20 - 108 - BENIGN EPITHELIAL TUMORS, HAMARTOMAS, AND HYPERPLASIAS Flashcards
1
Q
Hallmark histopathologic findings of seborrheic keratosis
A
acanthosis, papillomatosis, pseudo-horn cysts, and hyperkeratosis
2
Q
most frequently detected mutations in seborrheic keratosis
A
The most frequently detected are activating mutations in the FGFR3 and PIK3CA genes.
3
Q
Which of the following is true about lichen striatus?
a. It occurs most commonly on the trunk of children and adolescents.
b. The eruption is self-limited, with spontaneous regression within a few months to 2 years.
c. Histopathologically, it spares the eccrine coils
d. There is male predilection
A
B
A -Limb,
C. There is characteristic accentuation around eccrine coils,
D. There is female predilection
4
Q
Which is the following are true about nevus sebaceous except?
a. Cases are typically sporadic.
b. More than 90% are believed to harbor an HRAS mutation.
c. These lesions can be verrucous at birth, later becoming more subtle under the hormonal influences of puberty.
d. Nevus sebaceous syndrome is also called Schimmelpenning syndrome.
A
C
Page 1809. These lesions can be subtle at birth, later becoming more elevated and verrucous under the hormonal influences of puberty.
5
Q
What is the most common tumor found within a nevus sebaceous?
A
Trichoblastoma
Page 1810. The most common are trichoblastoma (7% of nevus sebaceous cases) and syringocystadenoma papilliferum (5%).
6
Q
Which of the following syndromes is associated with ILVEN?
a. Proteus Syndrome (AKT1 mutation)
b. Type 2 Segmental Cowden Disease (PTEN mutation)
c. CHILD mutation (NSHDL mutation)
d. Schimmelpening syndrome (HRAS/KRAS mutation)
A
c. CHILD mutation (NSHDL mutation)
7
Q
Syndromes associated with Keratinocytic epidermal nevi (nonepidermolytic)
A
- HRAS/FGFR3 mutation
- Proteus syndrome (AKT1 mutation)
- Type 2 segmental Cowden disease (PTEN mutation)
8
Q
Syndrome associated with ILVEN
A
CHILD syndrome (NSDHL mutation)
9
Q
Syndromes associated with Nevus sebaceous
A
- Schimmelpenning syndrome (HRAS/KRAS mutation)
- Phakomatosis pigmentokeratotica (HRAS mutation)
10
Q
Syndrome associated with Nevus comedonicus
A
Nevus comedonicus syndrome: ocular (eg, ipsilateral cataract, corneal erosion), skeletal, and CNS anomalies
11
Q
Epidermoid cysts are lined by epithelium resembling the ____________
A
infundibulum of the hair follicle
12
Q
Trichilemmal cysts arise from the _________
A
Outer root sheath
13
Q
True on the histopathology of steatocystoma multiplex except
a. absent granular layer
b. eosinophilic cuticle on the luminal side
c. sebaceous glands are found within the cyst lumen
d. composed of stratified, squamous epithelium
A
c. sebaceous glands are found within the cyst lumen
Page 1816. Sebaceous glands are characteristically located in the cyst wall, and keratin, oil, and hairs are typically found in the lumen.
14
Q
Branchial cysts located on the angle of the mandible arise from _______
A
first branchial cleft
angle of the mandible = first branchial cleft;
middle to lower third of the anterior border of the sternocleidomastoid = second branchial cleft
15
Q
most common acquired benign epidermal tumor of the skin
A
SEBORRHEIC KERATOSIS
16
Q
A
Seborrheic keratosis showing a rough surface and stuck-on appearance.
17
Q
A
18
Q
A
19
Q
A
20
Q
A
Dermatosis papulosa nigra.
21
Q
drug associated with “pseudo-sign of Leser-Trélat”
A
Chemotherapeutic agents, such as cytarabine
22
Q
most common associated malignancy in Leser-Trélat sign
A
The most common associated malignancy is gastrointestinal, followed by lymphoproliferative disease.
23
Q
A
Figure 108-8 Lichen striatus. Linear configuration of slightly scaling papules in a blaschkoid distribution on the leg.
24
Q
A
25
Solitary, shiny, erythematous or orange to brown, blanching, well-demarcated papule or nodule with **collarette of scale**
**Clear cell acanthoma** presents as a solitary, shiny, erythematous to brown, well-demarcated papule or nodule that blanches almost fully with pressure
26
Clear cell acanthoma is composed of distinctive, _______-rich keratinocytes
**glycogen-rich**
27
28
chronic tender nodule that develops most commonly on the helix of the ear in men older than 50 years of age
CHRONDRODERMATITIS NODULARIS HELICIS
29
■ Solitary focus of acantholytic dyskeratosis
■ Skin-colored, umbilicated papule on the head or neck of middle-aged and older individuals
**WARTY DYSKERATOMA**
30
**ACANTHOMA FISSURATUM (GRANULOMA FISSURATUM)**
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benign hamartomatous proliferations of the epithelium and adnexae that present at birth or early childhood.
**Epidermal nevi**
32
The most common variant of epidermal nevi, usually presenting at birth or before adolescence
**KERATINOCYTIC EPIDERMAL NEVUS**
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unilateral subtype of systematized Keratinocytic epidermal nevus
**nevus unius lateris**
35
bilateral subtype of Keratinocytic epidermal nevus
**ichthyosis hystrix**
36
variant of keratinocytic epidermal nevus that clinically appears psoriasiform (erythematous with thick scale) and can be very pruritic
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38
NEVUS SEBACEOUS
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term collectively describing several distinct syndromes that all demonstrate an epidermal nevus occurring in the context of other developmental abnormalities
**EPIDERMAL NEVUS SYNDROME**
43
The most common extracutaneous features of epidermal nevus syndrome involve what organs
**ocular, neurologic, and skeletal systems**
44
45
derived from the **infundibular portion of hair follicle** and thus show flattened surface epithelium and keratohyaline granules
**Epidermoid cysts**
46
ining resembles the **isthmic portion of hair follicle** and thus show a scalloped surface epithelium **lacking a granular layer**
**Trichilemmal cyst (Pilar Cyst)**
47
thought to be the result of plugging of a vellus hair follicle or eccrine sweat duct; resemble small epidermal cysts
**Milia**
48
a sebaceous duct cyst characterized by a waxy, eosinophilic cuticle lining the cyst and characteristic sebaceous glands in the cyst walls
**Steatocystoma**
49
: collections of epidermis located along embryologic fusion planes, most commonly on the forehead, lateral eye, or neck
**Dermoid cysts**
50
asymptomatic cysts caused by occlusion of branchial cleft sinuses that are located along the angle of the mandible if arising from the first branchial cleft and the middle to lower third of the anterior border of the sternocleidomastoid in cases arising from the second branchial cleft
**Branchial cysts**
51
epithelial invaginations located in the preauricular area that arise from the incomplete fusion of the first and second branchial arches in the preauricular area
**Preauricular cysts or sinuses**
52
What HPV subtypes have been detected in palmoplantar epidermoid cysts
**HPV types 57 and 60 DNA**
53
milia on the palate
**Epstein pearls**
54
Steacystoma can be found in what syndromes
**Alagille syndrome and pachyonychia congenita type II**
55
Eruptive lesions of seborrheic keratosis may be a sign of internal malignancy , what is this sign called?
Leser-Trélat sign
56
hallmark histologic findings of seborrheic keratosis
- hyperkeratosis
- acanthosis
- papillomatosis
- pseudohorn cyst
57
This medication may cause inflammation of preexisting SKs, which then become more clinically apparent (“pseudo-sign of Leser-Trélat”).
Chemotherapeutic agents, such as cytarabine
58
The most frequently detected Genetic Alteration Reported in Association with Seborrheic Keratosis
FGFR3 and PIK3CA
59
site of predilection of stucco keratosis
distal extremities, particularly the lower legs
60
multiple small 1- to 3-mm white to tan keratotic papules with predilection for the distal extremities, particularly the lower legs
STUCCO KERATOSIS
61
histologic term used to describe **intraepidermal whorls of banal keratinocytes**
CLONAL SEBORRHEIC KERATOSIS
62
Irritated seborrheic keratosis with surrounding eczematous changes is called?
Meyerson phenomenon
63
rare, idiopathic, papular eruption that usually resolves in 1 to 2 years
Lichen Striatus
The cause is unknown, and the disease is self-limiting.
64
lichen striatus usually occurs in what population and gender?
It occurs most commonly on the limbs of children and adolescents (5 - 15 years old) with a female predilection.
65
lichen striatus has been associated with what condition?
An association with atopic dermatitis has also been suggested
66
refers to a more extensively distributed variant of keratinocytic epidermal nevi
systematized epidermal nevus
67
More than 90% of nevus sebaceus are believed to harbor an what mutation
HRAS mutation
68
nevus sebaceous syndrome AKA
Schimmelpenning syndrome
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most common benign neoplasm associated with nevus sebaceus
Trichoblastoma
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most common malignant neoplasm associated with nevus sebaceus
Basal cell carcinoma
71
Nevus comedonicus syndrome is the association of nevus comedonicus with noncutaneous findings such as
- skeletal defects,
- cerebral abnormalities, and
- cataracts
72
a potential regulator of follicular homeostasis, has been postulated to be defective in nevus comedonicus
NEK9 Kinase
73
characterized by a segmental smooth muscle hamartoma in association with musculoskeletal defects such as ipsilateral breast hypoplasia
Becker nevus syndrome
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consists of an epidermal hamartoma with long, fine white hair in addition to ocular, neurologic, and skeletal deficiencies
Angora hair nevus syndrome
75
ILVEN is associated with what syndrome
CHILD syndrome (NSDHL mutation): ipsilateral bone, visceral, and neurologic defects; epidermal nevi lateralized with flexural affinity
76
Nevus sebaceous is associated with what syndromes
Schimmelpenning syndrome (HRAS/KRAS mutation): ocular (eg, lipodermoid, coloboma), cerebral, and skeletal anomalies
Phakomatosis pigmentokeratotica (HRAS mutation): CNS anomalies; speckled lentiginous nevi and epidermal nevi following checkerboard pattern
77
Keratinocytic epidermal nevi (nonepidermolytic) is associated with what syndromes?
Proteus syndrome (AKT1 mutation): palmoplantar cerebriform hyperplasia, asymmetrical macrodactyly, soft tissue overgrowth; epidermal nevi characteristically soft and flat
Type 2 segmental Cowden disease (PTEN mutation): focal segmental glomerulosclerosis, soft tissue overgrowth, colon polyps; epidermal nevi characteristically thick and papillomatous (also called linear Cowden nevi)
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79
branchial cyst located along the angle of the mandible arises from the?
first branchial cleft
80
branchial cyst located on the middle to lower third of the anterior border of the sternocleidomastoid arises from the?
second branchial cleft
81
branchial cyst arising from the first branchial cleft will be located where?
along the angle of the mandible
82
branchial cyst arising from the second branchial cleft will be located where?
middle to lower third of the anterior border of the sternocleidomastoid
83
What HPV types have been detected in palmoplantar epidermoid cysts?
HPV types 57 and 60 DNA
84
These genetic disorders may predispose individuals to having epidermoid cyst.
- Gorlin syndrome (nevoid BCC syndrome)
- Pachyonychia congenita type 2 (Jackson-Lawler type),
- Gardner syndrome
85
Arise from the epithelium located between the orifice of the sebaceous gland and the arrector pili muscle. This squamous epithelium undergoes rapid keratinization, resulting in a cyst wall without a granular layer.
TRICHILEMMAL CYST (PILAR CYST )
86
Trichilemmal cysts arise from what part of the hair follicle ?
outer root sheath
87
plaque-type, inflammatory variant of milia that is commonly located on the ear
Milia en plaque
88
Steatocystoma multiplex if familial, is inherited in what mode of inheritance?
Autosomal Dominant
89
epithelial-lined cysts containing various appendageal structures resulting from retained epithelium along embryonic fusion planes. These cysts are either congenital or develop in childhood
DERMOID CYST
90
most common benign epidermal tumor
Seborrheic keratosis
91
* clinically present as pigmented patches or thin papules
* Histologically, these epidermal tumors are composed of **interconnected** delicate downgrowths of pigmented basilar epithelial cells with horn cysts
* In some instances, they may** arise from a solar lentigo.**
Reticulated (adenoid) SKs
92
* present as multiple small 1- to 3-mm white to tan keratotic papules with predilection for the distal extremities, particularly the** lower legs**
* Histologically, they show orthokeratosis, papillomatosis, and acanthosis with** fusion of rete pegs.**
Stucco keratoses
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* Clinically, this lesion may resemble melanoma and is commonly seen on the head and neck or trunk of older adults.
* It is characterized by an acanthotic **proliferation of basaloid and spinous keratinocytes**, often with horn cysts.
* Between keratinocytes, there are **sporadically distributed pigmented dendritic melanocytes**.
* **Melanin-laden histiocytes** are often present in the dermis.
MELANOACANTHOMA
94
* most commonly presents as multiple small dark brown to black keratotic papules on the malar region of individuals with **Fitzpatrick skin Type IV or greater**
* They may also occur on the neck and upper chest.
* Histologically, they show orthokeratosis, acanthosis, papillomatosis, and **interconnected rete with basal layer hyperpigmentation.**
Dermatosis papulosa nigra
95
area of predilection of clear cell acanthoma
lower legs
95
Similar to skin tags, SKs may assume a polypoid configuration, particularly on the neck or intertriginous areas.
POLYPOID SEBORRHEIC KERATOSIS
96
presents as a solitary, shiny, erythematous to brown, well-demarcated papule or nodule that **blanches almost fully with pressure**
Clear cell acanthoma
97
characterized by a sharply demarcated zone of **pale staining epidermal keratinocytes** with psoriasiform epidermal hyperplasia and a parakeratotic scale
Clear cell acanthoma
## Footnote
Keratinocyte **pallor** is because of heavy cytoplasmic **glycogen**, as evidenced by periodic acid–Schiff positivity, which can be digested with diastase.
98
refers to a more extensively distributed variant of keratinocytic epidermal nevi
systematized epidermal nevus
99
Activating mutations of keratinocytic epidermal nevi
40% of keratinocytic epidermal nevi harbor **RAS mutations (usually HRAS)**, and another 40% have an **FGFR3 or PIK3CA **mutation
100
This variant of epidermal nevus demonstrates marked orthokeratosis with **vacuolization** and **coarse keratohyalin granule deposition within the granular layer**
The **epidermolytic variant** demonstrates marked orthokeratosis with vacuolization and coarse keratohyalin granule deposition within the granular layer, defined as **epidermolytic hyperkeratosis.**
101
If histopathologic evaluation of an epidermal nevus reveals features of epidermolytic hyperkeratosis, the patient is at risk of having a child with what condition?
the patient is at risk of having a child with **epidermolytic ichthyosis** if the postzygotic mutation for **K1 or K10 **is also present in gonadal tissue.
102
hairless waxy yellow linear plaque presenting on the head and neck between birth and adolescence
Nevus sebaceus
103
histopathologic findings of nevus sebaceus
* Histologic findings consist of **epidermal hyperplasia, immature and vellus hair follicles**, **ectopic apocrine glands, and sebaceous glands** located in the superficial dermis (Fig. 108-16).
* In prepubescent patients, the sebaceous glands usually appear immature and decreased in number.
104
histology shows keratin-filled epidermal invaginations associated with **atrophic** pilosebaceous units/ sebaceous glands or follicles
NEVUS COMEDONICUS
105
Nevus comedonicus syndrome is the association of nevus comedonicus with noncutaneous findings such as..
skeletal defects, cerebral abnormalities, and cataracts
106
well-known variant of epidermal nevus characterized by a **segmental smooth muscle hamartoma** in association with **musculoskeletal defects such as ipsilateral breast hypoplasia.**
Becker nevus syndrome
107
consists of an epidermal hamartoma with long, fine white hair in addition to ocular, neurologic, and skeletal deficiencies.
Angora hair nevus syndrome
108
histopath findings of dermoid cyst
* Cysts are lined by stratified squamous epithelium and typically contain keratin and hairs.
* **Adnexal structures are characteristically present in the cyst walls** and may include pilosebaceous units, apocrine or eccrine glands, smooth muscle bundles, or goblet cells.
109
* lined with epithelium containing a lymphocytic infiltrate and lymphoid follicles are common.
* Mucous glands may be found in the walls, and in these cases, the cyst contents are often mucinous.
Branchial cleft cysts
110
Preauricular cysts or sinuses have been associated with what conditions?
branchial cleft anomalies and hearing and renal abnormalities
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