Benign skin and soft tissue lesions & tumours Flashcards
Classify and list benign pigmented lesions.
- 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
Describe acquired nevocellular nevi
- 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
Classify and describe congenital melanocytic nevi
- 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
What do you tell the parents of an infant born with a giant cutaneous CMN regarding prognosis and treatment planning?
- 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
What are the indications and goals for operative treatment of CMN?
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)
How are congenital nevis differentiated from acquired nevi on histopathology?
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
What is your differential for a congenital nevus?
- Congenital: Nevus of Ito/Ota, dermal melanosis, nevus sebaceous
- Acquired: acquired nevus, cafe au lait, becker’s nevus, nevus spilus
what syndromes do you see cafe au lait spots
NF-1
McCune-Albright
What are treatment options for nevus of Ota/Ito
– Q switched ruby/Nd:YAG laser
Differential diagnosis blue nevus
- Malignant nodular melanoma
- Metastatic melanoma
- Kaposi sarcoma
- Venous malformation
Why are congenital dermal melanoses (“mongolian spots”) seen as a blue colour?
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
What is the histopathological definition of atypical nevus?
- 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
What is FAMM syndrome?
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
What is Waardenburg syndrome?
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
Define and describe neurocutaneous melanosis, including clinical symptoms, risk factors, treatment considerations.
· 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
List types of biopsy, and ideal type of biopsy
Types:
- Shave
- FNAB
- Core, truCut
- Incisional
- Mapping
- Excision
Ideal:
- Excisional biopsy with 1-2mm margin
What is Cowden syndrome?
Multiple trichilemmomas, breast Ca, thyroid Ca, colon CA
What is Muir-Torre Syndrome?
– sebaceous neoplasm (adenoma, carcinoma) + ≥ 1 visceral malignancy (colon cancer>GU), keratoacanthoma, BCCs, ?subtype of hereditary nonpolyposis colorectal cancer (HNPCC)
What is Cowden Sydrome?
· 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
List benign epidermal, appendage, mesynchymal lesions
- 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
What are the diagnostic criteria for neurofibromatosis?
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:
Describe seborrheic keratosis
- 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
What is clear cell acanthoma?
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)
Describe epidermal cyst
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
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
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
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
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
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
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
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
What is your differential for a midline / glabellar cyst?
- dermoid cyst
- glioma
- meningioma
- encephalocele
- hemangioma
- vascular malformation
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
What is your differential for a bright red / purple raised lesion?
- cherry hemangioma
- angiokeratoma
- kaposi sarcoma
- keloid scar
- poroma (hands/feet)
What is your differential for a dermatofibrome?
- HTS, KS
- blue nevus,
- dysplastic nevus,
- KA,
- leiomyoma,
- neurilemmoma,
- pilomatricoma,
- mets,
- DFSP,
- BCC,
- SCC,
- melanoma
- kaposi sarcoma
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
What is your differential witha lesion that has a central keratin scale?
- DF
- clear cell ananthoma
- molluscum
- KA
- AK
- FB granuloma
- acne
- BCC, SCC
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)
+
List microscopic changes consistent w/ pre-malignant change
- abnormal epidermal maturation
- change is size and shape of cells
- change in polarity / loss of polarity
- nuclear hyperchromatism
- prominent nucleoli
- abnormal mitotic figures
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
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
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
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
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
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
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)
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
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
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)
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
- 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
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.
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.
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
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
what are RF for hemangioma?
- more common in caucasian, female
- rf are prematurity, CVS
describe a morphologic classification for hemangioma
- superficial: focal, segmental, multiple
- deep: hepatic or gastric
- multiple disseminated hemangiomatosis
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
describe the clinical stages of hemangioma
- origin (1st 4 wks) - herald spot, small telangectasia
- initial growth (2-5 wks) - closely packed pinhead lesions
- intermediate growth (2-10 mos) - enlargement, red & tense
- completed growth (6-20 mos) - becomes quiescent
- initial involution (6-24 mos) - softer, flatter, colour fades
- intermediate involution (1.5 - 5 yrs) - decreased size, decreased blanching, fibrosis
- completed involution (> 4 yrs; majority of improvement is by 4 yrs) - variable degree of atrophy and contour deformity
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
what syndromes are associated w/ hemangiomas?
- von hippel lindau
- Retinal hemangioma
- Cerebellar Hemangioblastomas
- Seizures, mental retardation
- Liver, kidney, pancreas cysts
- PHACES
- Posterior fossa malformation (Chiari)
- Hemangioma (facial)
- Arteries and Aorta - coarctation
- C - cardiac
- E - eye abnormalities - microphthia, cataracts
- S - sternal defects
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)
list different treatment options for infantile hemangioma and different indications.
- 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
- Intra-lesional steroid
- small (~ < 3cm), superficial, well circumscribed lesions commonly of nasal tip, cheek, peri-orbita, ear, lip (ie head and neck)
- Systemic steroid
- Large (~ > 3cm), destructive, functionally important (vision, airway - obstruction of nose/oral cavity/oral pharynx), destructive (ulcerating, bleeding) or life-threatening
- 1st line for life threatening
- Systemic propanolol
- generally same indications as for oral steroid “potential to impair function or cause disfigurement”, generally for larger lesions not amenable to intralesional steroid
- Interferon 2a/2b
- 2nd line for life threatening hemangioma (after steroid)
- Vincristine chemotherapy
- 2nd line for kasselbach-merritt phenomenon
- Laser
- involuting, ulcerated lesion or involuted w/ residual colour/telangectasia
- Embolization
- selective embolization for life-threatening,
- Excision
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
- Size and location:
- Other lesions that can be treated by MOHs: KA, DFSP,
If MOHs is unavailable but indicated, what is an alternative?
excision of complete circumferential and deep margin and intra-operative frozen section assessent
How do you perform mohs?
- debulk central tumour
- excise deep and peripheral margins, with small margin of normal tissue, tangential at 45’
- divide into quadrants & map
- serial horizontal section, examine
list painful cutaenous lesions
- angioleiomyomas,
- neuroma,
- glomus tumour,
- eccrine spiradenoma,
- angiolipoma,
- blue rubber bleb nevus
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
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
List 4 benign epidermal tumors
- SK
- CCA
- VV
- FP
Sebarrheic keratosis, clear cell acanthoma, verruca vulgaris, fibroepitheial polyp
LIst 4 causes of epidermal cysts
- Congenital (occurs in fusion lines)
- Inclusion (traumatic)
- Hereditary (gardners syndrome)
- follicular (acne)
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
What is your diferential diagnosis for a dome shaped skin colored mass on scalp
- Dermoid cyst
- Tricholemmal cyst
- Epidermal cyst
- Pilomatrixoma
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
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
List 5 benign cutaneous cysts
- Epidermal cyst
- Tricholemmal cyst
- Dermoid cyst
- Steatocytoma multiplex
- Digital mucoid cyst
- Milia
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
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
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
What is differential dx for a smooth skin colored nodule on the face?
BCC
Trichoepithelioma
Basloid follicular hamartoma
Microcystic adnexal carcinoma
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
What is Cowdens disease
Mutation of PTEN
Associated with
- Breast Ca, fibroadenoma
- GI polyps
- Thyroid cancer
Physical features
- tricholemmoma
- mucosalpapilloma
- arched palate
- craniomegaly
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
What is Muir torre syndrome
Mutation in MSH2/MLH1
Association
- Sebaceous neoplasm
- Keratoacanthoma
- Colon ca>GU
- Breast Ca
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
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
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
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
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
List benign dermal tumors
- Vascular
- Glomus tumor
- Angiokeratoma
- Pyogenic granuloma
- Neural
- Neurofibroma
- Fibrous
- Dermatofibroma
- Histiocytic
- Xanthoma
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
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
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
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
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
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
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
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)
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
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
