Benign skin and soft tissue lesions & tumours Flashcards
(100 cards)
1
Q
Classify and list benign pigmented lesions.
A
- Nevocellular
- Congenital: congenital nevi (small, medium, giant)
- Acquired: junctional, compound, intradermal nevi
- Special: halo nevus, spitz nevus
- Melanocytic
- Epidermal: ephilides, lentigo simplex, solar lentigines, nevus spilus, cafe au lait, becker’s nevus
- Dermal: Nevus of Ota/Ito, congential dermal melanosis, blue nevus
2
Q
Describe acquired nevocellular nevi
A
- Acquired brown macule/papule composed of nevus cell nests
- Tend to be small, homogenous, smooth round symmetrical borders, sun exposed areas
- 3 types, tend to mature through these stages over time
- Junctional - flat, darker pigments, nevus cells at epidermal-dermal junction
- Compound - raised, nevus cells in epidermis, epidermal-dermal junction, dermis
- Dermal - raise, less pigmented, nevus cells in dermis only
3
Q
Classify and describe congenital melanocytic nevi
A
- Typically classified by size (anticipated size at maturity)
- Small < 1.5cm2
- Medium - 1.5 - < 20cm2
- Small and medium have similar properties
- tan to brown, irregularly shaped; tend to darken, become elevated, have nodular properties and grow hair in puberty
- Treatment is excision, serial excision (medium) if symptomatic, clinical change or for cosmesis, malignant transportion is low (? 1%)
- Giant - > 20cm (6cm body / 9cm head in infant)
- pale brown and hairless to dark, hairy, verrucous, colour variegation +/- satellite lesions
- locations: trunk > extremities > H&N; may extend to leptomeninges - central lesions consider CNS involvement
4
Q
What do you tell the parents of an infant born with a giant cutaneous CMN regarding prognosis and treatment planning?
A
- Prognosis is related risk of malignant transforation to melanoma - quote overall 5 to 10%
- among those who develop melanoma, 50% by 3 yrs; 60% by 7 yrs; 70% by adolesc
- RF: 3+, age of patient, posterior scalp/body, size (giant), sun exposure
- Would recommend surgical treatment for giant, non-surgical treatment with close observation for small/medium
- Non-operative treatment
- close observation: serial exams, photography, clinical assessments
- Operative
- simple excision
- exision and grafting
- serial excision
- tissue expansion and exision or locoregional flap of expanded tissue
5
Q
What are the indications and goals for operative treatment of CMN?
A
Goals
- excise as much nevus in as few stages as possible
- preserve function and cosmesis
Indications
- Cosmetic / social concern
- Functional complaints - symptomatic: pruritis, pain, hair, impaired function
- Clinical change
- Risk of malignant transformation (giant)
6
Q
How are congenital nevis differentiated from acquired nevi on histopathology?
A
Congenital nevi have nevus cells of NCC origin in ectopic locations
- Nevomelanocytes in epidermis
- Sheets, nests, cords and single cells in reticular dermis and SC tissue and btwn collagen bundles
- Nevomelanocytes in epidermal component of appendages, piloerector muscles
- Neurovascular infiltration
- Perivascular and perifollicular distribution
7
Q
What is your differential for a congenital nevus?
A
- Congenital: Nevus of Ito/Ota, dermal melanosis, nevus sebaceous
- Acquired: acquired nevus, cafe au lait, becker’s nevus, nevus spilus
8
Q
what syndromes do you see cafe au lait spots
A
NF-1
McCune-Albright
9
Q
What are treatment options for nevus of Ota/Ito
A
– Q switched ruby/Nd:YAG laser
10
Q
Differential diagnosis blue nevus
A
- Malignant nodular melanoma
- Metastatic melanoma
- Kaposi sarcoma
- Venous malformation
11
Q
Why are congenital dermal melanoses (“mongolian spots”) seen as a blue colour?
A
Because of the Tyndall effect – Long wavelength light (reds) is transmitted and therefore pass by melanin (brown/black in colour), while short wavelength light (blues) is scattered, some being reflected backwards to the skin surface as blue colour
12
Q
What is the histopathological definition of atypical nevus?
A
- proliferation of intra-epidermal melanocytes seen singly or in irregular nests along the basal layer or just above the rete ridges
- variable and discontinuous melanocytic cellular atypia
13
Q
What is FAMM syndrome?
A
familial atypical mole and melanoma syndrome
- > 50 atypical moles + FHx of 1st/2nd degree relative w/ H/O melanoma
- AD inheritance
- 10% risk / 10 years; 100% lifetime risk
- Prophylactic excision does not change prognosis because melanoma can be de novo
- Photographs & monitor; excise when they change / express worriesome featues
14
Q
What is Waardenburg syndrome?
A
Congenital absence of melanocytes from skin, hair, eyes, or stria vascularis of the ears resulting in auditory (deaf) & Hypo-pigmentary Disorders, cleft lip and palate
15
Q
Define and describe neurocutaneous melanosis, including clinical symptoms, risk factors, treatment considerations.
A
· NCM is defined as a congenital disorder including multiple (> 3) or giant melanocytic nevi and infiltration of brain or leptomeninges by abnormal melanin deposits
· Triad – Fox’s 1972 diagnostic criteria: (radiopaedia.org; Kadonaga et al, acad dermatol 1991) -
o multiple or giant melanocytic nevi with leptomeningeal melanosis or melanoma
o no evidence of malignant change in cutaneous lesions
o no evidence of malignant melanoma in any organ except for leptomeninges
· Risk Factors – any size Congenital Nevocellular Nevi on scalp, neck, or spinal midline; giant CNN crossing the midline; satellitosis in these locations increases risk
· Clinical features – symptoms present at median age of 2 years à (signs consistent w/ increased ICP or SOL): hydrocephalus, seizures, focal neurologic deficits
· Imaging - MRI with gadolinium: detects melanin; do for all children with risk factor(s) by 6 months
· Extremely poor prognosis; progressive deterioration to death
· Treatment – Symptomatic: delay cutaneous excision d/t poor prognosis; excision, radiation, chemotherapy, interferon, retinoids
16
Q
List types of biopsy, and ideal type of biopsy
A
Types:
- Shave
- FNAB
- Core, truCut
- Incisional
- Mapping
- Excision
Ideal:
- Excisional biopsy with 1-2mm margin
17
Q
What is Cowden syndrome?
A
Multiple trichilemmomas, breast Ca, thyroid Ca, colon CA
18
Q
What is Muir-Torre Syndrome?
A
– sebaceous neoplasm (adenoma, carcinoma) + ≥ 1 visceral malignancy (colon cancer>GU), keratoacanthoma, BCCs, ?subtype of hereditary nonpolyposis colorectal cancer (HNPCC)
19
Q
What is Cowden Sydrome?
A
· Cowden Syndrome
o Physical Features – trichilemmomas, mucosal papillomas, multiple benign skin lesions
- also adenoid facies, craniomegaly, arched palate, scrotal tongue, sclerotic fibromas, punctate palmoplantar keratosis, acral keratosis,
o Systemic neoplasms – breast adenocarcinoma (age: 20s), breast fibroadenomas, GI polyps, thyroid cancer
20
Q
List benign epidermal, appendage, mesynchymal lesions
A
- Epidermal
- Seborrheic keratosis
- Clear cell acanthoma
- Achondroma
- Veruca vulgaris
- Epidermal cyst
- epidermal cyst
- dermal cyst
- milia
- Appendage
- Trichoepithelioma
- Tricholemmoma
- Pilomatrixoma
- Appendage cyst
- Pilar cyst
- Sebacceous gland
- Sebaceous hyperplasia
- Sebaceous adenoma
- Nevus sebaceous (of Jadassohn)
- Eccrine
- Poroma
- Syringoma
- Spiradenoma
- Apocrine
- Cylindroma
- Mesenchyme
- Vascular: pyogenic granuloma, glomus (other vascular tumours/malformations), angiofibroma
- Nerve: neuroma, schwannoma, neurofibroma
- Fibrous: DF, nodular fasciitis
- Fat: lipoma
- Histiocytic: xanthoma
21
Q
What are the diagnostic criteria for neurofibromatosis?
A
NF1 - > 2 of the following
- > 2 NF or > 1 plexiform NF
- > 2 Lisch nodules
- Optic glioma
- > 6 CALMs (> 5mm kid, > 15mm adult)
- Axillary / inguinal freckling
- Distinctive osseous lesion
- FHx (1st degree)
NF2 -
- Bilateral acoustic neuroma OR
- FHx and Unilateral acoustic neuroma or 2 of:
- Schwannoma
- Glioma
- Neurofibroma
- Meningioma
- Juvenile posterior subcapsular opacity
- FHx and Unilateral acoustic neuroma or 2 of:
22
Q
Describe seborrheic keratosis
A
- Description: skin colour to pigmented waxy, “stuck on” acquired lesion
- Patients: typically older
- Location: typically face and trunk
- Path: acanthosis, parakeratosis, hyperkeratosis, papillomatosis
- Management:
- Observation, a-hydroxy-acid, TCA, cryo, EDC, shave, excise
- Differential: flat pigmented and raised pigmented lesion differential
- Leser-Trelat sign - sudden multiple SK associated w internal cancer or malignant acanthosis nigricans
23
Q
What is clear cell acanthoma?
A
Benign epidermal lesion that is raised, red, rare, slow growing; treat with excision
Path: large epidermal cells filled with glycogen (appear clear)
Location: legs and covered wiht thin crust (peripheral collarette)
24
Q
Describe epidermal cyst
A
Description: subcutaneous nodule, mobile, punctum, +/- history of trauma that arises from epidermis / epithelium of hair follicle
Histopathology: filled with keratin and sebum, lined with stratified squamous epithelium (not stratum corneum though)
Management: if actively infected: I&D, PO Abx, delay excision; if not actively infected: excise w/ ellipse of skin including the punctum
25
Describe dermoid cyst
Congenital cyst that arrises when cells destined from epidermis (ectoderm origin) get trapped along embryonic lines of fusion - making a keratin-filled sac- containing tissues from multiple germ layers (ectoderm/mesoderm)
Location: often H&N; lateral brow (angular cyst), nasal root, midline, scalp
Treatment: non-midline - excise down to periosteum
Treatment midline: pre-op imaging CT/MRI to r/o intracranial extension of base; other differential diagnosis
DDx midline: dermoid, glioma, hemangioma, vascular malformation, encephalocele, meningioma
26
What is your differential for a junctional nevus?
(*ie what is you differential for a flat, pigmented lesion?)*
* Lentigo: simplex, solar, atypical
* flat atypical/dysplastic nevus
* congenital dermal melanosis
* nevus of Ota/Ito
* nevus spilus
* cafe au lait
* hyperpigmented scar
* traumatic tattoo
* lentigo maligna, lentigo maligna melanoma, superficial spreading melanoma
* Seborrheic keratosis
* pigmented AK
27
What is you differential for a compound nevus?
(*ie what is your differential for a raised pigmented lesion?)*
* compound, dermal nevus
* epidermal nevus
* seborrheic keratosis
* dysplastic nevus
* small superficial spreading melanoma, early nodular melanoma
* pigmented basal cell carcinoma
* DF
* spitz nevus
* blue nevus
* keloid scar, HTS
* FB granuloma
* vascular: angiokeratoma
* kaposi sarcoma
28
What is your differential for a dermal nevus?
(*ie what is your differential for a tan/skin coloured lesion?*)
* BCC
* neurofibroma
* trichoepithelioma
* DF
* sebaceous hyperplasia, adenoma
* skin tag - acrochordon
29
What is your differential for a blue nevus?
(*ie what is your differential for a nodular darkly pigmented lesion?)*
* DF
* glomus tomor
* primary nodular or metastatic melanoma
* pigmented spitz nevus
* traumatic tattoo
* angiokeratoma
* kaposi sarcoma
* pigmented BCC
30
What is your differential for a congenital nevus?
(ie list congenital pigmented lesions)
* congenital melanocytic nevus (small, medium, giant)
* congenital dermal melanosis
* aquired nevus
* becker’s nevus
* nevus spilus
* cafe-au-lair spot
* lentigo
* ephilides
* nevus sebaceous
* epidermal nevus
* vascular anomaly
31
What is your differential for a cystic lesion?
* skin cysts:
* epidermoid cyst
* dermoid cyst
* milia
* pilar / trichilemmal cyst
* steatocytoma simplex/multiplex
* digital mucous cyst
* other cysts
* branchial cleft cyst
* other soft nodular structures
* lipoma
* FB granuloma
* xanthoma
* pilomatrixoma
* other fluctuant skin structures
* abscess
* folliculitis / faruncle / caruncle
32
What is your differential for a midline / glabellar cyst?
* dermoid cyst
* glioma
* meningioma
* encephalocele
* hemangioma
* vascular malformation
33
What is your differential for a tender cutaneous lesion?
* Glomus
* Neuroma
* Angioleiomyoma
* Angiolipoma
* Blue rubber bleb nevus (venous malformation that is spontaneous painful/tender)
* Eccrine spiradenoma
34
What is your differential for a bright red / purple raised lesion?
* cherry hemangioma
* angiokeratoma
* kaposi sarcoma
* keloid scar
* poroma (hands/feet)
35
What is your differential for a dermatofibrome?
* HTS, KS
* blue nevus,
* dysplastic nevus,
* KA,
* leiomyoma,
* neurilemmoma,
* pilomatricoma,
* mets,
* DFSP,
* BCC,
* SCC,
* melanoma
* kaposi sarcoma
36
What is your differential for a flat or slightly raised skin coloured lesion around the EYELID
* syringoma
* xanthoma/xanthelesma
* milia
* Apocrine cystadenoma
* molluscum contagiosum
* steatocytoma
* acne
37
What is your differential witha lesion that has a central keratin scale?
* DF
* clear cell ananthoma
* molluscum
* KA
* AK
* FB granuloma
* acne
* BCC, SCC
38
Compare the risk factors for development of cutaneous malignancy
**Factor**
**BCC**
**SCC**
**MM**
**Patient Factors**
Skin type (I/II \> IV/V/VI)
+
+
+
Hair, eyes
Fair hair, blue eye
Red hair (blond), blue eyes (green), freckling (esp \> 50)
Male
+
+
+
Immunosuppression
(+)
+
+
Chronic wound
+
Cigarette
+
HPV (16, 18, 31, 33)
+
Previous NMSC
+
+
(+)
Previous MSC, FHx MSC
+
Other, precursors
none
AK, cut horn, Bowen’s, leukoplakia,
Dysplastic nevi (number), MIS, lentigo maligna
**Environmental factors**
Solar and UV exposure\*
UVB \> UVA; UVA accentuates UVB)
+ (intermittent)
+ (cumulative)
+ (intermittent, sunburns, tanning beds)
Chemicals (_arsenic_, psoralins, nitrogen mustard, tar, soot, mineral oil, hydrocarbons)
+
+
Radiation
(+)
+
**Genetic syndrome/predisposition**
Xeroderma pigmentosum
+
+
+
Gorlin
+
Gardner
+
Bazez
+
Epidermolysis bullosa
+
Muir Torre
+
Ferguson Smith
+
Nevus Sebaceous of Jadasshon
+
Albinism
+
+
Porokeratosis
(+)
+
Atypical mole syndrome (FAMM)
+
39
List microscopic changes consistent w/ pre-malignant change
1. abnormal epidermal maturation
2. change is size and shape of cells
3. change in polarity / loss of polarity
4. nuclear hyperchromatism
5. prominent nucleoli
6. abnormal mitotic figures
40
Describe actinic keratosis
* precancerous (pre-SCC) epithelial lesion characterized by erythemetous hyperkeratotic plaque on with white/yellow scale in sun exposed areas (H&N, extremities, back) - often in background of other solar changes - dyskeratosis, telangectasia, wrinkling
* path findings: atypical keratinocyte changes (see pre-malignant histopath findings) in background of other histopath changes consistent w/ chronic sun damage
* differential: SK, discoid lupus, psoriasis, Bowen's, SCC, superficial BCC,
* prognosis: progression to SCC 0.1% / lesion / year but avg person has 7.7 lesions therefore 10 year risk is 10%
* Medical treatment: on-label: imiquimoid, topical 5-FU, PDT
* Surgical treatment: cryo (mainstay), shave, EDC, dermabrasion, excision
41
describe cutaneous horn
* central keratin plug in central basal nodular lesion
* 15-20% have malignancy component at presentation (SCC \>\>\>\> BCC, KA, KS, sebaceous Ca)
* treat w/ excision
42
Describe arsenic keratosis
* palms and soles most commonly affected w/ parakeratotic and hyperkeratotic, scaling lesions on erythemetous base
* pathology shows VACUOLATED KERATINOCYTES
* 5-20% conversion / transformation to SCC and more aggressive than de novo
* treatment - medical: 5-FU, retinoids, chelation when acute (dimercaperol)
* treatment - surgical: cryo, excision
43
Describe radiodermatitis
* chronic radiation wound / dermatitis
* usually 20+ years after treatment; risk when XRT \> 1000 rads
* very aggressive, 25% metastatsis at presentation
* biopsy to diagnose, excise, poor prognosis
44
Describe bowen's disease
* Cutaneous SCC in situ; in anogenital region (esp glans) called Erythroplasia of Queryat, also oral mucosa, conjunctiva, nail bed (subungual epidermoid carcinoma)
* sharply demarcated hyperkeratotic plaque on erythemetous sometimes indurated base
* Pathology: WINDBLOWN appearance; list 6 features of pre-malignant change
* prognosis: 5-10% risk malignant transformation/progression to SCC (higher for oral, anogenital, nailbed ~ 30%)
* Biopsy to diagnose
* Treat - medical: Imiquimod, PDT, consider XRT
* Treat - surgical excision w 2-5mm margin (want negative margin), consider MOHS
45
List and describe non-surgical options for treatment of pre-malignant and malignant cutaneous lesions
* Imiquimod (Aldara)
* MOA: immune response modifier - act through TLR to amplify NK and B-cell activity
* Uses - On-label: AK, superficial BCC, genital warts; Off-label: nodular BCC, Bowen's disease, porokeratosis, Lentigo meligna, extra-mammary paget, Keloid, +ve margins
* How to use: apply topical qhs (leave overnight) x wks (2-16; often ~ 6)
* 5-FU (Effudex)
* MOA: blocks DNA synthesis as a pyrimidine antagonist
* Uses - On-Label: AK, superficial BCC; Off-label: Bowen, porokeratosis, extra-mammary paget
* How to use: apply BID for 2-6 wks
* Retinoids
* MOA: inhibits hyperproliferating keratinocytes
* Uses - all off-label: AK (probably most accepted), arsenic keratosis, lentigo meligna, superficial BCC
* 0.5 - 2mg/kg/d PO x wks
* Photodynamic Therapy
* MOA: photodynamic activiation of a topical sensitizing agent leads to cytotoxic effect against oxygen free radicals
* Uses - all investigational at present: AK, BCC, SCC in situ
* Radiation
* Uses: BCC, SCC, Merkel cell, Keloid scar, Kaposi sarcoma
* Indications: non-operative candidate, refractory to other treatments, perineural or lymphovascular invasion, + margins, as adjuvant therapy when LN +
* Contraindications: Gorlin disease, pregnancy, Lupus and some other CTD, Verrucous carcinoma
46
What is the mechanism for development of cutaneous malignancy?
* Radiation or other stimuli induce electron excitation in absorbing atoms, which causes chemical damage to DNA
* Genetic mutations (inherited, spontaneous) contribute: p53 (BCC, SCC), oncogenes ras & fos, PTCH (BCC), CDK2NA & CDK4 genes (MM)
47
Describe effect of UV light
· UVB causes DNA damage; UVA accentuates DNA effects of UVB
· Excessive UVB also interferes with normal immune functions
o Sunscreens may not prevent the immunologic suppression
o Black- and white-skinned individuals are equally susceptible to adverse immunologic effects of acute low-dose UVB
48
define BCC
* cutaneous malignancy originating from basal layer of keratinocytes in epidermis and epidermal appendages
* doubling time 4 d (0.5cm / yr)
* arise de novo; no precursor lesion
49
How do you classify BCC? Describe classification.
* There are many histological subtypes (\> 26)
* Many (~ 40%) BCC are histologically a combination of \> 1 subtype
* Clinical subtypes:
* Nodular / nodulo-ulcerative: 50-70% - firm, round skin coloured or lightly pigmented papule with defined borders, often pearly border and telangectasia. As grows larger will outstrip blood supply centrally and form characteristic central ulcer (rodent ulcer)
* Superficial spreading: 10-20% - lightly pigmented erythemetous macule/patch often trunk/shoulder
* Pigmented: 5% looks like pigmented nodular w/ similar behaviour; MM in ddx
* Morpheaform/sclerosing: 2-3%, white/yellow/pink with central sclerosis, thick ropey *looks like a scar* and ill-defined borders; most likely to recur / have +ve margins
* Others: infiltrative, desmoplastic, basosquamous, (all higher risk recurrence); cystic (central tumour degeneration), micronodular (higher recurrence than nodular; looks like collection little hair bulbs)
50
how do you diagnose and what is characteristic pathologic finding of bcc
* diagnosis can be suspected on history and physical
* definitive diagnosis by biopsy and pathologic review
* charactertistic path finding is peripheral palisading of basaloid cells, basaloid cells in dermis
51
* List and briefly describe different treatment modalities for primary BCC including indications and techniques
MEDICAL
* Imiquimod & 5-FU - superficial BCC (off label nodular BCC) - apply at night x wks
* disadvantages is can't assess margins and difficult to measure response
* PDT - in infancy, not mainstream
* Radiation - 92% cure; for older patients, non-surgical candidate, recurrent, + margins, perineural or lymphovascular invasion, or as adjuvant for N+ or T3/4
SURGICAL
* Cryotherapy - series of applications of -40'c
* Electrodessication and curretage - debulk tumour, currette bed, dessicate bed
* both above cannot assess margin status
* for low-risk and small lesions with defined borders
* Surgical excision
* Margins - for low risk tumours \< 2cm diameter then 2-5mm margins acceptable; general safe is 4mm (95% cure) vs. 2mm (82% negative margin and 4% recurrence)
* for high risk tumours or \> 2cm then 1cm is reasonable.
* MOHS
* Indications: indistinct borders (morpheaform/sclerosing/infiltrative/some ss), sensitive areas (H&N, specifically eye, NL fold, nose, peri-oral), some say very large.
* excise deem and rim at 45' wtih small margin, map and section horizontally
52
How do you define hemangioma?
* Benign vascular tumour of the neonatal period characterized by rapid proliferation of endothelial cells and undergoing characteristic phases of proliferation, involuting (stabilization) and involuted.
53
Define vascular malformation
* A vascular malformation is a lesion that is present from birth, grows proportionally with the growing infant and child, and is characterized by dysmorphic channels lined by mature endothelium; very slow rate of turnover and no involution.
54
Compare vascular tumours to vascular malformations
**Hemangiomas (vascular tumors)**
**Vascular malformations**
**Hemangioma**
**Clinical**
* Usually not present at birth (30%)
* F: M = 3:1
* Initial period of rapid progression
* All present at birth, grow in prop
* F=M
* Slow progression, proportional
**Cellular**
* *Cellular turn over:*
* *No. of mastocytes:*
* *Basement membrane:* thick
* *Cellular turn over:* Normal
* *No. of mastocytes:* Normal
* *Basement membrane:* Normal thin
**Pathology**
* Distinctive aspects of all 3 phases
* Depending on type
**Immuno-phenotype**
* ***GLUT1 +ve – in all phases IH (neg in CH)***
* ***GLUT1 -ve***
**Hematologic**
* Primary platelet trapping
* Thrombocytopenia (Kasabach-Meritt Syndrome only in _KHE + TA)_
* Primary stasis (venous), localized
* Consumptive coagulopathy
**Radiological**
* Angio: *well-circumscribed*, lobular- parenchymal solid tumor + equatorial vessels
* MRI: Well-delineated tumor + flow voids
* Angio: diffuse, no parenchyma
* **Low-flow: phlebolith**, ectatic channels
* **High flow: enlarged tortious** channels + AV shunting
* MRI: Hypersignal on T2 sequences
**Skeletal**
* Infrequent “mass effect” on bone
* Hypertrophy rare
* Low-flow: distortion, hypertrophy or hypoplasia
* High-flow: destruction, distortion or hypertrophy
**TREATMENTS**
* Observation
* Intra-lesional steroid (\< 3cm)
* Systemic steroid or propranolol (symptomatic \> 3cm or problematic location)
* Other: topical steroid, vincristine, interferon, sclerotherapy, embolization, excision
* CM: laser
* VM: sclerotherapy (
* LM – macro: doxycycline sclerotherapy
* LM – micro: excision or bleomycin sclerotherapy
* AVM/F: embolization alone (temporiz’n) vs. embolization and excision
55
List the ISSVA classification of vascular anomalies
* Benign vascular tumours: Hemangioma (infantile, congenital), tufted, spindle cell, epithelioid and pyogenic granuloma
* Locally aggressive vascular tumours: kaposiform hemangioendothelioma, retiform hemangioendothelioma
* Malignant vascular tumours: angiosarcoma, epitheliod hemangioendothelioma
Simple vascular malformations:
* Low flow: Capillary, lymphatic, venous
* High flow: arteriovenous malformation/fistual
Combined vascular malformations
* Low flow: CLM, CVM, CLVM (kts) LVM
* High flow: CAVM, CLAVM, CLAVF
56
what are RF for hemangioma?
* more common in caucasian, female
* rf are prematurity, CVS
57
describe a morphologic classification for hemangioma
* superficial: focal, segmental, multiple
* deep: hepatic or gastric
* multiple disseminated hemangiomatosis
58
what are the histologic stages of hemangioma
* proliferating
* increased endothelial cells, increased mast cells, increased thickness of BM
* new draining and feeding channels
* involuting
* flattening, deposition of fibrous tissue
* cell atrophy begins at ~ 1 yr
* involuted
* flat, mature endothelium, loose-fibrofatty tissue
59
describe the clinical stages of hemangioma
1. origin (1st 4 wks) - herald spot, small telangectasia
2. initial growth (2-5 wks) - closely packed pinhead lesions
3. intermediate growth (2-10 mos) - enlargement, red & tense
4. completed growth (6-20 mos) - becomes quiescent
5. initial involution (6-24 mos) - softer, flatter, colour fades
6. intermediate involution (1.5 - 5 yrs) - decreased size, decreased blanching, fibrosis
7. completed involution (\> 4 yrs; majority of improvement is by 4 yrs) - variable degree of atrophy and contour deformity
60
what are radiologic findings present w hemangioma?
· U/S: Proliferative phase= high flow, solid & dense (unlike VM), well circumscribed + prominent feeding and draining vessels
· CT/MRI: proliferating à enhancement, Involuting à lobular architecture (low attenuation on CT)
· MRI: clearly defined, low-intermediate signal, homogenous mass on T1 - fibrofatty, high signal intensity on T2; good for visceral lesions
· Nuclear scanning (99m –Tc labeled RBCs): visceral and brain hemangiomas
· Arteriography: Indicated only if embolization is planned
61
what syndromes are associated w/ hemangiomas?
1. von hippel lindau
* Retinal hemangioma
* Cerebellar Hemangioblastomas
* Seizures, mental retardation
* Liver, kidney, pancreas cysts
2. PHACES
* Posterior fossa malformation (Chiari)
* Hemangioma (facial)
* Arteries and Aorta - coarctation
* C - cardiac
* E - eye abnormalities - microphthia, cataracts
* S - sternal defects
62
describe principles of management of hemangioma
·
* **History & Physical** –
* When was the first lesion noticed? How has it grown with the child?
* Dermatome, vision, stridor, ulceration, multiple lesions, lumbosacral, perineal involvement
* **Investigations** – Photographs, U/S, CT, MRI
* **Consultations** – General surg, neuro, optho, peds, IVR, derm, ENT, heme, path, social work, etc.
* **Treatment**
* Observation & reassurance: Frequent follow-up (more frequent if problematic tumor), photographic documentation
* Consider for lesions that are: small, located off the face, \>1 year of age, already entering involutional stage (graying or softening of lesion)
* Supportive (splints, compression garments)
* Therapeutic Intervention (medical, surgical, radiological, combo)
63
list different treatment options for infantile hemangioma and different indications.
1. Generally, lesions that require treatment are those that are in a conspicous area that may have a problematic residual deformity, ulcerated/bleeding, in an area where there is functional consequence, extremely large
2. Intra-lesional steroid
1. small (~ \< 3cm), superficial, well circumscribed lesions commonly of nasal tip, cheek, peri-orbita, ear, lip (ie head and neck)
3. Systemic steroid
1. Large (~ \> 3cm), destructive, functionally important (vision, airway - obstruction of nose/oral cavity/oral pharynx), destructive (ulcerating, bleeding) or life-threatening
2. 1st line for life threatening
4. Systemic propanolol
1. generally same indications as for oral steroid "potential to impair function or cause disfigurement", generally for larger lesions not amenable to intralesional steroid
5. Interferon 2a/2b
1. 2nd line for life threatening hemangioma (after steroid)
6. Vincristine chemotherapy
1. 2nd line for kasselbach-merritt phenomenon
7. Laser
1. involuting, ulcerated lesion or involuted w/ residual colour/telangectasia
8. Embolization
1. selective embolization for life-threatening,
9. Excision
64
List indications for MOHs
* High risk BCC or SCC lesions
* Size and location:
* \>=6mm H face (mask face), genitalia, hands, feet (H face is periorbital, nose, perioral, pre/postauricular, temple, ear)
* \>=10mm cheek, forehead, scalp, neck, pre-tibia
* \>= 20mm anywhere else
* Indistinct borders
* Rapidly progressive
* Recurrent
* Previous XRT of bed
* Pathologic subtype
* SCC, poorly or undifferentiated, adenocystic, adenosquamous, basosquamous
* BCC, morpheaphorm/sclerosing, micronodular
* Other pathologic features
* LVI, PNI
* depth \> 2mm
* Other lesions that can be treated by MOHs: KA, DFSP,
65
If MOHs is unavailable but indicated, what is an alternative?
excision of complete circumferential and deep margin and intra-operative frozen section assessent
66
How do you perform mohs?
1. debulk central tumour
2. excise deep and peripheral margins, with small margin of normal tissue, tangential at 45'
3. divide into quadrants & map
4. serial horizontal section, examine
67
list painful cutaenous lesions
* angioleiomyomas,
* neuroma,
* glomus tumour,
* eccrine spiradenoma,
* angiolipoma,
* blue rubber bleb nevus
68
Describe fibroepithelial polyp
Soft skin colored pedunculated papilloma.
Synonym: acrocordon, skin tag
Associated with obeisty, hormonal imbalance and in areas of skin irritation. Increase in size/# w time/pregnancy
Tx: snip off or ED
69
Describe verruca vulgaris
Def: epidermal proliferations 2' to HPV infection (1,2,3,4,7), with variegations, thrombosed capillary loops on sites of trauma (hands, knees)
Epid: occurs in younger adults/kids via skin/skin contact and must enter basal layer
Path: anathosis, parakeratosis, hyperkeratosis, koilocytosis
Tx
* Medical - salicylic acid, TCA, immunomodulators (IFN-gamma, imiquimod), antiviral
* Surgical - cryotherapy (q1mx4), laser, ED. Avoid excision b/c recurrence/scar
70
List 4 benign epidermal tumors
* SK
* CCA
* VV
* FP
Sebarrheic keratosis, clear cell acanthoma, verruca vulgaris, fibroepitheial polyp
71
LIst 4 causes of epidermal cysts
* Congenital (occurs in fusion lines)
* Inclusion (traumatic)
* Hereditary (gardners syndrome)
* follicular (acne)
72
Describe a tricholemmal cyst
Def: firm dome mass on scalp filled w dense keratin core
Syn; pilar cyst
Epid: middle age, located on scalp
* Path
* cyst wall - straitifed squamous epithelium BUT with palisadting outer layer like outer root sheath
* cyst core - dense keratin + cholesterol cleft
* Prognosis
* most benign
* 2% rapidly grow -\>prolierating tricholemmal cyst
73
What is your diferential diagnosis for a dome shaped skin colored mass on scalp
* Dermoid cyst
* Tricholemmal cyst
* Epidermal cyst
* Pilomatrixoma
74
Describe Steatocytoma multiplex
Def: heritable considtion characterized by multiple dermal cysts filled with sebum
Epid: onset puberty, AD
Path
* abortive hir follcile at site of sebaceous gland attachment
* contains velus hair, sebum, keratin
Tx
* aspiration,excision - likely too many, CO2 laser
Prognosis: can develop Steatocytoma suppurativa - inflammatory variant - treat w tetracycline
75
Describe digital mucoid cyst
Def: a pseudocyst arising at DIP joint
Syn: myxoid cyst, periarticular cyst
? due ot mucoid degenration of CT around OA joint
Epid: 60s, F:M2:1
Path; Cyst containing viscous fluid of mucin and HA
Tx
* Conservative: warmth, massage
* MEdical: AgNO3
* Surgery
* cryotherapy, aspiration +/- steroid injection, ED, excision
* Best - attempt aspiration and if multi recurrence, excise. 50% resolve spontaneously
76
List 5 benign cutaneous cysts
* Epidermal cyst
* Tricholemmal cyst
* Dermoid cyst
* Steatocytoma multiplex
* Digital mucoid cyst
* Milia
77
Describe milia
Def: epidermal cyst containig keratin
1' neonatal - due to immature sebaceous cyst - resolve spontaneously
2' adult - post truaam (dermabrasion/surgery) or blistering disease where sebaceous cyst tracts damaged - need incision/deroof as no spontaneous regression
78
Classify benign ADNEXAL tumors
By Appendage type:
* Hair Follicle
* Trichoepithelioma
* Trichilemmoma
* Pilomatrixoma
* Sebaceous Gland
* Sebaceous hyperplasia
* Sebaceous adenoma
* Nevus sebaceous of Jadassohn
* Sweat Gland (ecrrine/apocrine)
* E: Syringoma
* E: Poroma
* E: Spiroadenoma
* A:Cylindroma
79
What is a trichoepithelioma
Being adnexal tumor of hair follicle. Smooth skin colored nodules on face, NL folds, nose, forehead, upper lip
HAve apeparance of nodular BCC without central crater/telangiectasia
AD, multiple, due to TSG - appear in childhood and gradullay increase in #
Epid: F\>M adult
Tx
- dermabrasion, excision
Prognosis
- if multiple, may recur post Dermabrasion
80
What is differential dx for a smooth skin colored nodule on the face?
BCC
Trichoepithelioma
Basloid follicular hamartoma
Microcystic adnexal carcinoma
81
What is a tricholemmoma
Benign neoplasm w differentiation toward pilosebaceous follicular epihtelioma
Mulitple small plaques flesh-colored in face/neck with hyerpkeratotic surface
Associated with Cowdens disease
Tx
- if suspect cowden, refer to derm/endo, gen sx
- shave/excision, ED, CO2 laser
82
What is Cowdens disease
Mutation of PTEN
Associated with
* Breast Ca, fibroadenoma
* GI polyps
* Thyroid cancer
Physical features
* tricholemmoma
* mucosalpapilloma
* arched palate
* craniomegaly
83
What is a pilomatrixoma
Matrix cell tumorigenesis, due to mutation bcl2
Subdermal nodule with possible calcifications occuring in children in face, upper extremtiies
Path
* encapsulated mass with epidermoid cells and extracellular Ca
Tx
* Surgical excision with margins? vs mohs
84
What is Muir torre syndrome
Mutation in MSH2/MLH1
Association
* Sebaceous neoplasm
* Keratoacanthoma
* Colon ca\>GU
* Breast Ca
85
What is sebaceous hyperplsia
Def. Harmartomatous enlargement of sebcaeous gland, appears as mutilple papule/nodule
IN elderly, face/nose/cheeks, in post-trasnapltn pts on cyclosporine
Tx
- topical - bicloracetic acid
- Surgical - ED, excision, cryo
86
What is sebaceous adenoma
On specrum of sebaceous hyperplsaia-\>carcinoma, associated with muir torres syndrome , due ot mutation in MSH/MLH
Smooth papule with central depression, can be multilobulated
Tx
- complete excision
87
What is sebaceous nevus of jadassohn
Assocaited with epilepsy, sz
Premlingnat lesion - 10% risk of BCC or appendage tumor in adulthood
History:
* Birth - yellow waxy plaque on scalp
* child - flat epithelial hyperplsia
* Puberty - verucous nodular raised darker - can involve lots of scalp
Tx
- excision
88
List and describe 3 benign eccrine gland tumors
* Syringoma
* most common, lcoated at eyelids/cheeks during/after puberty, assocaited w DM, downs
* 1-2mm papules
* Tx w dermabrasion, ED
* Spira-adenoma
* rare pigmented pink/purple/red/blue
* solitary nodule on trunk/head -PAINFUl with manipulation - maybe confused w glomus tumor
* Tx: excision
* Poroma
* skin colored single nodule on sole/foot/palms
* Tx w excision
89
List and describe an apocrine benign tumor
Cylindroma
* can be solitary or multiple firm rubbery pink/red/blue in head/scalp/neck - synonym with turban tumor
* Tx: serial excision, low risk of malig degen
Assocated with Brooke-Spiegler syndrome
* break out with alot of Skin tumors cylindorma, trichoepithelioma, AD
90
List benign dermal tumors
* Vascular
* Glomus tumor
* Angiokeratoma
* Pyogenic granuloma
* Neural
* Neurofibroma
* Fibrous
* Dermatofibroma
* Histiocytic
* Xanthoma
91
Describe angiokeratoma
Benign dermal tumor of vascular tissue
Red scaly plaque qith dilated vessls and epidermal thickening that grow rapidly during adoelscence
On LE\>\>UE
Tx
- excision to r/o melnaoma
92
Describe Glomus tumor
Benign dermal tumor of AV shunts. Appear blue/purple papule/nodule
Triad: cold sensitivity, pinpoint pain, severe paroxysmal pain
Love sign - pain with precise touch with pencil
Hildreth sign - relief of painw ith tourniquet
Acral/subungal
Tx - excision
93
Describe pyogenic granuloma
Benign dermal tumor of unknown etiology - red friable polypoid nodule
Rapidly grows over weeks on head, fingertips, trunk
Tx
- silver nitrate to base post shave
94
What is a dermatofibroma
Def - firm cutaneous nodule in the extremities - variation in pigment. Abnormal growth of dermal dendritic histiocytes.
yound adult, F:M 4:1
Syn; histiocytoma
When pinched, dimple forms
Tx: excision
95
What is a neurofibroma
Infiltration of dermal connective tissue, non-encapsulated
Associated with Vonrechlinhausen disease (NF)
3 types
* cutaneous
* subcutaneous - both soft, compressible nodules
* plexiform - thick irregular, can entwine important structures
Tx
excision or biopsy if increasing in size or painful
if NF suspected - work-up
96
What are clinical features of NF
* Neurofibromas
* cutaneous or subcutaneous
* plexiform
* CALM (appear in first 3yrs of life)
* Iris harmartoma - Lisch nodule
* Bone deformities - long boen bowing, pseudoarthrosis of tibia, sphenoid deficit with pulsatle exopthalmus
* Endocrine: precocious puberty, pheochromocytoma
97
What are diagnostic criteria of NF1
NF1 - peripheral - 17q mutation - NFFBLOC
2 or more of
* NF _\>_2 of anytype of 1 plexiform
* Freckling - axilla/inguinal
* FDR
* Bone dysplasia (sphenoid absence, bowing, tibia PA)
* Lisch nodule (Iris Hamartoma) _\>_2
* Optic Glioma
* CALM _\>_6, _\>_5 mm prepubertal, _\>_15mm postpub
98
What are principles of biopsy vs excision of suspected neurofibroma
* NF can be cutaneous, subcut, plexiform
* Biopsy - can debulk - not excise if risk of sacrifice important nerve
* Biopsy if increase size or painful
* Malignant degeneration NF1 5-15%, NF2 \<1% (MPNST)
99
What are diagnostic criteria for NF2
NF2 - central - mutation on 22q
* CN8 massess bilateral
OR
2 of following NOMSG
* neurofibroma
* Opacity (junenile posterior subcapsular opacity)
* meningioma
* schwannoma
* glioma
100
What is an xanthoma
Benign dermal tumor of histiocyte = lipid laden macrophage - arise due to lipid metabolism alteration
arise spontensoulsy, associated with hyperlipidemia disorders or lympho/myeloproliferative disorders
5 types
* Palpebrarum xanthelasma = eyelids
* Tuberous xanthoma = firm PAINLESS nodules on pressure points - knee/elbow/buttock
* tendinous xanthoma = subcut nodule related to achilles/hand/foot extensor
* eruptive xanthoma = red/yellow papule w erythema on extensors surface
* plane xanthoma = macular covering large area
Path: foamy histiocytes
W/U - medical asx for hyperlipidemia
Tx - treat underlying dyslipidemia. lesion treatw TCa, ED, excision
