Benign Melanocytic Neoplasms Flashcards

1
Q

What is the difference between ephelides and lentigo?

A

Ephelides can come and go with sun exposure

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2
Q

Pathogenesis of ephelides?

A

Increased melanogenesis and melanin transfer to keratinocytes

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3
Q

Histology of ephilids

A

Increased basilar keratinocyte pigmentation and enlarged melanocytes w/o increased density

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4
Q

Multiple cafe au lait macucles (CALMs) is a/w what conditions?

A

Russell-Silver, Bloom syndrome, Neurofibromatosis type 1>type 2, tuberous sclerosis, ataxia telangietasias, noonan sydrome, fanconi syndrome, mccune-albright syndrome; MEN-1

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5
Q

What are the CALMS like in Mccune Albright syndrome?

A

Fewer CALMs, larger, midline demarcation, segmental distribution, patter, broad bands along Blaschko lines

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6
Q

What are the CALMS like in NF type 1?

A

Can have superimposed lentigines

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7
Q

Which melanocytic lesion increases hair and can have acneiform lesions?

A

becker’s nevus

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8
Q

What is becker’s nevus a/w?

A

Hypoplasia of ipsilateral breast, areola, nipple and arm, ipsilateral arm shortening, lumar spina bifida, thoracic scoliosis, pectus carnatum, enlargement of the ipsilateral foot.

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9
Q

Do solar lentigines fade over time?

A

No

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10
Q

Histology of solar lentigines?

A

Dirty socks (hyperpigmentation of the bottom of the rete pegs), epidermal hypoplasia, and sun damage

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11
Q

What lentigos are in younger people?

A

Lentigo simplex and mucosal melanotic lesions

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12
Q

The most common location for lentigines or melanosis of the female genital area?

A

Labia minor most common

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13
Q

What can lentigo simplex and mucosal melanotic lesions be a/w?

A

Cronkhite-Canada, Carney complex (LAMB/NAME), LEOPARD (aka Noonan w/ multiple lentigines), Laugier-Hunziker, Bannayan-Riley-Ruvalcaba (penile), Peutz-Jeghers (especially oral/perioral), xeroderma pigmetosum, Cowden syndrome

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14
Q

Why are dermal melanocytosis grey-blue appearing

A

Tyndall effect

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15
Q

Extensive dermal melanocytosis could be related to which genetic disorders?

A

Phakomatosis cesioflammea (type II phakomatosis pigmentosvascularis (PPV)), phakomatosis cesiomarmorata (type V PPV)

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16
Q

Epidemiology of the nevus of Ota?

A

First year of life or puberty, increased in Asians and Blacks

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17
Q

Most common location of nevus of Ota?

A

Coalescing gray/blue mauces in V1/V2 distribution. frequent scleral involvment (60%); unilateral (90%)>bilateral

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18
Q

What is the mutation in nevus of Ota that degenerates to uveal melanoma?

A

GNAQ

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19
Q

What can nevus of Ota turn in to?

A

Uveal melanoma

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20
Q

What is nevus of Ito?

A

Located on shoulder, suprclavicular, scalpular regions; essentially no risk of progression to melanotic

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21
Q

What is a Hori’s nevus?

A

Acquired nevus of Ota-like macules bilateral zygomatic regions; Most common in East Asian females

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22
Q

What is a Sun’s nevus?

A

Acquired, unilateral (Only one sun) nevus. This is different because nevus of Ota/Ito are usually present at birth

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23
Q

Histology differences between nevus of Ota and dermal melanocytosis?

A

Elongated dendritic melanocytes are more numerous in nevus of Ota and involve levels of the dermis to varying degrees (superficial dominant, deep dominant, etc)

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24
Q

Epidemiology of blue nevi?

A

Onset is usually in childhood or adolescence 25% are cellular blue nevi

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25
Q

Most common sites of blue nevus?

A

Scalp, sacral area, distal extensor extremities

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26
Q

Where do the melanocytes that make up blue nevi come from?

A

They are retained melanocytes in the dermis that persist during embryogenesis rather than populating epidermis

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27
Q

What mutations can you see in blue nevi?

A

GNAQ and GNA11

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28
Q

What are the different variants of blue nevus?

A

Common blue nevi (<1cm), hands, feet, face scalp cellular (larger 1-3cm, favors buttocks or scalp) epithelioid blue nevus (a/w carney complex, trunk, and extremities) Malignant blue nevus (melanoma, GNAQ/GNA11, and BAP-1 mutations)

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29
Q

How to distinguish blue nevus from nevus of Ota or dermal melanocytosis?

A

Sclerotic collagen in the dermis which is seen in blue nevus and not in nevus of Ota or dermal melanocytosis

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30
Q

What blue nevus type does malignant blue nevus arise from?

A

Cellular blue nevus

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31
Q

What location is most common with malignant blue nevus?

A

Scalp

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32
Q

What two diseases can you see with eruptive acquired nevi?

A

Epidermolysis bullosa and LS+A

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33
Q

What is the abtopfung hypothesis

A

Nevus cells start as juncitonal proliferation–>subsequently migrate into dermis (compound) –> later become entirely intradermal –> may involute

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34
Q

What mutations are in nevi?

A

BRAF mutation in up to 80% more so than NRAS

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35
Q

So what is a key between malignant vs benign pigment on the acral surface on dermoscopy?

A

Should affect the furrows not the ridges

36
Q

Epidemiology of spitz nevi?

A

Most common on the head and neck,

On children, acquired during first two decades

37
Q

What mutations commonly occur in spitz nevi?

A

HRAS/11p gains NO BRAF

38
Q

What stains are + in spitz nevi?

A

S100A6+, S100+, melan A +, p16 + (if negative more scary)

39
Q

What stain is important for atypical spitz or spitzoid melanoma?

A

Loss of P16 is often lost or diminished

40
Q

What is the epidemiology of pigmented spindle cell nevus of reed?

A

Most common in F>M

Most commonly located on the thigh

41
Q

What are the key components on the histology of pigmented spindle cell nevus of Reed?

A

Pigmented parakeratosis, spindle cell melanocytes

42
Q

What disease can be associated with multple dyplastic nevi?

A

Familial atypical multiple mole melanoma syndrome (FAMMM)

43
Q

What mutations is FAMMM associated with?

A

CDKN2A which encodes p16 and p14 ARF

44
Q

What is the heritibility of FAMMM

A

AD

45
Q

Description of FAMMM

A

AD; multip melanocytic nevi (50+), family hx of melanoma, mutation in CDKN2A

46
Q

Treatment of dysplastic nevi?

A

severly atypical are excised

47
Q

What are the components of a dysplastic nevus?

A

Asymmetry

  • Lack of circumscription, junctional shoulder extends >3 rete ridges beyond dermal component, irregular size, and placement of junctional nests with bridging or lentiginous pattern (single-cell junctional growth)
  • Papillary dermal concentric and or lamellar fibrosis
  • Cytologica atypia: nuclei enlarged (can have prominent nucleoli), “dirty” gray cytoplasm
48
Q

What mutations are associated w/ congenital melanocytic nevus?

A

NRAS mutations > BRAF mutations

49
Q

What are the divisions of congenital melanocytic nevus?

A

Small (<1.5cm), medium (1.5-19.9cm), large (>20cm)

50
Q

What congenital melanocytic nevi are associated with problems?

A

Large (>20cm)

note that this is projected size it is not current size)

51
Q

What is the risk associated with large congenital melanocytic nevus?

A

Increased melanoma risk (2-3%) majority develop melanoma in the first decade

52
Q

What is the tx of a congenital melaocytic nevus?

A

if larger posterior axial congenital nevi or multiple satellite lesions –> MRI screening for neurocutaneous melanosis

For large ones surgical resection in stages after 6 months of age

53
Q

What is the difference between deep penetrating nevus and blue nevus?

A

Lack of GNAQ/GNA11 mutations, wedge-shaped, dark melanocytes

54
Q

What is the clinical presentation of nevus spilus?

A

Presents within the first year of life

  • Most commonly located on the trunk and extremities
  • Presents as a tan patch w/ pigmented macules and papules
55
Q

What disorders can nevus spilus be associated with?

A

Phakomatosis pigmentovascularis and phakomatosis pigmentokeratotica

56
Q

What is the risk of melanom in nevus spilus?

A

Very low

57
Q

What is the clinical presentation of a halo nevus?

A

Most commonly occurs in the second decade, and most commonly occurs on the back

Presents as a pigmented nevus with surrounding hypopigmented zone

58
Q

What medications are associated with halo nevi?

A

infliximab

59
Q

What is the difference in histology between a halo nevus and a regressing melanoma?

A

bland nevus w/ lymphocytes intertwined with melanocytes. it is throughout rather than around it like you might see w/ melanoma

60
Q

What are the 3 key features of recurrent melanocytic nevus (vs melanoma)?

A
  1. Dermal scar which the nevus is confined within
  2. Atypical junctional melanocytic proliferation which resembles MIS but is confined to the area above the dermal scar
  3. Bland dermal nevus remnants below or adjacent to scar.
61
Q

What is the histology of a solar lentigo?

A

Elongated bulbous rete ridges with hyperpigmentation (“Dirty socks”)

+/- mild increase in melanocyte density

Solar elastosis in the dermis

62
Q

What is Cronkite Canada syndrome?

A

Happens in 50-60 y/o, extremely rare.

They get GI polyps, alopecia, lentigo simplex/mucosal melanocytic lesions, and basically FAP that is acquired. Happens in 50-60 y/o, extremely rare. They get GI polyps, alopecia, lentigo simplex/mucosal melanocytic lesions, and onychodystrophy.

63
Q

What is the difference between Hori’s nevus and Sun’s nevus?

A

Hori’s: acquired macules b/l zygomatic region; east Asian women

Sun’s Nevus: acquired, a unilateral variant of Hori’s nevus

64
Q

What is the difference between nevus of Ota and dermal melanocytosis on path?

A

Melanocytes are more numerous and more superficial in nevus of Ota.

65
Q

3 components of recurrent melanocytic nevus?

A

Dermal scar, atypical junctional melanocytic proliferation confined to the area above the dermal scar, and dermal nevus remnants below/adjacent to scar.

66
Q

What eye findings can occur in Nevus of Ota?

A

Glaucoma can develop in 10%

67
Q

What is the significance of concomitant BAP-1 loss in uveal melanoma?

A

Leads to increased risk of metastasis and death

68
Q

What is the presentation and histology of a common blue nevus?

A
  • Blue/gray macule or papule < 1cm
  • Elongated, dendritic melanocytes containing melanin pigment in the upper 2/3 of derms associated w/ sclerotic collagen *no junctional component*
69
Q

What is the clinical and histology of the cellular blue nevus?

A
  • Blue/gray/black plaques or nodules, larger often (1-3cm), favors buttocks or scalp
  • Dense proliferation of plump/fusiform pale gray melanocytes w/ little pigment + admixed dendritic melanocytes
  • Bulges into the subcutis (dumbell configuration)
70
Q

Which type of blue nevus is associated with epithelioid blue nevus?

A

Carney complex

71
Q

What type of blue nevus does malignant blue nevus/melanoma arise from?

A

Often arises within cellular blue nevi (Scalp #1)

72
Q

What is the histology of a balloon cell nevus?

A

Histology: >50% dermal melanocytes are “balloon cells” (large, pale, and polygonal melanocytes with foamy/vacuolated cytoplasm and variable pigmentation)

-This is a result of melanosome degeneration

Conventional nevus is always

73
Q

What is the most common location for Spitz nevus?

A

Most common on the lower extremities overall, in children can also be common on the head and neck

74
Q

Can Spitz nevi be diagnosed later in life?

A

They can occur, but if they are older than 30’s use caution, this has a higher likelihood of being a melanoma

75
Q

What is the clinical presentation of a Spitz nevus?

A

Rapidly growing pink-red papulonodule (usually <1cm)

76
Q

What is the histology of the Spitz nevus?

A

Symmetric and circumscribed, epidermal hyperplasia is present, large junctional nests w/ clefting around the whole nest (not the individual cells like discohesion in melanoma), Kamino bodies: pink clumps of BMZ material (collagen IV) within the epiderms

77
Q

What would suggest that a spitz nevus is actually a melanoma?

A

Mitoses in the deeper parts of the lesion, lack of cells “maturing” with depth, and loss of p16

FISH w/ homozygous loss of 9p11 –> corresponds to p16/CDKN2a –> increased risk of metastasis and death

78
Q

What is the histology of pigmented spindle cell nevus of Reed?

A

Similar to a Spitz (related) but much heavier melanosome component and also the melanocytes are more superficial/kept to the junction

79
Q

Clinical presentation of deep penetrating nevus?

A

Well-circumscribed blue to black papule <1cm in size

Face, upper trunk and extremities; usually second and third decades

80
Q

What is the histology of the deep penetrating nevus?

A

Compound melanocytic proliferation with the minimal junctional component, superficial dermal nests resembling ordinary nevus nests

  • Prominent wedge-shaped dermal component that extends deep into the dermis or subcutis, tracks along with adnexal structures or neurovascular bundles
  • Has epithelioid pigmented melanocytes in loose nests with lots of melanophages
81
Q

What is neurocutaneous melanosis/melanocytosis and what is its relation to congenital melanocytic nevus?

A

Occurs w/ large congenital nevi posterior axial or multiple satellitis it is a melanocytic proliferation within the leptomeninges and brain parenchyma can have a high mortality in symptomatic patients

82
Q

What is the histology of congenital melanocytic nevi?

A

Compound or intradermal melanocytic proliferation. The dermal component extends deeper and can surround infiltrates vessels, adnexal structures, muscle, and nerve (not concerning)

83
Q

What should be done if a pt has a congenital nevus that is posterior axial or has multiple satellites?

A

Recommend MRI screening for neurocutaneous melanosis

84
Q

What is the recommended excision margins for dysplastic nevi (if they need to be removed)?

A

2-3mm

85
Q

What is the histology of a Becker’s nevus?

A

Increased basal melanocytes, epidermal thickening (perhaps androgen-mediated), elongation of rete ridges, and dermal smooth muscle hamartoma-like changes

86
Q

Clinical presentation of Becker’s nevus?

A

Hyperpigmented plaque w/ thickening, irregularity, and/or hypertrichosis on upper torso most commonly

Can see ipsilateral breast hypoplasia in some patients and even skeletal defects or limb asymmetry rarely