20 - 108 - BENIGN EPITHELIAL TUMORS, HAMARTOMAS, AND HYPERPLASIAS Flashcards
Hallmark histopathologic findings of seborrheic keratosis
acanthosis, papillomatosis, pseudo-horn cysts, and hyperkeratosis
most frequently detected mutations in seborrheic keratosis
The most frequently detected are activating mutations in the FGFR3 and PIK3CA genes.
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
B
A -Limb,
C. There is characteristic accentuation around eccrine coils,
D. There is female predilection
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.
C
Page 1809. These lesions can be subtle at birth, later becoming more elevated and verrucous under the hormonal influences of puberty.
What is the most common tumor found within a nevus sebaceous?
Trichoblastoma
Page 1810. The most common are trichoblastoma (7% of nevus sebaceous cases) and syringocystadenoma papilliferum (5%).
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)
c. CHILD mutation (NSHDL mutation)
Syndromes associated with Keratinocytic epidermal nevi (nonepidermolytic)
- HRAS/FGFR3 mutation
- Proteus syndrome (AKT1 mutation)
- Type 2 segmental Cowden disease (PTEN mutation)
Syndrome associated with ILVEN
CHILD syndrome (NSDHL mutation)
Syndromes associated with Nevus sebaceous
- Schimmelpenning syndrome (HRAS/KRAS mutation)
- Phakomatosis pigmentokeratotica (HRAS mutation)
Syndrome associated with Nevus comedonicus
Nevus comedonicus syndrome: ocular (eg, ipsilateral cataract, corneal erosion), skeletal, and CNS anomalies
Epidermoid cysts are lined by epithelium resembling the ____________
infundibulum of the hair follicle
Trichilemmal cysts arise from the _________
Outer root sheath
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
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.
Branchial cysts located on the angle of the mandible arise from _______
first branchial cleft
angle of the mandible = first branchial cleft;
middle to lower third of the anterior border of the sternocleidomastoid = second branchial cleft
most common acquired benign epidermal tumor of the skin
SEBORRHEIC KERATOSIS
Seborrheic keratosis showing a rough surface and stuck-on appearance.
Dermatosis papulosa nigra.
drug associated with “pseudo-sign of Leser-Trélat”
Chemotherapeutic agents, such as cytarabine
most common associated malignancy in Leser-Trélat sign
The most common associated malignancy is gastrointestinal, followed by lymphoproliferative disease.
Figure 108-8 Lichen striatus. Linear configuration of slightly scaling papules in a blaschkoid distribution on the leg.
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
Clear cell acanthoma is composed of distinctive, _______-rich keratinocytes
glycogen-rich
chronic tender nodule that develops most commonly on the helix of the ear in men older than 50 years of age
CHRONDRODERMATITIS NODULARIS HELICIS
■ Solitary focus of acantholytic dyskeratosis
■ Skin-colored, umbilicated papule on the head or neck of middle-aged and older individuals
WARTY DYSKERATOMA
ACANTHOMA FISSURATUM (GRANULOMA FISSURATUM)
benign hamartomatous proliferations of the epithelium and adnexae that present at birth or early childhood.
Epidermal nevi
The most common variant of epidermal nevi, usually presenting at birth or before adolescence
KERATINOCYTIC EPIDERMAL NEVUS
unilateral subtype of systematized Keratinocytic epidermal nevus
nevus unius lateris
bilateral subtype of Keratinocytic epidermal nevus
ichthyosis hystrix
variant of keratinocytic epidermal nevus that clinically appears psoriasiform (erythematous with thick scale) and can be very pruritic
NEVUS SEBACEOUS
term collectively describing several distinct syndromes that all demonstrate an epidermal nevus occurring in the context of other developmental abnormalities
EPIDERMAL NEVUS SYNDROME
The most common extracutaneous features of epidermal nevus syndrome involve what organs
ocular, neurologic, and skeletal systems
derived from the infundibular portion of hair follicle and thus show flattened surface epithelium and keratohyaline granules
Epidermoid cysts
ining resembles the isthmic portion of hair follicle and thus show a scalloped surface epithelium lacking a granular layer
Trichilemmal cyst (Pilar Cyst)
thought to be the result of plugging of a vellus hair follicle or eccrine sweat duct; resemble small epidermal cysts
Milia
a sebaceous duct cyst characterized by a waxy, eosinophilic cuticle lining the cyst and characteristic sebaceous glands in the cyst walls
Steatocystoma
: collections of epidermis located along embryologic fusion planes, most commonly on the forehead, lateral eye, or neck
Dermoid cysts
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
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
What HPV subtypes have been detected in palmoplantar epidermoid cysts
HPV types 57 and 60 DNA
milia on the palate
Epstein pearls
Steacystoma can be found in what syndromes
Alagille syndrome and pachyonychia congenita type II
Eruptive lesions of seborrheic keratosis may be a sign of internal malignancy , what is this sign called?
Leser-Trélat sign
hallmark histologic findings of seborrheic keratosis
- hyperkeratosis
- acanthosis
- papillomatosis
- pseudohorn cyst
This medication may cause inflammation of preexisting SKs, which then become more clinically apparent (“pseudo-sign of Leser-Trélat”).
Chemotherapeutic agents, such as cytarabine
The most frequently detected Genetic Alteration Reported in Association with Seborrheic Keratosis
FGFR3 and PIK3CA
site of predilection of stucco keratosis
distal extremities, particularly the lower legs
multiple small 1- to 3-mm white to tan keratotic papules with predilection for the distal extremities, particularly the lower legs
STUCCO KERATOSIS
histologic term used to describe intraepidermal whorls of banal keratinocytes
CLONAL SEBORRHEIC KERATOSIS
Irritated seborrheic keratosis with surrounding eczematous changes is called?
Meyerson phenomenon
rare, idiopathic, papular eruption that usually resolves in 1 to 2 years
Lichen Striatus
The cause is unknown, and the disease is self-limiting.
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.
lichen striatus has been associated with what condition?
An association with atopic dermatitis has also been suggested
refers to a more extensively distributed variant of keratinocytic epidermal nevi
systematized epidermal nevus
More than 90% of nevus sebaceus are believed to harbor an what mutation
HRAS mutation
nevus sebaceous syndrome AKA
Schimmelpenning syndrome
most common benign neoplasm associated with nevus sebaceus
Trichoblastoma
most common malignant neoplasm associated with nevus sebaceus
Basal cell carcinoma
Nevus comedonicus syndrome is the association of nevus comedonicus with noncutaneous findings such as
- skeletal defects,
- cerebral abnormalities, and
- cataracts
a potential regulator of follicular homeostasis, has been postulated to be defective in nevus comedonicus
NEK9 Kinase
characterized by a segmental smooth muscle hamartoma in association with musculoskeletal defects such as ipsilateral breast hypoplasia
Becker nevus syndrome
consists of an epidermal hamartoma with long, fine white hair in addition to ocular, neurologic, and skeletal deficiencies
Angora hair nevus syndrome
ILVEN is associated with what syndrome
CHILD syndrome (NSDHL mutation): ipsilateral bone, visceral, and neurologic defects; epidermal nevi lateralized with flexural affinity
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
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)
branchial cyst located along the angle of the mandible arises from the?
first branchial cleft
branchial cyst located on the middle to lower third of the anterior border of the sternocleidomastoid arises from the?
second branchial cleft
branchial cyst arising from the first branchial cleft will be located where?
along the angle of the mandible
branchial cyst arising from the second branchial cleft will be located where?
middle to lower third of the anterior border of the sternocleidomastoid
What HPV types have been detected in palmoplantar epidermoid cysts?
HPV types 57 and 60 DNA
These genetic disorders may predispose individuals to having epidermoid cyst.
- Gorlin syndrome (nevoid BCC syndrome)
- Pachyonychia congenita type 2 (Jackson-Lawler type),
- Gardner syndrome
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)
Trichilemmal cysts arise from what part of the hair follicle ?
outer root sheath
plaque-type, inflammatory variant of milia that is commonly located on the ear
Milia en plaque
Steatocystoma multiplex if familial, is inherited in what mode of inheritance?
Autosomal Dominant
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
most common benign epidermal tumor
Seborrheic keratosis
- 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
- 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
- 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
- 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
area of predilection of clear cell acanthoma
lower legs
Similar to skin tags, SKs may assume a polypoid configuration, particularly on the neck or intertriginous areas.
POLYPOID SEBORRHEIC KERATOSIS
presents as a solitary, shiny, erythematous to brown, well-demarcated papule or nodule that blanches almost fully with pressure
Clear cell acanthoma
characterized by a sharply demarcated zone of pale staining epidermal keratinocytes with psoriasiform epidermal hyperplasia and a parakeratotic scale
Clear cell acanthoma
Keratinocyte pallor is because of heavy cytoplasmic glycogen, as evidenced by periodic acid–Schiff positivity, which can be digested with diastase.
refers to a more extensively distributed variant of keratinocytic epidermal nevi
systematized epidermal nevus
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
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.
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.
hairless waxy yellow linear plaque presenting on the head and neck between birth and adolescence
Nevus sebaceus
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.
histology shows keratin-filled epidermal invaginations associated with atrophic pilosebaceous units/ sebaceous glands or follicles
NEVUS COMEDONICUS
Nevus comedonicus syndrome is the association of nevus comedonicus with noncutaneous findings such as..
skeletal defects, cerebral abnormalities, and cataracts
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
consists of an epidermal hamartoma with long, fine white hair in addition to ocular, neurologic, and skeletal deficiencies.
Angora hair nevus syndrome
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.
- 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
Preauricular cysts or sinuses have been associated with what conditions?
branchial cleft anomalies and hearing and renal abnormalities
