Chapter 122 Benign Neoplasias and Hyperplasias of Melanocytes

Question 1

The term _________ is used to describe the presence of melanocytic cells in epidermal nests [defined as_________________ in direct contact (also known as _____)], within the dermis, or in other tissues

Answer 1

melanocytic neoplasia
three or more melanocytic cells
thèque

Question 2

The term _____________ is used to indicate increased melanocytes confined to the basal layer of the epidermis

Answer 2

melanocytic hyperplasia

Question 3

The vast majority of CNNs noted at ____ are small and singular, and no __________

According to other studies, the prevalence of CNN appears to be slightly higher in ______________

Answer 3

birth
gender predilection

nonwhites compared to whites

Question 4

CNNs:
This is presumably due to a mutation (_____) that occurs in a progenitor cell that results in the abnormal extensive accumulation of ______ along migration pathways during normal development.

CNNs in Eyelids:
it may be presumed that the nevomelanocytes migrated into this location sometime during or after eyelid fusion but before the eyelids split which is ______

Answer 4

often NRAS
melanocytic cells

before 24 weeks / 6th uterine month

Question 5

CNN:
rare relatively large nevomelanocytic nevi (____ cm) that appear for the first time__________, according to parental observations and corroborated by photographs (__________)

disproportionate growth, especially _____mos, or change in a nonuniform manner is of concern for possible _______

Answer 5

> 1.5 cm
between 1 month and 2 years of life
tardive congenital nevi

after 6 months
melanoma

Question 6

CNN:
Lesions attaining a diameter of _______ are likely to be congenital, atypical melanocytic nevi, or melanoma

Arbitrary size criteria have also considered small (___cm), medium (____cm), and large (____cm)

Giant –> defined as a lesion ___________ if it occurs on the head and neck (and twice that area for other anatomic sites), ______ or ________ in adults (or smaller if it involves a major anatomic area)

Answer 6

1.5 cm or more

<1.5
1.5–19.9
≥20

as large as the patient’s palm
30% of the body surface, or 900 cm2

Question 7

CNN:
Dermoscopy may reveal a _____ or _____ pattern and may be useful in the identification of small foci of _______

CNNs of the head, neck, or posterior midline, and/or the presence of multiple satellite lesions associated with large CNN may be complicated by underlying _______________

Answer 7

reticular pattern or globular/cobblestone
melanoma

cranial and/or spinal leptomeningeal melanocytosis

Question 8

CNN:
Tumors indistinguishable from _______________________ may develop in association with giant varieties of CNN

Malignant degeneration of large CNNs may be
associated with the relatively sudden appearance of ____________________

Answer 8

neurofibroma in the absence of von Recklinghausen neurofibromatosis

a dermal or subcutaneous nodule, very dark pigmentation, itching, pain, bleeding, or ulceration

Question 9

CNNs are characterized by the presence of nevomelanocytes in the epidermis as______________ and/or nevomelanocytes in the dermis, which are present as ____________________

Nevomelanocytic type CNN:
CNNs are more likely than acquired nevi to have nevomelanocytes in the_________________ and to be associated with_______________ structures

Answer 9

well-ordered thèques
sheets, nests, cords, and/ or single cells

lower two-thirds of the reticular dermis
appendageal and neurovascular

Question 10

neuroid type of giant CNN:
melanocytic elements take on the appearance of____________________ (lames foliacée), a palisaded arrangement of cells around a cellular mass of homogeneous material (_________), and sheathing of nerves by neuroid tissue (neuroid tubes)

spindle cell &/or epithelioid cell type of giant CNN:
may have atypical cellular and architectural features, making differentiation from _________ extremely difficult

blue (dermal melanocytic) type of giant CNN:
heavily pigmented ___________ or intermixed with nevomelanocytes in the reticular dermis or deeper tissues. There may be some biologic overlap with___________ and __________

Answer 10

Wagner–Meissner corpuscles
Verocay body

melanoma

spindle-shaped melanocytic cells alone
nevus of Ito/Ota and Mongolian spot

Question 11

CNN:
The cumulative 5-year risk has been calculated to be ________% in patients with congenital nevi that involve ______ of the body surface

Melanoma may develop in large CNNs at any time, but the diagnosis of melanoma was established in the ________ in approximately one-half of published cases in which patients ultimately developed melanoma in association with giant CNN

Answer 11

2.3% and 5.7
over 5%

first 3–5 years of life

Question 12

CNN:
excision of large CNNs should be considered as early as possible, but it is probably prudent to wait until____________ to reduce surgical and anesthetic risks

Unlike surgical excision,_____________ do not address the malignant potential of CNN; nevomelanocytes may still be left behind in the dermis, and the cosmetic results associated with destructive therapy are unpredictable.

Answer 12

after the first 6 months of life

dermabrasion and other modes of destructive therapy

Question 13

Nevus spilus:
More commonly, the lesion becomes evident during ________________

The tan macular background pigmentation ranges from _____in diameter. Although nevus spilus may occur anywhere, lesions have been noted primarily on the ____________.

Answer 13

infancy or early childhood

less than 1 cm to greater than 10 cm
torso and extremities

Question 14

Nevus spilus:
The tan background pigmentation usually consists of increased numbers of melanocytes in a _______________ pattern

nevus spilus lesions are presumed to ___________, although it is possible that some elements within the nevus spilus or even the entire nevus spilus itself could regress with time

Answer 14

lentiginous epidermal

persist throughout life

Question 15

majority of common acquired nevi appear to develop during the_________ decades of life, although some lesions may appear in the first 3–6 months of life

Acquired nevi may be attacked by the patient’s immune system, resulting in the development of a _______

Answer 15

second and third

halo nevus

Question 16

common acquired nevi :
the _____ mutation that is present in the majority of common acquired (including atypical) nevi appears to be polyclonal

In these patients (eruptive nevi), there is the abrupt appearance of numerous similar appearing nevi often in the setting of________ or _______

Answer 16

B-RAF

immunosuppression or chemotherapy

Question 17

common acquired nevi :
growing lesion in an older adult has a greater risk of being ______

Spontaneous and concurrent development of scattered nevomelanocytic nevi often similar in appearance has been termed _________

Answer 17

melanoma

eruptive nevi

Question 18

common acquired nevi :

halo nevus may be associated with acquired and CNN as well as with_____________ and ________

Answer 18

melanoma and nonmelanocytic tumors

Question 19

common acquired nevi:
More elevated and less pigmented lesions tend to have a prominent_________ nevus component, whereas flatter and darker lesions have a more prominent__________ or _______ component and a less prominent dermal component

Answer 19

intradermal

junctional melanocytic or nevomelanocytic

Question 20

common acquired nevi:
(Dermoscopy) In general, these lesions reveal a ______ or _______ pattern. On the palms or soles, a parallel-furrow, _____ or ____ pattern may be present. Nonuniform patterns and parallel-ridge patterns are worrisome for _______

(halo nevus)
Any nevus in any anatomic site may be involved, but the _________ is involved most commonly. Lesions are usually asymptomatic.

Answer 20

reticular or globular
lattice, or fibrillar
melanoma

posterior torso

Question 21

common acquired nevi:
A second process that may provide some insight into factors involved in melanocytic interactions with the immune system is a process that results in the development of an eczematous dermatitis presenting as a red halo around nevi (_____, ______)

For patients with eruptive nevi, there may be findings suggestive of a _________ or _____. For patients with halo nevi, the most common associated condition is _____, occurring in 18% to 26% of patients

Answer 21

halo dermatitis, Meyerson’s nevus

blistering disease or immunosuppression.
vitiligo

Question 22

common acquired nevi:
Epidermal nevomelanocytes are arranged in ______ surrounded by a smooth perimeter of epidermis, and the epidermis is separated from nevomelanocytes by a _____________

Melanin-laden macrophages are usually apparent in the ________________ of nevi, their number usually proportional to degree of melanin production

Answer 22

nests
retraction artifact

superficial papillary dermis

Question 23

common acquired nevi:
Intradermal nevi that have few or no junctional nests frequently have a __________ relatively free of nevomelanocytes just below the epidermis

Nevomelanocytes in the deep dermis may be disposed within a collagenous framework that is loose, pale, and wavy in formations called ________, similar to a neurofibroma

Answer 23

grenz zone

neuroid tubes

Question 24

common acquired nevi:
balloon cell nevus ->In addition to clear cells with single basophilic nuclei, ______ and ________ are seen frequently.

Most ____________ are found in association with a benign compound nevus (acquired or congenital)

Answer 24

multinucleated balloon cells and multinucleated giant cells

combined blue nevi

Question 25

common acquired nevi:
_____________ (pseudomelanoma) is the name given to recurrent lesions after incomplete removal of a benign nevomelanocytic nevus

________ and _________, present occasionally in nevi, also may be related to degeneration or regression

Answer 25

Recurrent melanocytic nevus

Psammoma bodies and amyloid bodies

Question 26

common acquired nevi:
Shrinkage clefts may resemble _____ or ______, prominent in the _________ and particularly in areas with hemorrhage

______ and ______ in dermal (deeper) cells may help confirm a benign diagnosis

Answer 26

lymphatics or vascular spaces
midportion of nevi

Low Ki67 activity and loss of HMB45

Question 27

common acquired nevi:
acquired nevi evolve through a life cycle, first becoming apparent after _____ in the vast majority of cases, peaking in number during the second and third decades of life, and then disappearing by the_________________

Answer 27

infanc

seventh to ninth decades

Question 28

common acquired nevi:
______, ______ and _______ may occur more frequently in individuals with halo nevi

______ nevi -> link the initiating event to a blistering skin disease, systemic immunosuppression, chemotherapy, or treatment with “biologics.”

Answer 28

Atypical nevi, melanoma, and vitiligo

eruptive

Question 29

common acquired nevi:
Complete removal of nevi is best accomplished by _____.

Sensible UVR exposure includes confining outdoor activities to the early morning or late afternoon/evening, and avoiding the most intense UVR fluence occurring _______________

Answer 29

excision

2 hours either side of high noon.

Question 30

Blue nevi:
Multiple blue nevi may be associated with ____, ______, and mucocutaneous myxomas

A specific mutation in the ______ has been identified in blue nevi

Answer 30

lentigines, cardiac myxoma
[Carney complex/LAMB syndrome (lentigines, atrial myxomas, mucocutaneous myxomas, and blue nevi)]

GNAQ gene

Question 31

Blue nevi:
Nevus of Ota and Ito may share similar underlying defects to blue nevi and are also often acquired (in early childhood, _____________) but are far more extensive, encompassing a portion of _____ (_______) and _____ (_____ and ____)nerve distributions, respectively.

Answer 31

often before age 1 year or around puberty
trigeminal (ophthalmic and maxillary)
brachial (posterior supraclavicular and lateral cutaneous)

Question 32

Blue nevi:
The blue–gray color of blue nevi is an optical effect of blue light backscatter from the skin over the dermal melanin (_________)

half the reported cases present on the dorsa of ______

The large plaque blue nevus (pilar neurocristic hamartoma) may occasionally be associated with a background of ___________

Answer 32

Tyndall effect

hands and feet

lentigo simplex

Question 33

Blue nevi:
Deep penetrating nevi are darkly pigmented, blue–black papules or nodules, mostly on the _____________________, 2–9 mm in diameter, and occurring predominantly in the first four decades of life, including childhood

Cellular blue nevus differs from the common blue nevus in that it is usually larger, more elevated, more ______ and occasionally associated with _____________.” Complicating the picture is the fact that ______ may develop in a cellular blue nevus

Answer 33

head and neck or upper extremities

aggressive locally
lymph node “benign metastasis
melanoma

Question 34

Blue nevi:
In common blue nevi, dermal melanocytes appear as_____________ grouped in irregular bundles admixed with melanin-containing macrophages, associated with ______________ in the middle or upper reticular dermis,

Pathology of the plaque-like blue nevus (pilar neurocristic hamartoma) reveals a peripilar grouped arrangement of mostly____________, patterns of a common blue nevus and dermal melanocytosis (Mongolian spot), and presence of abnormal (granular) melanosomes

Answer 34

melanin-containing fibroblast- like cells
excessive fibrous tissue

spindle cells containing varying amounts of melanin

Question 35

Blue nevi:
Epithelioid blue nevi have been reported in association with ________ or in isolation

A major complication of blue nevi is the potential risk of _____. This risk appears to be highest for ______ blue nevi.

Answer 35

cardiac myxoma

melanoma
cellular

Question 36

Blue nevi:
Sudden appearance of a blue nodule, ______ of a preexisting blue nodule, a ________ blue nodule, or a relatively large blue nodule or plaque greater than _____ in diameter should be considered for histopathologic examination

Answer 36

expansion
congenital
10 mm

Question 37

PIGMENTED SPINDLE CELL NEVUS:
There is a preference for the lower extremity especially the ____, with the _____ being the next most common site

lesions are uniformly dark, with a sharp interface with surrounding skin often exhibiting streaks/pseudopods and giving the lesion a _____________

Answer 37

thigh
back

**Female patients outnumber male patients

starburst appearance

Question 38

PIGMENTED SPINDLE CELL NEVUS:
PSCN consists of vertically oriented fascicles of spindle-shaped, pigment-producing _________; some pagetoid upward migration may be present, but the lesion can be distinguished from melanoma by uniform _____, uniform _________, and distinctive pattern of growth.

these lesions should be excised with ________ margins of normal skin

Answer 38

melanocytes
nuclei
cellular detail

3–5 mm

Question 39

Spitz nevus:
Amplifications of _____ and _____ and activating mutations of _____ have been noted in a subset of Spitz nevi

duration of solitary Spitz nevus before presentation is usually less than ________

Answer 39

chromosome 11p and H-RAS
H-RAS

9 months

Question 40

Spitz nevus:
most common variety of Spitz nevus is solitary, ____, ____, ____, firm, and dome-shaped

__________ often occur in the early years of life within a background of congenital (sometimes acquired) macular pigmentation (_______) or occasionally within a hypopigmented plaque.

Answer 40

asymptomatic, pink or red, hairless,

Agminated Spitz nevi
nevus spilus

Question 41

Spitz nevus:
Unlike ordinary nevi and melanomas, melanocytic cells in Spitz nevi are ____ - ________ ________, with prominent mononuclear or multinucleated giant cells in the epidermis and/or dermis

Coalescent eosinophilic globules (______), periodic acid-Schiff-______ and diastase-______ (resembling colloid bodies), have been reported in 60% of Spitz nevi

Answer 41

large—often twice the size of epidermal basal keratinocytes

Kamino bodies
positive
resistant

Question 42

Spitz nevus:
Based on patients with eruptive Spitz nevi, it is clear that ___________ can occur

Complete excision with a ________ clear margin of normal skin is generally sufficient treatment for Spitz nevi

Answer 42

spontaneous regression

3–5 mm

Question 43

Nodal nevi:
Nevomelanocytes can be identified in lymph nodes with a frequency ranging from________% in lymphadenectomies not related to melanoma and up to 22% in regional nodes removed because of melanoma

The first theory is that during development, nevomelanocytes _______ in developing nodal tissues

The second theory suggests that there may be _______ from the cutaneous lesion to the lymph node

Answer 43

0.3% to 7.3%

get trapped

passive transfer

Question 44

Nodal nevi:
Lack of proliferative markers _____ and possibly high levels of _____ immunohisto- chemical markers support a benign diagnosis

Answer 44

(Ki67)

p16

Question 45

Lentigo simplex:
The increased density of melanocytes in lentigines is presumably due to an underlying developmental or intrinsic defect in _________

Lentigo simplex may appear as ___________ and may occur anywhere on skin or mucous membranes

Answer 45

melanocyte homeostasis

early as the first decade

Question 46

Lentigo simplex:
__________ may occur as an isolated phenomenon without known familial aggregation and first appear at birth, during infancy, or during adulthood.

Answer 46

Generalized lentigines

Question 47

Lentigo simplex:
In Moynahan (LEOPARD) syndrome, lentigines occur on \_\_\_\_\_\_\_ and \_\_\_\_\_\_\_\_ , including genitalia, conjunctiva, oral mucosa, palms, and soles. 

In Peutz–Jeghers syndrome, lentigines are almost always present on the _________

Answer 47

both sun-exposed and sun-protected sites

oral mucosa.

Question 48

Lentigo simplex:
Lentigines in the myxoma syndrome (LAMB) occur mainly on the ____ and _____ as tan to black macules

Lentigines in Laugier– Hunziker pigmentation occur on the__________________, on fingertips and nail matrix

Answer 48

face and genitalia

buccal and labial mucosa of the mouth

Question 49

Lentigo simplex:
_________________ may be required to differentiate agminated lentigines from nevus spilus

____, ____ and ____ histochemistry may be useful to demonstrate increased number of melanocytes confined to the basal layer and lacking junctional nest formation

Answer 49

Wood’s lamp examination

Mart-1, Mel-5, and DOPA

Question 50

Lentigo simplex:
Cosmetic removal may be achieved with
_____ or other destructive approaches such as ________.

Answer 50

cryotherapy
Q-switched laser

**There is a theoretical risk of malignant transformation of any variety of melanocytic hyperplasia or dysplasia using any type of laser

Question 51

Solar lentigo:
they are present in 90% of white people over ______ of age

Lentigines may be induced by photochemotherapy [psoralen and UVA light (PUVA) lentigo], occurring in 40% to 50% of patients an average of _______ after starting therapy,

Answer 51

60 years

5.7 years

Question 52

Solar lentigo:
Lesions similar in gross and microscopic appearance to solar lentigo, _________, may occur in children and young adults.

They may appear within the first ______ of life, tend to aggregate in families,

Answer 52

freckle (ephelis)

5 years

Question 53

Solar lentigo:
Single exposures of experimental UVB radiation (_____ mm) that is _______ times the minimal erythema dose induces “freckling” in susceptible individuals

Phototoxic doses of PUVA may lead to the development of lentigines ____ months later

Answer 53

320

six to ten

6–8

Question 54

Solar lentigo:
In xeroderma pigmentosum (XP), in which patients have an increased sensitivity to UVR, hyperpigmented macules develop on light-exposed skin within the first ______ of life, and these lesions demonstrate hyperplasia of variably atypical epidermal melanocytes (i.e., __________)

Answer 54

5 years

atypical lentigines

Question 55

Solar lentigo:
When examined by dermoscopy, solar lentigines reveal a uniform ________

Freckles are common on the _____ and often first noted in _______, presumably developing after significant sun exposure and are said to__________ when sun exposure is discontinued

Answer 55

reticular network

central face
early childhood
fade or even disappear

Question 56

Solar lentigo:
The presence of solar lentigines is a risk factor for ____, ____, _____

Solar lentigines reveal elongated epidermal _______ with club-shaped or bud- like extensions, frequent branching and fusing of rete ridges, a thinned or ________ between rete ridges, and increased numbers of _________ without nesting.

Answer 56

melanoma, basal cell cancer, and squamous cell cancer

rete ridges
atrophic epidermis
epidermal melanocytes

Question 57

Solar lentigo:
Electron microscopy -> Compared with melanocytes in sun-protected skin, melanocytes in solar lentigo reveal increased activity manifested by marked _______ (suggesting increased _____ activity),

Answer 57

DOPA reactivity

tyrosinase

Question 58

Solar lentigo:
Pathologic features of PUVA-induced lentigo include increased numbers of melanocytes in elongated ________, with large________

melanosomes in keratinocytes of PUVA lentigines are often______ instead of ________ as in solar lentigines

Answer 58

epidermal rete ridges
cell bodies

large and single
small and compound

Question 59

Solar lentigo:
____ and ___ histochemical studies may be useful to demonstrate increased number of melanocytes confined to the______ and a lack of __________ formation

Answer 59

Mart-1 and DOPA
basal layer
junctional nest

Question 60

Solar lentigo:
PUVA lentigines may persist ______ or longer after therapy is discontinued

_______ or other superficial destructive techniques (such as ________) may be an option if the lesion is clearly benign, but lesions may recur after therapy

Answer 60

1–2 years

Cryotherapy
Q-switched laser

Question 61

Solar lentigo:

______ supplementation needs to be considered for patients practicing UVR protection and avoidance

Answer 61

Vitamin D