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HealthCert Education > Rare skin tumours COPY > Flashcards

Rare skin tumours COPY Flashcards

1
Q

Tumours we are covering

A

Tumours of fibrous tissue Merkel cell carcinoma Angiosarcoma Adnexal tumours Sebaceous tumours

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2
Q

Tumours of fibrous tissue

A

Atypical fibroxanthoma

Malignant fibrous histiocytoma (Malignant part of above) Dermatofibrosarcoma protuberans

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3
Q

Atypical fibroxanthoma

A

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

dermoscopy: 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

tendency to recur after incomplete excision > wide local excision

excellent prognosis

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4
Q

Malignant histiocytoma

A

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

ddx: bcc, scc, melanoma
dermoscopy: Polymorphous vessels Ulceration Hemorrhage

overall survival 50% aggressive tumour metastasizes

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5
Q

Adnexal tumours - classification

A

follicular

sebaceous

eccrine

apocrine

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6
Q

Adnexal tumour with associated syndromes

A

Cyclindroma

Spiradenoma

Trichodiscoma

Trichilemmoma

Sebaceous tumours

Keratoacanthomas

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7
Q

Brooke-Spiegler syndrome

A

Multiple skin tumours Developing from skin appendages

Spiradenomas

Cylindromas

Trichoepitheliomas

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8
Q

Cylindroma and spiradenoma —

A

Cylindromas and spiradenomas are sweat duct tumors centered in the dermis that often show overlapping features in the same tumor (spiradenocylindroma). They occur sporadically in the older adult population, typically as a solitary papule or nodule on the head or neck or, less commonly, on the trunk and extremities (picture 1B, 1D). Detection of multiple cylindromas (picture 12) or spiradenomas should raise the suspicion of Brooke-Spiegler syndrome or familial cylindromatosis caused by mutation of CYLD. (See ‘Syndromes associated with cutaneous adnexal tumors’ below.)

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9
Q

Trichoblastoma and trichoepithelioma —

A

Trichoblastoma and trichoepithelioma are benign epithelial tumors composed of follicular germinative cells resembling those seen in the embryologic buds of primitive folliculosebaceous units. They present as smooth, nonulcerated, skin-colored papules, sometimes with associated telangiectasias usually located on the head and neck . The majority of cases occur in adults after the age of 40. The presence of multiple trichoepitheliomas should raise the suspicion of Brooke-Spiegler syndrome or multiple familial trichoepithelioma.

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10
Q

Birt-Hogg-Dube syndrome

A

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

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11
Q

Trichodiscoma and fibrofolliculoma —

A

Although originally described as distinct entities, evidence suggests that trichodiscomas and fibrofolliculomas represent a spectrum of the same neoplastic process [80]. There is also overlap with perifollicular fibroma (angiofibroma). Both fibrofolliculoma and trichodiscoma present as small, 2 to 4 mm, dome-shaped, skin-colored papules on the face, scalp, ears, or upper trunk. They occur sporadically in adults and are seen in multiplicity in patients with Birt-Hogg-Dubé syndrome

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12
Q

Cowden syndrome

A

AKA multiple hamartoma syndrome.

The common lesions are trichilemmomas, acral keratoses, and facial papules/oral papillomas

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.

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13
Q

Trichilemmomas

A

hamartomatous tumors of the outer root sheath of the hair follicle or other skin appendages that occur on the face and neck of patients with Cowden syndrome. Trichilemmoma is a clinically significant sign of Cowden syndrome when seen in multiplicity (three or more). They present as wart-like, skin-colored papules with slightly rough surfaces; histologically, the lesions contain large glycogen-rich cells [44]. Clinically, trichilemmomas are indistinguishable from trichoepitheliomas and other benign follicular tumors affecting the pilosebaceous unit, including fibrofolliculomas and trichodiscomas (which are characteristic lesions of Birt-Hogg-Dube syndrome) [45]. At least one lesion should be biopsied given the difficulty with clinical diagnosis [46]. (See “Birt-Hogg-Dubé syndrome”.)

A trichilemmoma is rarely a sporadic feature. One study found a complete loss of PTEN expression by immunohistochemistry in five of six (83 percent) trichilemmomas from patients with Cowden syndrome compared with only 1 of 33 (3 percent) sporadic trichilemmomas [47]

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14
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)

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15
Q

Which sebaceous tumours are associated with Muir Torre Syndrome?

A

Sebaceomas Sebaceous adenoma Sebaceous carcinoma

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16
Q

Trichilemmoma - associated syndrome

A

Cowden syndrome

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17
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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18
Q

Dermatofibrosarcoma protuburans - incidence

A

1% of soft tissue sarcomas most common cutaneous sarcoma

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19
Q

Dermatofibrosarcoma protuburans - Ddx

A

dermatofibroma

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20
Q

Dermatofibrosarcoma protuburans - Dermoscopy

A

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

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21
Q

Dermatofibrosarcoma protuburans - Malignant potential

A

Low to intermediate rarely metastasizes locally aggressive high recurrence rate

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22
Q

Dermatofibrosarcoma protuburans - management

A

Wide local excision - 5 cm

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23
Q

Cutaneous angiosarcoma of head and neck - clinical

A

Unique entity elderly men poor prognosis

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24
Q

Cutaneous angiosarcoma - associated with lymphedema

A

occurring years after radiation occurring after years of lymphedema

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25
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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26
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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27
Q

Merckel cell carcinoma - Clinical

A

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

28
Q

Merckel cell carcinoma - Dermoscopy

A

milky red area polymorphous vascular pattern may be arborizing white lines

29
Q

Merckel cell carcinoma - Management

A

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

30
Q

Merckel cell carcinoma - Ddx

A

BCC, SCC, Melanoma

31
Q

Merckel cell carcinoma - Prognosis

A

Poor tend to recur after incomplete excision Metastasizes

32
Q

Adnexal tumours - features overlapping with BCC

A

arborizing vessels blue grey dots or globules

33
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.

34
Q

Pilomatricoma - clinical

A

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

35
Q

Pilomatricoma - Dermoscopy

A

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

36
Q

Eccrine poroma - clinical

A

Typical arises on feet and palms of hands Slowly enlarging nodule

37
Q

Eccrine poroma - Dermoscopy

A

Polymorphous vessels with white halo Peripheral yellowish structures

38
Q

Eccrine poroma - the great masquerader

A

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

39
Q

Eccrine porocarcinoma - clinical

A

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

40
Q

Tumours of hair follicle

A

desmoplastic

trichoepithelioma

pilomatricoma

41
Q

Eccrine porocarcinoma - Dermoscopy

A

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

42
Q

Hidrocystoma - clinical

A

predilection for peri-orbital areas lesions may be found on other areas of face, head and trunk

43
Q

Hidrocystoma - Dermoscopy

A

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

44
Q

Syringomas - clinical

A

commonest sweat gland tumour

45
Q

Syringomas - Dermoscopy

A

yellowish/brownish structureless background possible combined with scarce fine linear vessels

46
Q

Eruptive syringoma - what is it?

A

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

47
Q

Syringocystadenoma papilliferum - clinical

A

Arises mainly from sebaceous tumours Nodule with a lot of yellow colour dt keratin masses

48
Q

Syringocystadenoma papilliferum - Dermoscopy

A

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

49
Q

Trichilemmal carcinoma - clinical

A

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

50
Q

Cyclindroma - clinical

A

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

51
Q

Spiradenoma - clinical

A

solitary painful nodular young adults trunk and upper extremities

52
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

53
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.

54
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.

55
Q

sporadic sebaceous carcinoma - clinical

A

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

56
Q

sporadic sebaceous carcinoma - management

A

wide local excision

57
Q

sporadic sebaceous carcinoma - Dermoscopy

A

white reddish background dotted vessels ulceration yellowish hyperkeratotic material

58
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.

59
Q

Trichilemmoma - clinical

A

Verrucous lesion

60
Q

Trichodiscoma - Ddx

A

Congenital nevus Seb keratosis

61
Q

Trichilemmoma - Dermoscopy

A

keratin masses perivascular whitish halos

62
Q

Trichodiscoma - Dermoscopy

A

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

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

Sweat gland tumours

A

Eccrine poroma Eccrine porocarcinoma Hidrocystoma Syringomas Syringocystadenoma papilliferum Trichilemmal carcinoma Cyclindroma Spiradenoma Trichodiscoma Trichilemmona

65
Q

Trichilemmal carcinoma - Management

A

Wide excision

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