow growing
Locally invasive
Does not metastasize

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10
Q

Which location of a BCC is more likely to invade adjacent structures?

A

Tumours near the medial canthus are more likely to invade the orbit and adjacent sinuses and can be more difficult to manage and are more likely to recur

Tumour that recur after incomplete treatment tend to be more aggressive

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11
Q

What is the histology of BCC?

A

Proliferation of the cells that form the basal layer of the epidermis. The cells proliferate downwards and exhibit palisading at the peripheries of the tumour lobule.

Squamous differentiation with the production of keratin results in a hyperkeratotic type of BCC. Sebaceous and adenoid differentiation can also occur whilst growth of elongated strands and islands of cells embedded in dense fibrous stroma results in a sclerosing (morphoeic) type of tumour

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12
Q

What are the main clinical features of epithelial cell malignancy?

A

Induration
Ulceration
Irregular borders
Destruction of lid margin architecture

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13
Q

What are the clinical types of BCC?

A

Nodular
Noduloulcerative (rodent ulcer)
Sclerosing
Other (not usually found on the lid) - cystic, adenoid, pigmented and multiple superficial

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14
Q

What is a nodular BCC?

A

Shiny, firm, pearly nodule with small dilated blood vessels on its surface. It is very slow growing and can take 1-2 years to reach a diameter of 0.5cm

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15
Q

What is a noduloulcerative BCC?

A

Also known as a rodent ulcer
central ulceration
Pearly rolled edges
Dilated irregular blood vessels (Telangectasia) over its lateral margins
Over time may erode a large portion of the eyelid

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16
Q

Sclerosing BCC

A

Also known as morphoeic
Difficult to diagnose because it infiltrates the epidermis laterally as an indurated plaque
Margins of the tumour may be impossible to delineate clinically and are often much more extensive on palpation compared to inspection
A sclerosing BCC may simulate a localised area of blepharitis

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17
Q

What are the general features of SCC?

A

SCC is much less common than BCC
It is more aggressive
It metastasizes to regional lymph nodes in 20% of cases. Careful surveillance of regional lymph nodes is an important part of initial management
Can also exhibit perineural spread to the intracranial cavity via the orbit
Accounts for 5-10% of eyelid malignancies
Can arise de novo or from pre-existing acitinic keratosis or carcinoma in situ (Bowen’s disease, intraepidermal carcinoma)
Immunocompromised patients such as AIDs patients or those with renal transplants are at increased risk
SCC has a prediliction for the lower eyelid and the lid margin
It occurs most commonly in fair skinned elderly individuals with chronic sun exposure
Diagnosis can be difficult to obtain because ostensibly benign lesions such as keratoacthoma or cutaneous horn may reveal histological evidence of invasive SCC at deeper levels of sectioning

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18
Q

What is the histology of SCC

A

The tumour arises from the squamous cell layer of the epidermis and consists of variable sized groups of atypical epithelial cells with prominent nuclei and abundant eosinophilic cytoplasm within the dermis. Well differentiated tumours may exhibit characteristic keratin ‘pearls’ or intercellular bridges (desmosomes)

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19
Q

What are the clinical types of SCC

A 
Variable, no pathognomic characteristics
Can be indistinguishable from BCC BUT
Surface vascularisation is often absent
Growth is more rapid
Hyperkeratosis is more often present

Nodular SCC
Ulcerative SCC
Cutaneous horn

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20
Q

What is a nodular SCC

A

Hyperkeratotic nodule which can develop crusting erosions and fissures

21
Q

What is an ulcerating SCC

A

Red base
Sharply defined
Indurated with everted borders
But pearly borders and telangiectasia is not usually present

22
Q

What is a cutaneous horn

A

Can have an underlying invasive SCC

23
Q

What is keratoacanthoma

A

Rare tumour
Usually occurs in fair skinned individuals with chronic skin exposure
Found more frequently than would expected by chance in patients on immunosuppressive therapy
Histologically regarded as part of the spectrum of SCC

24
Q

What is the histology of keratoacanthoma

A

Irregular thickened epithelium surrounded by acanthotic squamous epithelium
The sharp transition from thickened to normal adjacent epidermis is known as shoulder formation
A keratin filled crater may be seen

25
Q

What are the signs of keratoacanthoma

A

In chronological order:-

A pink, rapidly growing hyperkeratotic lesion often on the lower lid

it can double or treble in size within weeks

Growth ceases for 2-3 months and then spontaneous involution occurs

During the period of regression a keratin filled crater may develop

Complete involution can take up to a year and may leave an unsightly scar

26
Q

What is the treatment of keratoacanthoma

A

Complete surgical excision
Cryotherapy
Radiotherapy
Topical or intralesional 5-flurouracil

27
Q

What are the general features of sebaceous gland carcinoma?

A

Very rare slowly growing tumour affecting elderly individuals with a predisposition for females

It usually arises from the meibomian glands although ti can sometimes arise from a gland of Zeiss or sebaceous gland in the caruncle

In contrast to BCC or SCC it more commonly occurs in the upper lid where the meibomian glands are more numerous

In about 5% of cases there is simulataneous involvement of the upper and lower lid either because of intraepithelial spread or the spontaneous development of multiple primaries

Clinically diagnosis of SGC is difficult because the early signs of malignancy may be so subtle it may resemble a chalazion or blepharitis

The presence of yellowish material within the tumour is highly suggestive of SGC

Because of frequent difficulties with diagnosis and delay in treatment mortality is 5-10%

28
Q

What are the adverse prognostic factors for SGC

A

Involvement of the upper lid
Tumour size 10mm or greater
Duration of symptoms over 6 months
If SGC arises from a gland of Zeis it is thought to have a more favourable prognosis

29
Q

What is the histology of SGC

A

The tumour consists of lobules of cells with pale, foamy, vacuolated lipid-containing cytoplasm and large hyperchromatic nuclei

30
Q

What are the clinical types of SGC

A

Nodular SGC
Spreading SGC
Pagetoid spread

31
Q

What is a noular SGC

A

Presents as a discrete hard nodule, usually in the upper lid. It can have a yellowish discolouration to the presence of lipid.

As it may masquerade as a chalazion it is recommended that any chalazion of an unusual consistency undergo full thickness resection and histological examination

32
Q

What is a spreading SGC

A

Infiltrates into the dermis and causes a diffuse thickening of the lid margin. It may result in the loss of lashes and may be mistaken for ‘chronic blepharitis’. Occasionally the tumour may exhibit multi-focal non-contiguous origins

33
Q

What is pategoid spread of SGC

A

Refers to the extension of the tumour within epithelium including palpebral, fornical or bulbar conjunctiva. This may lead to a mistaken diagnosis of an inflammatory condition

34
Q

What is a hallmark of melanoma?

A

Pigmentation, but half of lid melanomas are non-pigmented and this can give rise to diagnostic difficulties

35
Q

What features are suggestive of melanoma?

A 
Recent onset of a pigmented lesion
Change in existing pigmented lesion
Asymmetrical shape
Irregular margin
Change in colour/presence of multiple colours
Diameter greater than 6mm
36
Q

What are alternate names for lentigo maligna

A

Melanoma in situ
Hutchinson freckle
Intraepidermal melanoma

37
Q

What is lentigo maligna

A

It is an uncommon condition that develops in sun damaged skin in elderly individuals

Malignant change may occur with infiltration of the dermis

38
Q

What is the histology of lentigo maligna

A

Intraepidermal proliferation of spindle shaped atypical melanocytes that replace the basal cell layer in the epidermis

39
Q

What are the signs of lentigo maligna

A

Slowly expanding pigmented macule with irregular borders

Nodular thickening and areas of irregular pigmentation is highly suggestive of malignant transformation

40
Q

What is the treatment of lentigo maligna

A

Excision

41
Q

What is merkel cell carcinoma

A

Fast growing tumour
Typically affects elderly patients
Even though Merkel cells are in the epidermis the tumour appears to arise from the dermis
As it is very rare - diagnostic difficulties and delay to treatment
Highly malignant, 50% of patients have metastatic spread at presentation

42
Q

What is the histology of merkel cell carcinoma

A

Sheets of cells with scanty cytoplasm, round or oval nuclei and numerous mitotic figures

43
Q

What are the signs of merkel cell carcinoma

A

Violaceous, well demarcated nodule with intact overlying skin. Most frequently involves the upper lid

44
Q

What is the treatment of merkel cell carcinoma

A

Excision frequently combined with chemotherapy

45
Q

What is Kaposi sarcoma

A

Vascular tumour
Typically affects AIDs patients who often have advanced systemic disease
May be only manifestation of HIV infection

46
Q

What is the histology of Kaposi sarcoma

A

Proliferating spindle cells, vascular channels and inflammatory cells within the dermis

47
Q

What are the signs of Kaposi sarcoma

A

Pink, red-violet to brown lesion which may be mistaken for a naevus or haematoma

48
Q

What is the treatment of Kaposi sarcoma

A

Radiotherapy or excision

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