epidermal nevi, neoplasms, and cysts part iii chapter 29 michael hohnadel 8/2005

Epidermal Nevi, Neoplasms, and Cysts Part III

Chapter 29

Michael Hohnadel 8/2005

Syringoma

Small translucent papuleSmall translucent papule Commonly on eyelids or upper cheeks. Also Commonly on eyelids or upper cheeks. Also

may occur on: Axilla, abdomen, forehead, may occur on: Axilla, abdomen, forehead, penis, vulva.penis, vulva.

Develop slowly and persist indefinitelyDevelop slowly and persist indefinitely 18% of adults with Down’s syndrome18% of adults with Down’s syndrome Dilated cystic sweat ducts.Dilated cystic sweat ducts. Clear cell variant assoc with DM.Clear cell variant assoc with DM. Treatment: Electrodessication, Laser ablation Treatment: Electrodessication, Laser ablation

cryotherapy.cryotherapy.

Syringoma

Dilated sweat ducts with tadpole appearance.

Eruptive syringoma

Numerous lesions on the neck, chest, axilla, upper arms and Numerous lesions on the neck, chest, axilla, upper arms and periumbilically. May resemble LP or 2periumbilically. May resemble LP or 2ndnd Syphilis as well as Syphilis as well as reticulated papillomatosis of Gougerot-Carteaud. reticulated papillomatosis of Gougerot-Carteaud.

Young persons. Young persons. Histologically identical to solitary.Histologically identical to solitary. Reported in Down’s syndromeReported in Down’s syndrome Clinically may be confused with reticulated papillomatosis of Clinically may be confused with reticulated papillomatosis of

Gougerot-CarteaudGougerot-Carteaud

Eccrine hidrocystomas Translucent papules Translucent papules

1-3mm which may 1-3mm which may have a bluish tint.have a bluish tint.

Occur on the face.Occur on the face. Usually solitary, Usually solitary,

however, multiple however, multiple lesions may be seen lesions may be seen

May become more May become more prominent in hot prominent in hot weather.weather.

Treatment – excisionTreatment – excision Topical atropine or Topical atropine or

scopolaminescopolamine

Eccrine hidrocystomas

Cyst cavity lined by double of of cuboidal cells.

Eccrine poroma

Benign, slow-growing, slightly protruding, Benign, slow-growing, slightly protruding, sessile, soft, reddish tumorsessile, soft, reddish tumor

Most commonly occur on the sole or the Most commonly occur on the sole or the side of the foot.side of the foot. May occur anywhere. May occur anywhere.

Bleeds with slight traumaBleeds with slight trauma Frequent cup-shaped shallow depression Frequent cup-shaped shallow depression

from which the tumor growsfrom which the tumor grows Benign – simple excisionBenign – simple excision Eccrine poromatosisEccrine poromatosis

Eccrine poroma

Eccrine poroma, scan power view. Note numerous epidermal connections by the tumor and the degree of acanthosis.

Eccrine poroma

Low power view of above showing the vascular stroma and relatively uniform cell population.

Eccrine poroma

High power view of the small kertinocyte population having distinct cytoplasmic borders.

Eccrine poroma

Malignant eccrine poroma(porocarcinoma)

Most arise from Most arise from longstanding eccrine longstanding eccrine poromas (50%)poromas (50%)

Clinically similarClinically similar May also manifest as a May also manifest as a

blue or black nodule, blue or black nodule, plaque or ulcerated plaque or ulcerated tumortumor

M=F, avg 70 yrs.M=F, avg 70 yrs.

Distribution: Legs 30%, Distribution: Legs 30%, feet 20%, face 12%, feet 20%, face 12%, thighs 8%thighs 8%

If metastatic, 70% If metastatic, 70% mortalitymortality

Mohs is TOCMohs is TOC

There are sharply demarcated nests of tumor within the epidermis. There is rim of normal epidermal keratinocytes in most areas

Malignant eccrine poroma

Atypia is prominent. There are no transitional atypical cells blending with the peripheral normal keratinocytes.

Malignant eccrine poroma

Chondroid Syringoma and Malignant Chondroid Syringoma

Chondroid SyringomaChondroid Syringoma1.1. Firm intradermal or Firm intradermal or

subcutaneous nodule, subcutaneous nodule, most commonly located most commonly located on the nose or cheekson the nose or cheeks

2.2. 80 % involving the head 80 % involving the head and neckand neck

3.3. Felt to be of eccrine Felt to be of eccrine originorigin

Malig. Chon. SyringomaMalig. Chon. Syringoma1.1. Malignant mixed tumor of the Malignant mixed tumor of the

skinskin

2.2. Most occur on extremities. Most occur on extremities. Reported on face, scalp, back, Reported on face, scalp, back, buttocksbuttocks

3.3. Grow rapidly. Metastasis more Grow rapidly. Metastasis more the 50%the 50%

4.4. Aggressive surgical excision, Aggressive surgical excision, Adjuvant radiation therapy Adjuvant radiation therapy w/wo chemotherapyw/wo chemotherapy

Chondroid Syringoma

Scanning power view of a sharply demarcated subcutaneous tumor. There are tubular foci, a cystic area and a solid component.

Chondroid Syringoma(mixed tumor of the skin)

A medium power view showing tubular differentiation.

Chondroid Syringoma

Clear cell hidradenoma(nodular hidradenoma)

Classified as an Classified as an eccrine sweat gland eccrine sweat gland tumortumor

Flesh colored or Flesh colored or reddish, nodular reddish, nodular protruding mass. May protruding mass. May be solid or cystic.be solid or cystic.

Location: Anywhere. Location: Anywhere. Most common site is Most common site is the headthe head

20% c/o pain on pressure20% c/o pain on pressure Multiple lesions reportedMultiple lesions reported Women 2x > menWomen 2x > men Extirpation is TOCExtirpation is TOC

Scan power view. Note: epidermal connections are not usually present. Circumscribed nodular architecture.


High power view. The luminal cells have some apocrine features. Some of the epithelial cells have clear cytoplasm (glycogen) and some do not.


Arrows point to some of the mucus producing cells.


Malignant clear cell hidradenoma(hidradenocarcinoma)

Extremely rareExtremely rare Presents as a solitary nodule Presents as a solitary nodule Lower extremity 32.9 %, upper extremity Lower extremity 32.9 %, upper extremity

27.6 %, trunk 11.9 %, head 26.3 %27.6 %, trunk 11.9 %, head 26.3 % Metastasis occurs 60%Metastasis occurs 60% Tx wide local excision, radiation and Tx wide local excision, radiation and

chemotherapychemotherapy

Eccrine spiradenoma Solitary, 1cm, deep-seated Solitary, 1cm, deep-seated

nodule. Most frequently nodule. Most frequently seen on the ventral seen on the ventral surface. surface.

Skin-colored, Skin-colored, blueblue or pink or pink with normal overlying with normal overlying skin.skin.

Paroxysmal painParoxysmal pain

Especially upper half Especially upper half of the bodyof the body

Multiple lesions, Multiple lesions, linear pattern may be linear pattern may be seenseen

Eccrine spiradenoma

Benign clinical courseBenign clinical course TX: Simple excisionTX: Simple excision

DDX may include:DDX may include: A - angiolipomaA - angiolipoma N - neuromaN - neuroma G - glomus tumorG - glomus tumor EE L – leiomyomaL – leiomyoma

Spiradenoma composed intertwining cords of light and dark cells having no visible cytoplasmic junctions.

Eccrine spiradenoma

Malignant eccrine spiradenoma

In long standing In long standing lesions malignant lesions malignant degeneration may degeneration may occur and my be occur and my be lethal. Malignant lethal. Malignant Eccrine SpiradenomaEccrine Spiradenoma

Papillary eccrine adenoma

Uncommon benign lesion.Uncommon benign lesion. Dermal nodules, most common on Dermal nodules, most common on

extremities of black patients.extremities of black patients. Tendency to recur.Tendency to recur. Treatment: Complete surgical excisionTreatment: Complete surgical excision

Papillary eccrine adenoma

Note the papillary intraluminal projections These and the long tubules help to differentiate this from a syringoma..

Eccrine syringofibroadenoma Most presentations are a Most presentations are a

solitary, hyperkeratotic solitary, hyperkeratotic nodule or plaque nodule or plaque involving the extremitiesinvolving the extremities

Characteristic marker of Characteristic marker of Schopf syndromeSchopf syndrome Hydrocystomas of the Hydrocystomas of the

eyelids, hypotrichosis, eyelids, hypotrichosis, hypodontia, and nail hypodontia, and nail abnormalitiesabnormalities

cylindroma Other names: Dermal eccrine cylindroma, Spiegler’s Other names: Dermal eccrine cylindroma, Spiegler’s

tumor, turban tumor, and tomato tumor.tumor, turban tumor, and tomato tumor. Pinkish to blue, solitary (usually), firm rubbery Pinkish to blue, solitary (usually), firm rubbery

nodule on scalp or face.nodule on scalp or face. Women chiefly affectedWomen chiefly affected Slow growing. Rarely undergo malignant Slow growing. Rarely undergo malignant

degenerationdegeneration Treatment :excisionTreatment :excision

cylindroma

Dominantly inherited formDominantly inherited form::

1.1. Numerous rounded Numerous rounded masses of various sizes on masses of various sizes on the scalpthe scalp

2.2. Appears soon after Appears soon after pubertypuberty

3.3. Resembles bunches of Resembles bunches of grapes or small tomatoesgrapes or small tomatoes

cylindroma

•Well fit jigsaw pattern.•Round aggregates of eosinophilic material (arrow)

Sweat gland carcinoma

Eccrine carcinomaEccrine carcinoma

1.1. No characteristic No characteristic clinical appearanceclinical appearance

2.2. High incidence of High incidence of metastatic spreadmetastatic spread

Mucinous eccrine carcinomaMucinous eccrine carcinoma

1.1. Commonly a round, elevated, Commonly a round, elevated, reddish, and sometimes reddish, and sometimes ulcerated massulcerated mass

2.2. Usually head and neck (75%)Usually head and neck (75%)

3.3. Slow growth, asymptomaticSlow growth, asymptomatic

4.4. 11% incidence of metastasis11% incidence of metastasis

5.5. Local excisionLocal excision

Aggressive digital papillary adenocarcinoma

Aggressive malignancy involving the digit Aggressive malignancy involving the digit between the nail bed and the distal between the nail bed and the distal interphalangeal joint spacesinterphalangeal joint spaces in most cases. in most cases.

Presents as a solitary nodulePresents as a solitary nodule 50% recurrence rate. 50% metastasis50% recurrence rate. 50% metastasis.. All patients should have CXRAll patients should have CXR Complete excision TOCComplete excision TOC Amputation may be required.Amputation may be required.

Microcystic Adnexal Carcinoma(sclerosing sweat duct carcinoma)

A very slow-growing plaque or nodule. A very slow-growing plaque or nodule. Occurs most commonly on the upper lip of Occurs most commonly on the upper lip of women. Central face.women. Central face.

Perineural infiltration is commonPerineural infiltration is common and may and may be extensivebe extensive

TOC Mohs.TOC Mohs. No reports of metastasesNo reports of metastases

Microcystic adnexal carcinoma

Microcystic adnexal carcinoma

1. Poorly circumscribed.2. Ducts.3. Tumor islands4. *deeper than wide*

APOCRINE GLANDS

ceruminoma

Rare apoeccrine tumor that rarely becomes Rare apoeccrine tumor that rarely becomes malignantmalignant

Firm nodular mass in the EACFirm nodular mass in the EAC Ulceration and crusting may occurUlceration and crusting may occur Obstruction if large.Obstruction if large. Questionable true entityQuestionable true entity Treatment - excisionTreatment - excision

Hidradenoma papilliferum

Benign solitary tumorBenign solitary tumor Almost exclusively on Almost exclusively on

the vulvathe vulva Bleeding, ulceration, Bleeding, ulceration,

discharge, itching and discharge, itching and painpain

Firm noduleFirm nodule excisionexcision

Syringadenoma papilliferum(syringocystadenoma papilliferum)

Most commonly Most commonly develops in a nevus develops in a nevus sebaceous of sebaceous of JadassohnJadassohn

Scalp or faceScalp or face Firm rose red papules Firm rose red papules

in groups, with in groups, with vesicle-like inclusions.vesicle-like inclusions.

May simulate MCMay simulate MC Transition to Transition to

carcinoma is rarecarcinoma is rare Excision is advisedExcision is advised

Syringoma papilliferum

Syringoma papilliferum

•Irreg. tubules lined by double cell layer with decapitation secretion. •Plasma cells in stroma

Apocrine hidrocystoma/cystadenoma(apocrine retention cyst)

Solitary, dome-Solitary, dome-shaped, smooth-shaped, smooth-surfaced translucent surfaced translucent nodule. Bluish or nodule. Bluish or brownish. brownish.

Benign tumorBenign tumor Occurs chiefly on the Occurs chiefly on the

face. Penile shaft- face. Penile shaft- median raphe cyst.median raphe cyst.

TX: Simple excisionTX: Simple excision

Apocrine hidrocystoma

Large cystic spaces. Decapitation secretion

A high power view of the linings of two of the cysts. A few brown, lipofuchsin pigment granules (PG) are in the basilar part of the epithelium. Apocrine snouts are prominent.

Apocrine hidrocystoma

Apocrine gland carcinoma

RareRare Axilla is the most common siteAxilla is the most common site May be seen in the nipple, vulva and EACMay be seen in the nipple, vulva and EAC May originate from aberrant mammary May originate from aberrant mammary

glandsglands Widespread metastases may occurWidespread metastases may occur

HAIR FOLLICLE NEVI AND TUMORS

Pilomatricoma(calcifying epithelioma of Malherbe)

Asymptomatic, solitary, deeply seated firm nodule, Asymptomatic, solitary, deeply seated firm nodule, covered with normal or pink skin. covered with normal or pink skin. Stretching may show “tent sign”Stretching may show “tent sign”

Most commonly on the face, neck or armsMost commonly on the face, neck or arms Derived from hair matrix cellsDerived from hair matrix cells Clinical DDX is impossibleClinical DDX is impossible TX: Simple excisionTX: Simple excision Familial patterns do occurFamilial patterns do occur

Multiple in Rubinstein-Taybi and Gardner Multiple in Rubinstein-Taybi and Gardner syndromesyndrome

Malignant variety exist. Rare. Not aggressive.Malignant variety exist. Rare. Not aggressive.

pilomatricoma

pilomatricoma

Well circumscribed. Basophilic and eosinophic areas in continuity.

pilomatricoma

Crowded basophilic cells blend into eosinophilic ‘ghost cells’

Ghost cells

Trichofolliculoma Benign, highly Benign, highly

structured adenoma of structured adenoma of the pilosebaceous unitthe pilosebaceous unit

Small dome-shaped Small dome-shaped nodule on the face or nodule on the face or scalpscalp

A small wisp of fine, A small wisp of fine, immature hairs immature hairs protrude from a protrude from a central porecentral pore

Simple excisional bxSimple excisional bx

Multiple follicles opening on central cystic space

Trichoepithelioma(epithelioma adenoides cysticum, multiple

familial trichoepitheliomas)Occur as multiple cystic and Occur as multiple cystic and

solid nodules typically on the solid nodules typically on the face. face. Nodules are small, Nodules are small,

rounded, smooth, shiny, rounded, smooth, shiny, slightly translucent and slightly translucent and firm.firm.

Flesh colored or slightly Flesh colored or slightly reddishreddish

Slightly depressed centerSlightly depressed center Often grouped and Often grouped and

symmetricalsymmetrical BenignBenign

Solitary trichoepitheliomaSolitary trichoepithelioma NonhereditaryNonhereditary Mostly on faceMostly on face

Giant solitary Giant solitary trichoepitheliomatrichoepithelioma May reach several cmMay reach several cm Mostly on thigh and Mostly on thigh and

perianalperianal Desmoplastic Desmoplastic

trichoepitheliomatrichoepithelioma Difficult to Difficult to

differentiate from differentiate from morphea-like BCCmorphea-like BCC

Solitary or multiple on Solitary or multiple on the face the face

trichoepithelioma

The tumor is composed of lobules with anastomosing streaks of uniform basaloid cells congregated in immature hair cell structures without atypia, all surrounded by a fibromyxoid stroma.

trichoblastoma

Benign neoplasms of follicular germinative Benign neoplasms of follicular germinative cellscells

AsymptomaticAsymptomatic Scalp and face. Scalp and face. Surgical excisionSurgical excision

Tend to ‘shell out’Tend to ‘shell out’

Trichilemmoma and Cowden’s disease (multiple hamartoma syndrome)

Benign neoplasm of Benign neoplasm of outer root sheath of outer root sheath of the hair folliclethe hair follicle

Small solitary papule Small solitary papule on the face, esp nose on the face, esp nose and cheeks.and cheeks.

Multiple lesions are a Multiple lesions are a marker for Cowden’s marker for Cowden’s syndrome.syndrome.

Cowden’s syndromeCowden’s syndrome 87% of patients with 87% of patients with

Cowden’s develop Cowden’s develop tricholemmomas.tricholemmomas.

38% develop 38% develop malignanciesmalignancies Breast 25-36%Breast 25-36% Thyroid 7%Thyroid 7% Colon Colon

adenocarcinomaadenocarcinoma Tumor suppressor geneTumor suppressor gene

Trichilemmoma

Oriented about a hair folicle. Varying degrees of clear cell differentiation. May have palisading. No mucin helps differentiate from BCC.

Trichilemmal carcinoma Sun exposed areasSun exposed areas Face and earsFace and ears Slow growing Slow growing

epidermal papule, epidermal papule, indurated plaque or indurated plaque or nodule with tendency nodule with tendency to ulcerateto ulcerate

Surgical excisionSurgical excision

Trichodiscoma and fibrofolliculoma

TrichodiscomaTrichodiscoma Hundreds of flat or Hundreds of flat or

dome-shaped, 2-4 mm dome-shaped, 2-4 mm skin-colored skin-colored asymptomatic papules asymptomatic papules occuring on face, occuring on face, trunk and extremities.trunk and extremities.

Autosomal dominant Autosomal dominant traittrait

Controversial entityControversial entity

FibrofoliculomaFibrofoliculoma 2-4 mm. Solitary, more 2-4 mm. Solitary, more

commonly multiple.commonly multiple. Scattered over the face, Scattered over the face,

trunk and extremitiestrunk and extremities Birt – Hogg – Dube syn.Birt – Hogg – Dube syn.

Renal CA. assoc.Renal CA. assoc. Abdominal CT.Abdominal CT.

Slits in collagen

Proliferating trichilemmal cyst

Large exophytic Large exophytic neoplasmsneoplasms

Almost exclusively Almost exclusively confined to scalp and confined to scalp and back of neckback of neck

May ulcerateMay ulcerate Assoc. with nevus Assoc. with nevus

sebaceoussebaceous Metastasis may occurMetastasis may occur Most respond to surgical Most respond to surgical

excisionexcision

Dermoid cyst Congenital in originCongenital in origin Chiefly along lines of Chiefly along lines of

cleavagecleavage Result from improper Result from improper

embryologic developmentembryologic development Potential for intracranial Potential for intracranial

communicationcommunication CT or MRI scan is required CT or MRI scan is required

to rule this out prior to BX to rule this out prior to BX over cranial cleavage over cranial cleavage planesplanes

Freely mobile and not Freely mobile and not attached to the skinattached to the skin

Pilonidal cyst Midline hairy patch or pit in the sacral Midline hairy patch or pit in the sacral

region with a sinus orifice in the bottom, or region with a sinus orifice in the bottom, or a cyst beneath ita cyst beneath it

Usually becomes symptomatic during Usually becomes symptomatic during adolescenceadolescence

Treatment: Opening cyst widely, debriding Treatment: Opening cyst widely, debriding it, and packing it with silver nitrate crystalsit, and packing it with silver nitrate crystals

SCC has been reported to arise from SCC has been reported to arise from chronic inflammatory pilonidal diseasechronic inflammatory pilonidal disease

Pilonidal sinus

Steatocystoma simplex

Noninheritable Noninheritable counterpart to the counterpart to the more familiar more familiar steatocystoma steatocystoma multiplexmultiplex

Face limbs or chestFace limbs or chest Simple excisionSimple excision

Steatocystoma multiplex Multiple, small, yellowish, cystic nodules 2-6 mm Multiple, small, yellowish, cystic nodules 2-6 mm

in diameter. in diameter. Contain a syrup-like, yellowish, odorless oily Contain a syrup-like, yellowish, odorless oily

materialmaterial Principally on the upper anterior trunk, upper Principally on the upper anterior trunk, upper

arms, axillae and thighsarms, axillae and thighs Lesions may be generalizedLesions may be generalized

High familial tendency hence likely an autosomal High familial tendency hence likely an autosomal dominant inheritance.dominant inheritance.

Tx- excision of individual lesionsTx- excision of individual lesions Incision and expression or aspirationIncision and expression or aspiration

Steatocystoma multiplex

Steatocystoma

•Corrugated cyst wall.•Cyst wall with mature sebaceous lobules

Eruptive vellus hair cysts

Yellowish to reddish Yellowish to reddish brown, small papules brown, small papules of the chest and of the chest and proximal extremitiesproximal extremities

Autosomal dominant Autosomal dominant inheritanceinheritance

Disseminated lesions Disseminated lesions reportedreported

Milia

White keratinous cysts, 1-4 mmWhite keratinous cysts, 1-4 mm Chiefly on the face esp under eyesChiefly on the face esp under eyes May occur in great numbersMay occur in great numbers Occur in up to 50 % of newbornsOccur in up to 50 % of newborns Primarily develop without a predisposing Primarily develop without a predisposing

conditioncondition Can develop in inflammatory conditions and skin Can develop in inflammatory conditions and skin

diseases such as epidermolysis bullosa, diseases such as epidermolysis bullosa, pemphigus, bullous pemphigoid, PCT, herpes pemphigus, bullous pemphigoid, PCT, herpes zoster, contact dermatitis, and after prolonged use zoster, contact dermatitis, and after prolonged use of NSAIDSof NSAIDS

milia

Variants include MEM (multiple eruptive Variants include MEM (multiple eruptive milia)milia) May be familial. ADMay be familial. AD MEP (milia en plaque) – many milia, MEP (milia en plaque) – many milia,

post auricular.post auricular. TxTx

incision and expressionincision and expression Tretinoin and minocycline for MEPTretinoin and minocycline for MEP

Pseudocyst of the auricle

Fluctuant, tense, noninflammatory swelling Fluctuant, tense, noninflammatory swelling of the upper earof the upper ear

Believed to be assoc. with traumaBelieved to be assoc. with trauma Tx – drainageTx – drainage Intralesional steroidsIntralesional steroids

Cutaneous columnar cysts

Four types of cyst that occur in the skin are Four types of cyst that occur in the skin are lined by columnar epitheliumlined by columnar epithelium

1.) Branchiogenic cyst1.) Branchiogenic cyst Small solitary lesions just above the Small solitary lesions just above the

sternal notchsternal notch2.) Thyroglossal duct cysts2.) Thyroglossal duct cysts

Anterior aspect of the neckAnterior aspect of the neck Malignancies reported 1%Malignancies reported 1%

Cutaneous columnar cysts

3.) Cutaneous ciliated cysts3.) Cutaneous ciliated cysts Usually located on the legs of femalesUsually located on the legs of females Perineum vulva and foot regionsPerineum vulva and foot regions

4.) Median raphe cyst4.) Median raphe cyst Developmental defects lying in the Developmental defects lying in the

ventral midline of the penis, usually on ventral midline of the penis, usually on the glansthe glans

Surgical intervention is standard therapySurgical intervention is standard therapy

The End

epidermal nevi, neoplasms, and cysts part iii chapter 29 michael hohnadel 8/2005

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