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HealthCert Education > Rare skin tumours > Flashcards

Rare skin tumours Flashcards

1
Q

diagnosis problems

A

non specific criteria

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2
Q

workup protocol

A

recognizing associated syndromes

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3
Q

treatment and follow up problems

A

lack of guidelines

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4
Q

Keyword in management of rare tumours

A

Multidisciplinary

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5
Q

Tumours we are covering

A 
Tumours of fibrous tissue
Merkel cell carcinoma
Angiosarcoma
Adnexal tumours
Sebaceous tumours
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6
Q

Tumours of fibrous tissue

A

Atypical fibroxanthoma
Malignant fibrous histiocytoma (Malignant part of above)
Dermatofibrosarcoma protuberans

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7
Q

Atypical fibroxanthoma - clinical

A 
elderly - Mainly men
sun exposed - Usually head
rapidly enlarging
reddish
dome shaped nodule
often eroded or crusted surface
darker hue dt hemosiderin deposition
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8
Q

Atypical fibroxanthoma - Ddx

A

Melanoma, BCC, SCC

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9
Q

Atypical fibroxanthoma - Dermoscopy

A

reddish and whitish areas - majority non pigmented
white structureless and or red structureless
white lines
may have white circles
polymorphous vascular pattern - vessels usually fine, linear
ulceration, crusting and keratin masses

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10
Q

Atypical fibroxanthoma - management

A

tendency to recur after incomplete excision > wide local excision

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11
Q

Atypical fibroxanthoma - prognosis

A

excellent prognosis

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12
Q

Malignant histiocytoma - clinical

A

elderly
sun exposed
enlarging subcutaneous nodule, may have significant size and ulcerate

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13
Q

Malignant histiocytoma - ddx

A

bcc, scc, melanoma

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14
Q

Malignant histiocytoma - Dermoscopy

A

Polymorphous vessels
Ulceration
Hemorrhage

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15
Q

Malignant histiocytoma - Management

A

Wide local excision
Adjuvant radioRx
Chemotherapy

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16
Q

Malignant histiocytoma - Prognosis

A

overall survival 50%
aggressive tumour
metastasizes

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17
Q

Malignant histiocytoma - histopathology pitfall

A

Can confuse atypical fibroxanthoma with malignant histiocytoma

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18
Q

Dermatofibrosarcoma protuburans - clinical

A

one or more firm, erythematous nodules or plaques
resembles scar but no h/o scar
may enlarge slowly over many years
May ulcerated or suppurate

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19
Q

Dermatofibrosarcoma protuburans - incidence

A

1% of soft tissue sarcomas

most common cutaneous sarcoma

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20
Q

Dermatofibrosarcoma protuburans - Ddx

A

dermatofibroma

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21
Q

Dermatofibrosarcoma protuburans - Dermoscopy

A

reddish background colour
fine linear vessels - may be arborizing
no criteria for dermatofibroma

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22
Q

Dermatofibrosarcoma protuburans - Malignant potential

A

Low to intermediate
rarely metastasizes
locally aggressive
high recurrence rate

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23
Q

Dermatofibrosarcoma protuburans - management

A

Wide local excision - 5 cm

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24
Q

Cutaneous angiosarcoma of head and neck - clinical

A

Unique entity
elderly men
poor prognosis

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25
Q

Cutaneous angiosarcoma - associated with lymphedema

A

occurring years after radiation

occurring after years of lymphedema

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26
Q

Dermoscopy head and neck angiosarcoma

A

Typical colours of vascular tumours - red, purple, blue and white lines in nodular area

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27
Q

Merckel cell carcinoma - Epidemiology

A

0.2-0.45 cases per 100 000
100 times rarer than melanoma
increasing incidence recent years

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28
Q

Merckel cell carcinoma - Clinical

A

sun exposed - because immune suppressed
mainly elderly but not only
rapidly enlarging reddish dome shaped nodule

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29
Q

Merckel cell carcinoma - Dermoscopy

A

milky red area
polymorphous vascular pattern
may be arborizing
white lines

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30
Q

Merckel cell carcinoma - Management

A

Wide local excision.
Some recommend sentinel bx.
Some recommend only wide excision and radiation.
role of radiotherapy debated

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31
Q

Merckel cell carcinoma - Ddx

A

BCC, SCC, Melanoma

32
Q

Merckel cell carcinoma - Prognosis

A

Poor
tend to recur after incomplete excision
Metastasizes

33
Q

Adnexal tumours - how are they classified?

A

classified according to adnexal differentiation

34
Q

Adnexal tumours - classification

A

follicular
sebaceous
eccrine
apocrine

35
Q

Adnexal tumours - associations

A

Associated with complex syndromes

36
Q

Adnexal tumours - features overlapping with BCC

A

arborizing vessels

blue grey dots or globules

37
Q

Tumours of hair follicle

A 
desmoplastic trichoepithelioma
pilomatricoma
Trichilemmona
Trichilemmal carcinoma
Trichodiscoma
38
Q

Desmoplastic trichoepithelioma - clinical characteristics

A 
Usually arises on face or scalp
Small nodule
Usually present for a long time
Ivory-white background colour
short vessels - not sharply focussed.
39
Q

Pilomatricoma - clinical

A

Young patients/ children
Firm on palpation.
Sometimes tender on palpation

40
Q

Pilomatricoma - Dermoscopy

A 
Irregular white and yellow structures
White streaks 
Reddish homogenous areas
Linear vessels
Ulceration
Blue grey areas
41
Q

Trichilemmoma - clinical

A

Verrucous lesion

42
Q

Trichilemmoma - Dermoscopy

A

keratin masses

perivascular whitish halos

43
Q

Trichilemmoma - associated syndrome

A

Cowden syndrome

44
Q

Trichilemmal carcinoma - clinical

A

may cause cicatricial alopecia (don’t see the hair follicle.)

45
Q

Trichilemmal carcinoma - Management

A

Wide excision

46
Q

Trichodiscoma - clinical

A

young patients
may be solitary
when multiple think about Birt Hogg Dube syndrome
usually syndrome is diagnoses before dermatologist sees patient.

47
Q

Trichodiscoma - Dermoscopy

A

whitish globular structures
distributed in a cobblestone-like pattern
combined with blue-grey nests
blurred linear vessels

48
Q

Trichodiscoma - Ddx

A

Congenital nevus

Seb keratosis

49
Q

Sweat gland tumours

A 
Eccrine poroma
Eccrine porocarcinoma
Hidrocystoma
Syringomas
Syringocystadenoma papilliferum
Cyclindroma
Spiradenoma
50
Q

Eccrine poroma - clinical

A

Typical arises on feet and palms of hands

Slowly enlarging nodule

51
Q

Eccrine poroma - Dermoscopy

A

Polymorphous vessels with white halo

Peripheral yellowish structures

52
Q

Eccrine poroma - the great masquerader

A

Can mimic everything - pigmented BCC, pigmented melanoma, amelanotic melanoma

53
Q

Eccrine porocarcinoma - clinical

A

arises on pre-existing benign poroid tumours as reddish ulcerated nodule

54
Q

Eccrine porocarcinoma - Dermoscopy

A

Amelanotic tumour
Polymorphous vascular pattern - dotted and linear irregular vessels
ulceration

55
Q

Hidrocystoma - clinical

A

predilection for peri-orbital areas

lesions may be found on other areas of face, head and trunk

56
Q

Hidrocystoma - Dermoscopy

A

structureless
skin coloured/ yellowish to bluish areas
possibly in combination with linear vessels

57
Q

Syringomas - clinical

A

commonest sweat gland tumour

58
Q

Syringomas - Dermoscopy

A

yellowish/brownish structureless background

possible combined with scarce fine linear vessels

59
Q

Eruptive syringoma - what is it?

A

Uncommon clinical condition
Multiple yellow/brown firm papule
No specific site predilection
Young adults

60
Q

Syringocystadenoma papilliferum - clinical

A

Arises mainly from sebaceous tumours

Nodule with a lot of yellow colour dt keratin masses

61
Q

Syringocystadenoma papilliferum - Dermoscopy

A

yellow colour dt keratin masses
pink background
linear not in focus vessels

62
Q

Adnexal tumour with associated syndromes

A 
Cyclindroma
Spiradenoma
Trichodiscoma
Trichilemmoma
Sebaceous tumours
Keratoacanthomas
63
Q

Cyclindroma - Dermoscopy

A

overlapping characteristics of BCC
linear vessels unfocused and arranged at periphery of the lesion.
sometimes yellowish pinkish background and arborizing vessels

64
Q

Brooke-Spiegler syndrome

A 
Multiple skin tumours
Developing from skin appendages
Spiradenomas
Trichoepitheliomas
Cylindromas
65
Q

Spiradenoma - clinical

A 
solitary
painful
nodular
young adults
trunk and upper extremities
66
Q

Cyclindroma - clinical

A

usually develops as solid lesion (nodule) on the scalp or face of adult females

66
Q

Birt-Hogg-Dube syndrome

A 
Multiple trichodiscomas
Associated with 
benign and malignant renal neoplasms, 
Lung cysts
Spontaneous pneumothorax
67
Q

Cowden syndrome

A 
AKA multiple hamartoma syndrome
Young age
Trichilemmoma 
Oral mucosal papillomatosis
Acral keratoses
Palmoplantar keratoses
Associated with malignancy - breast Ca, thyroid Ca, Colon Ca, Renal cell Ca. 
Usually the dermatologist is last one to see the patient.
68
Q

Muir Torre Syndrome

A

Phenotypic variant of Lynch syndrome (non polyposis colon cancer syndrome)
Autosomal dominant
Sebaceous tumours
Multiple keratoacanthoma
Early onset visceral tumours (Colon and urinary tract)

69
Q

Which sebaceous tumours are associated with Muir Torre Syndrome?

A

Sebaceomas
Sebaceous adenoma
Sebaceous carcinoma

70
Q

sebaceous adenoma - Dermoscopy

A

One presentation:
Yellowish ovoid nests
Arborizing vessels not in focus

Alternate presentation:
Short dotted and linear vessels at periphery
In centre, either keratin mass, or ulceration, or yellowish mass. More resembling keratoacanthoma

71
Q

sebaceous carcinoma vs BCC, Dermoscopy

A

SC: unfocussed arborizing vessels over a white to yellow background and few loosely arranged yellow comedo like globules.

BCC: In focus arborizing vessels and ulceration.

72
Q

sebaceous adenoma vs sebaceous hyperplasia vs squamous cell carcinoma

A

SA: Central crater, crown vessels, opaque structureless, white centre, blood crusts.
SH: Crown vessels, page structureless, white/yellow centre.
SCC: Keratotic material. White circles.

73
Q

sporadic sebaceous carcinoma - clinical

A

very rare
very aggressive
typical in immunosuppressed patient
Upper eyelid is common site

74
Q

sporadic sebaceous carcinoma - management

A

wide local excision

75
Q

sporadic sebaceous carcinoma - Dermoscopy

A

white reddish background
dotted vessels
ulceration
yellowish hyperkeratotic material

76
Q

Guidelines for dealing with uncommon tumours

A
  1. Collect and document cases
  2. Excise rapidly evolving nodular lesions with atypical vascular pattern in Dermoscopy. High chance of malignancy.
  3. Collaborate with pathologist
  4. Multidisciplinary approach to management.
  5. Look for associated syndromes.
  6. Genetic counselling.

HealthCert Education (24 decks)

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