25 - Benign Skin Neoplasms Flashcards

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1
Q

Skin is composed of 3 layers

A
  • Epidermis
  • Dermis
  • Hypodermis
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2
Q

Benign epithelial tumors – Seborrheic keratoses

A
  • Tumors occur most frequently in middle-aged or older individuals (rare before 30)
  • Round, flat, coin like, waxy plaques that vary in diameter from mm to several cm (with or without pigment)
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3
Q

Treatment of seborrheic keratoses

A
Not necessary unless lesions are bothersome
o	Surgery
o	Chemical peeling agents
o	Electrocautery
o	Cryotherapy
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4
Q

What do you NEED TO KNOW about seborrheic keratoses?

A
  • Separating these from melanoma can be tricky
  • VERY HARD TO LOOK AT SOMETHING AND KNOW WHAT IT IS
  • Biopsy is very important – don’t treat things until you have a diagnosis
  • Seborrheic keratoses can MIMIC OTHER LESIONS*** - NEED TO KNOW
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5
Q

Laser-Trelat sign

A

o Sudden onset of numerous seborrheic keratoses may indicate an underlying visceral malignancy

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6
Q

Biopsy image of seborrheic keratoses

A

o Can see microcysts of keratosis
o It is NOT invading the dermis – this is how you know it is not a squamous cell carcinoma
o If you do too shallow of a biopsy, the pathologist would not be able to distinguish
o Need to be able to see normal dermis

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7
Q

Fibroepithelial polyp-acrochordon

A
  • Morphology = fibrovascular core covered by benign squamous epithelium
  • Covered by a benign epithelial
  • Can sometimes be HPV driven tumors in the genital region
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8
Q

Epithelial cysts

A

Epithelial cysts are divided into several histological types:
o Epidermal inclusion cyst (Epidermoid Cyst)
o Pilar or trichilemmal cysts
o Dermoid cyst
o Steatocystoma multiplex

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9
Q

Epidermoid cyst

A

Not typically recommended to drain them – risk of infection, usually recur

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10
Q

Trichilemmal cyst

A
  • Tend to be seen on the scalp
  • Hot pink core – very densely compacted hyperkeratosis
  • Only exists in the hair follicle
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11
Q

Dermoid cyst

A
  • Common presentation: Single subcutaneous nodule at birth on lateral aspect of upper eyelid
  • Embryologically has a different origin
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12
Q

Steatocystoma

A
  • Multiplex (autosomal Dominant mutation in Keratin 17, seen in sternal, axillae, groin regions)
  • Simplex = solitary lesion
  • Kind of looks like an epidermal cyst, but there are attached sebaceous nodules
  • Very thin layer of keratosis over top
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13
Q

Tumors of the hair follicle

A
Types
o	Trichoadenoma
o	Trichilemmoma
o	Pilomatrixoma
o	Trichofolliculoma
o	Trichoepithelioma
o	Fibrofolliculoma
o	Trichodiscoma

We will only go through some of them

Some are associated with different mutations or cancers, so just know those

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14
Q

Cowden’s disease and trichilemmoma

A

There is a nodule of squamous cells with a clear, pale cytoplasm

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15
Q

Cowden’s Disease

A

o Autosomal Dominant disease with mutation of tumor suppressor PTEN
o High risk for breast and thyroid carcinomas
o Oral Lesions, acral keratoses, macrocephaly all seen

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16
Q

Trichilemmoma

A

Can be solitary or multiple (multiple is diagnostic of Cowden’s disease)

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17
Q

Pilomatrixoma

A
  • Solitary firm nodule on head>upper limbs>neck>trunk
  • Cheek most common location
  • 60% before age 20
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18
Q

Birt-Hogg-Dube Syndrome

A
  • Mutation to BHD gene on chromosome 17
  • Encodes protein folliculin – function unknown
  • Multiple fibrofolliculomas, trichodiscomas, and acrochordons
  • Association with internal disease
  • Renal tumors – benign and malignant
  • Lung disease – spontaneous pneumothorax
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19
Q

Benign tumors of sebaceous glands

A
  • Sebaceous hyperplasia
  • Sebaceous adenoma
  • Sebaceoma
  • Sebaceous carcinoma
  • Nevus sebaceous
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20
Q

Nevus sebaceous

A
  • Also called organoid nevus
  • Not a melanocytic proliferation
  • Often presents at birth, M=F
  • Presents on head/neck, particularly scalp
  • **SECONDARY TUMORS CAN ARISE WITHIN THESE ** This is why they are important clinically
    o Benign syringocystadenoma papilliferum and trichoblastoma
    o BCC
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21
Q

Sebaceous hyperplasia

A
  • Common on the face of older adults
  • Misdiagnosed as BCC
  • Yellowish dome shaped papules
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22
Q

Sebaceous adenoma

A
  • Rare, mistaken for BCC
  • Face and scalp of older people
  • Can be seen in Muir-Torre Syndrome
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23
Q

Sebaceoma

A
  • Intermediate aggression
  • No metastasis reported
  • Also related to MTS
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24
Q

Muir-Torre syndrome

A
  • Related to Hereditary Non-polyposis Colorectal Carcinoma cancer syndrome
  • Inherited defect in a DNA mismatch repair gene (MMR)
  • Loss of genetic stability during replication leads to microsatellite instability (repetitive sequences of DNA)
  • Most common gene MSH2
  • Autosomal Dominant
  • Need the family to be screen early for colorectal tumors
  • Notes: when mismatch repair genes are defective, they cannot repair inappropriate sequences – you get a buildup of mutations
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25
Q

Cutaneous findings in Muir-Torre syndrome

A

Sebaceous tumors

  • Adenoma, sebaceoma, and carcinoma
  • Keratoacanthoma
26
Q

Visceral malignancies in Muir-Torre syndrome

A

GI tract (colon)

  • Colon tumors tend to be more proximal
  • Tends to be less aggressive than other colon CA

Bladder

Endometrium

Breast

27
Q

Diagnosing MTS

A

o Immunostaining for Mismatch repair enzymes
o Loss of staining is ‘positive’
o Follow up positive result with genetic sequencing

28
Q

Benign tumors of sweat glands

A
  • Syringocystadenoma papilliferum
  • Nipple adenoma
  • Poroma
  • Chondroid Syringoma (mixed tumor)
  • Hidrocystoma
  • Syringoma
  • Hidradenoma
  • Hidradenoma papilliferum
  • Cylindroma
  • Spiradenoma
29
Q

Cylindroma

A
  • 90% occur on head and neck
  • Pink/red nodule
  • F:M 9:1
  • Brooke-Spiegler Syndrome*
  • Can be referred to as “Turban tumor”
  • On microscopy, appear like a “jigsaw puzzle”
30
Q

What is Brooke-Spiegler syndrome?

A
o	Familial cylindromas
o	Trichoepitheliomas
o	Milia
o	Spiradenomas
o	Autosomal Dominant: CYLD gene on chromosome 16 (lack of CYLD results in NF-kb pathway activation)
31
Q

Histiocytic “tumors”

A
  • Xanthomas/Xanthelasma
  • Juvenile Xanthogranuloma
  • Reticulohistiocytoma
  • Langerhans cell histiocytosis
32
Q

Xanthomas

A

Less a tumor and more of a benign aggregate of histiocytes with cytoplasmic lipid

Five clinical types
o	Eruptive
o	Tendinous
o	Tuberous
o	Planar
o	Disseminated (Only one with normal serum lipid levels)
33
Q

Xanthogranuloma

A
  • Often called Juvenile Xanthogranuloma (JXG)
  • Usually arises in first 5 years (20% of cases in young adults)
  • Solitary lesions on head/neck>trunk>upper limbs
  • Yellow brown/orange papule or nodule (Differential diagnosis: Spitz nevus, mastocytoma)
  • Rare association with chronic myelogenous leukemia (CML) and neurofibromatosis – boards question
  • Spontaneously regress within several years
  • JXG pathology (Granulomous eruption, “Tuton” giant cells)
34
Q

Reticulohistocytoma

A
  • Age: middle age
  • F>M
  • KEY WORD = “ground glass histiocytes” on microscopy

Two forms:
o Solitary
o Multicentric reticulohistiocytosis

35
Q

Multicentric reticulohistiocytosis

A
  • Cutaneous nodules, destructive arthritis, intermittent pyrexia
  • 25% have concurrent malignancy
  • Also associated with vasculitis and connective tissue disease
36
Q

Langerhans cell histocytosis (LCH)

A
  • Variable clinical presentation
  • All characterized by proliferation of Langerhans cell-type histiocytes in tissue

3 types
o Acute Generalized LCH (Letterer-Siwe Disease)
o Multifocal Chronic LCH (Hand-Schuller-Christian Disease)
o Focal chronic LCH (eosinophilic granuloma)

Most aggressive – high morbidity/mortality
o Skin and internal organ involvement
o Infancy

37
Q

Multifocal chronic LCH (Hand-Schuller-Christian disease)

A
  • First 5 years of life, can affect older
  • Multisystem but more chronic course
  • Regionally localized disease (head)
  • Triad: lytic skull lesions, diabetes insipidus, and proptosis
  • Treatment: radiation and chemotherapy
38
Q

Eosinophilic granuloma

A
  • Most benign – very good prognosis
  • Older children and adults
  • Solitary
  • Treatment: Excision or radiation
39
Q

LCH pathology

A
  • Classically have a nuclear groove
  • DC1a positive
  • “Birbeck granules” on electron microscopy
40
Q

Benign connective tissue tumors

A
  • Dermatofibroma
  • Lipoma
  • Hemangioma
  • Peripheral Nerve Tumors
  • Leiomyoma
  • Giant cell tumor of tendon sheath
  • Ganglion Cyst
  • Fibromatosis
  • Numerous Others
41
Q

Benign fibrous histiocytoma (dermatofibroma)

A
  • Refers to a heterogeneous family of morphologically and histogenetically related benign dermal neoplasms of fibroblasts and histiocytes
  • Firm, tan to brown papules = great mimicker of other things
  • Asymptomatic or tender, size may increase or decrease slightly over time
42
Q

Histogenesis of Benign fibrous histiocytoma (dermatofibroma)

A

o Remains a mystery

o Many cases have a history of antecedent trauma, suggesting an abnormal response to injury and inflammation

43
Q

Microscopy of Benign fibrous histiocytoma (dermatofibroma)

A
  • Microscopy shows dermis is affected by proliferation of spindle cells, lesion cells form “donut sign” of collagen trapping
44
Q

Induction phenomenon

A

o Looks like a basal cell carcinoma
o If you took a biopsy that is not deep enough, we would diagnose a basal cell carcinoma
o Need to get a deep enough shave biopsy

45
Q

Lipoma

A
  • Most common connective tissue tumor

- Benign, excision is curative

46
Q

Hemangioma

A

Tumors of benign blood vessels

Multiple subtypes
o Pyogenic granuloma is something related – it is ulcerated– common on nail bed
o Cavernous hemangioma
o Arteriobenous hemangioma

47
Q

Peripheral nerve tumors

A
Reactive Lesions and Hamartomas
o	Traumatic neuroma
o	Morton’s neuroma
o	Digital Pacinian Neuroma
o	Mucosal Neuroma
Benign Tumors
o	PEN
o	Schwannoma
o	Neurofibroma
o	Granular cell tumor
48
Q

Traumatic neuroma

A
  • Arise where a peripheral nerve is severed and does not heal (ie amputation)
  • Clinically painful nodule
  • Disorderly proliferation of bundles of peripheral nerves
  • All components: Schwann cells, nerve fibers, perineural cells, fibroblasts)
  • Basically normal nerves that are haphazardly arranged
49
Q

Morton’s neuroma

A
  • Severe pain in sole of foot
  • Region of metatarsal heads (high-heeled shoes)
  • Exacerbated by walking
  • Lesion is usually not palpable
50
Q

Mucosal neuroma

A
  • Typically multiple and clustered around the mouth
  • Part of multiple endocrine neoplasia syndrome (MEN IIb)
    o Patients should be evaluated for genetic testing and thyroid imaging
51
Q

Palisaded encapsulated neuroma (PEN)

A
  • Solitary painless nodule
  • Face is most common site
  • Although can be found on any mucocutaneous site
  • Middle aged-elderly
  • PEN on microscopy = Well-circumscribed, subtotal capsule, nerve of origin can often be id’d at base, fascicles of small wavy cells
52
Q

Schwanomma

A
  • AKA neurilemmoma
  • Wide distribution
  • Extremities slightly more prevalent
  • No association with neurofibromatosis
  • Solitary painless subcutaneous mass
  • Little capacity for recurrence
  • Encapsulated
  • Prominent blood vessels (thick hyaline walls)
53
Q

Two components of a schwanomma

****TEST QUESTION****

A

o **Antoni A: cellular with nuclear palisading (Verocay bodies) = CELLULAR –> Know that VEROCAY bodies occur here
o **
Antoni B: also Schwann cells but hypocellular, myxoid matrix = HYPOCELLULAR

**KNOW THIS FOR EXAM**

54
Q

Neurofibroma

A
  • Common, solitary polypoid or nodular lesion
  • Wide distribution
  • Deeper lesions more often associated with NF-1
55
Q

Plexiform neurofibroma

A
  • Pathognomonic of NF-1
  • Children and young adults, can become large and disfiguring
  • Thick convoluted cords of expanded nerve fibers – myxoid change common
56
Q

FOR EXAM…

A

**KNOW Schwannoma and neurofibroma **

57
Q

Granular cell tumor

A
  • Middle aged, Female > Male
  • Any anatomic site, trunk and tongue most common
  • Slow growing, recurrences are rare
  • Excision

Microscopy
o Variable circumscription, can be infiltrative
o Tumor cells in nests
o Large, polygonal cells with finely granular cytoplasm
o Nuclei small
o Rare mitoses OK

58
Q

Leiomyoa

A
  • Pilar leiomyoma and angioleiomyoma

- Images were shown of these two

59
Q

Giant cell tumor of tendon sheath

A
  • AKA: Nodular tenosynovitis
  • Localized form of pigmented vilonodular synovitis
  • Can occur at any age, typically adults
  • Typically hands, feet, wrists, knees, and rarely other joints.
  • Benign, but can recur in 5-30% of cases.
  • Treatment: Complete excision

Microscopy
o Well circumscribed, lobulated, partially encapsulated
o Variable proportions of mononuclear cells (small, round to spindled, with pale cytoplasm and round or grooved nuclei), osteoclast-like giant cells with 3-50 nuclei, foam cells, siderophages, epithelioid cells with glassy cytoplasm, round vesicular nuclei
o Varying amounts of dense collagenous stroma

60
Q

Ganglion cyst

A
  • Common, but not technically a tumor
  • Myxoid degeneration of joint capsule or tendon sheath
  • Sites: Wrist, hand, foot, and ankle
  • Secondary to injury or overuse, symptoms are pain, weakness

Microscopic appearance
o Not a true cyst because not lined by epithelial lining
o Cystic space lined by histiocytes and granulation tissue with moderate acute and chronic inflammation of cyst wall
o Mucin pockets within wall

61
Q

Superficial fibromatosis

A
  • Hand = Dupuytren contracture
  • Plantar = Ledderhose disease
  • Penile = Peyronie disease
  • Not associated with Beta-catenin mutations
  • These are seen in desmoid fibromatosis (this is DIFFERENT than superficial fibromatosis)
  • That would be a familial cancer system – we are NOT talking about that here
  • Nodular proliferation of fibroblasts
  • Can be infiltrative (even though it is benign and not malignant)
  • Not malignant, but can recur
  • Treatment is surgical incision
62
Q

Exam questions

A
  • Very straight forward questions
  • Nothing tested that isn’t written out in the slides
  • Only things you will see on the lower extremity will be tested