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
Cutaneous angiosarcoma - associated with lymphedema
occurring years after radiation | occurring after years of lymphedema
26
Dermoscopy head and neck angiosarcoma
Typical colours of vascular tumours - red, purple, blue and white lines in nodular area
27
Merckel cell carcinoma - Epidemiology
0.2-0.45 cases per 100 000 100 times rarer than melanoma increasing incidence recent years
28
Merckel cell carcinoma - Clinical
sun exposed - because immune suppressed mainly elderly but not only rapidly enlarging reddish dome shaped nodule
29
Merckel cell carcinoma - Dermoscopy
milky red area polymorphous vascular pattern may be arborizing white lines
30
Merckel cell carcinoma - Management
Wide local excision. Some recommend sentinel bx. Some recommend only wide excision and radiation. role of radiotherapy debated
31
Merckel cell carcinoma - Ddx
BCC, SCC, Melanoma
32
Merckel cell carcinoma - Prognosis
Poor tend to recur after incomplete excision Metastasizes
33
Adnexal tumours - how are they classified?
classified according to adnexal differentiation
34
Adnexal tumours - classification
follicular sebaceous eccrine apocrine
35
Adnexal tumours - associations
Associated with complex syndromes
36
Adnexal tumours - features overlapping with BCC
arborizing vessels | blue grey dots or globules
37
Tumours of hair follicle
``` desmoplastic trichoepithelioma pilomatricoma Trichilemmona Trichilemmal carcinoma Trichodiscoma ```
38
Desmoplastic trichoepithelioma - clinical characteristics
``` Usually arises on face or scalp Small nodule Usually present for a long time Ivory-white background colour short vessels - not sharply focussed. ```
39
Pilomatricoma - clinical
Young patients/ children Firm on palpation. Sometimes tender on palpation
40
Pilomatricoma - Dermoscopy
``` Irregular white and yellow structures White streaks Reddish homogenous areas Linear vessels Ulceration Blue grey areas ```
41
Trichilemmoma - clinical
Verrucous lesion
42
Trichilemmoma - Dermoscopy
keratin masses | perivascular whitish halos
43
Trichilemmoma - associated syndrome
Cowden syndrome
44
Trichilemmal carcinoma - clinical
may cause cicatricial alopecia (don't see the hair follicle.)
45
Trichilemmal carcinoma - Management
Wide excision
46
Trichodiscoma - clinical
young patients may be solitary when multiple think about Birt Hogg Dube syndrome usually syndrome is diagnoses before dermatologist sees patient.
47
Trichodiscoma - Dermoscopy
whitish globular structures distributed in a cobblestone-like pattern combined with blue-grey nests blurred linear vessels
48
Trichodiscoma - Ddx
Congenital nevus | Seb keratosis
49
Sweat gland tumours
``` Eccrine poroma Eccrine porocarcinoma Hidrocystoma Syringomas Syringocystadenoma papilliferum Cyclindroma Spiradenoma ```
50
Eccrine poroma - clinical
Typical arises on feet and palms of hands | Slowly enlarging nodule
51
Eccrine poroma - Dermoscopy
Polymorphous vessels with white halo | Peripheral yellowish structures
52
Eccrine poroma - the great masquerader
Can mimic everything - pigmented BCC, pigmented melanoma, amelanotic melanoma
53
Eccrine porocarcinoma - clinical
arises on pre-existing benign poroid tumours as reddish ulcerated nodule
54
Eccrine porocarcinoma - Dermoscopy
Amelanotic tumour Polymorphous vascular pattern - dotted and linear irregular vessels ulceration
55
Hidrocystoma - clinical
predilection for peri-orbital areas | lesions may be found on other areas of face, head and trunk
56
Hidrocystoma - Dermoscopy
structureless skin coloured/ yellowish to bluish areas possibly in combination with linear vessels
57
Syringomas - clinical
commonest sweat gland tumour
58
Syringomas - Dermoscopy
yellowish/brownish structureless background | possible combined with scarce fine linear vessels
59
Eruptive syringoma - what is it?
Uncommon clinical condition Multiple yellow/brown firm papule No specific site predilection Young adults
60
Syringocystadenoma papilliferum - clinical
Arises mainly from sebaceous tumours | Nodule with a lot of yellow colour dt keratin masses
61
Syringocystadenoma papilliferum - Dermoscopy
yellow colour dt keratin masses pink background linear not in focus vessels
62
Adnexal tumour with associated syndromes
``` Cyclindroma Spiradenoma Trichodiscoma Trichilemmoma Sebaceous tumours Keratoacanthomas ```
63
Cyclindroma - Dermoscopy
overlapping characteristics of BCC linear vessels unfocused and arranged at periphery of the lesion. sometimes yellowish pinkish background and arborizing vessels
64
Brooke-Spiegler syndrome
``` Multiple skin tumours Developing from skin appendages Spiradenomas Trichoepitheliomas Cylindromas ```
65
Spiradenoma - clinical
``` solitary painful nodular young adults trunk and upper extremities ```
66
Cyclindroma - clinical
usually develops as solid lesion (nodule) on the scalp or face of adult females
66
Birt-Hogg-Dube syndrome
``` Multiple trichodiscomas Associated with benign and malignant renal neoplasms, Lung cysts Spontaneous pneumothorax ```
67
Cowden syndrome
``` 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
Muir Torre Syndrome
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
Which sebaceous tumours are associated with Muir Torre Syndrome?
Sebaceomas Sebaceous adenoma Sebaceous carcinoma
70
sebaceous adenoma - Dermoscopy
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
sebaceous carcinoma vs BCC, Dermoscopy
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
sebaceous adenoma vs sebaceous hyperplasia vs squamous cell carcinoma
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
sporadic sebaceous carcinoma - clinical
very rare very aggressive typical in immunosuppressed patient Upper eyelid is common site
74
sporadic sebaceous carcinoma - management
wide local excision
75
sporadic sebaceous carcinoma - Dermoscopy
white reddish background dotted vessels ulceration yellowish hyperkeratotic material
76
Guidelines for dealing with uncommon tumours
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.