Epidermal Nevi, Neoplasms, and Cysts Part III Chapter 29 Michael Hohnadel 8/2005

Syringoma Small translucent papule Small translucent papule Commonly on eyelids or upper cheeks. Also may occur on: Axilla, abdomen, forehead, penis, vulva. Commonly on eyelids or upper cheeks. Also may occur on: Axilla, abdomen, forehead, penis, vulva. Develop slowly and persist indefinitely Develop slowly and persist indefinitely 18% of adults with Down’s syndrome 18% 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 cryotherapy. Treatment: Electrodessication, Laser ablation cryotherapy.

Syringoma Dilated sweat ducts with tadpole appearance.

Eruptive syringoma Numerous lesions on the neck, chest, axilla, upper arms and periumbilically. May resemble LP or 2 nd Syphilis as well as reticulated papillomatosis of Gougerot-Carteaud. Numerous lesions on the neck, chest, axilla, upper arms and periumbilically. May resemble LP or 2 nd Syphilis as well as reticulated papillomatosis of Gougerot-Carteaud. Young persons. Young persons. Histologically identical to solitary. Histologically identical to solitary. Reported in Down’s syndrome Reported in Down’s syndrome Clinically may be confused with reticulated papillomatosis of Gougerot-Carteaud Clinically may be confused with reticulated papillomatosis of Gougerot-Carteaud

Eccrine hidrocystomas Translucent papules 1-3mm which may have a bluish tint. Translucent papules 1-3mm which may have a bluish tint. Occur on the face. Occur on the face. Usually solitary, however, multiple lesions may be seen Usually solitary, however, multiple lesions may be seen May become more prominent in hot weather. May become more prominent in hot weather. Treatment – excision Topical atropine or scopolamine

Eccrine hidrocystomas Cyst cavity lined by double of of cuboidal cells.

Eccrine poroma Benign, slow-growing, slightly protruding, sessile, soft, reddish tumor Benign, slow-growing, slightly protruding, sessile, soft, reddish tumor Most commonly occur on the sole or the side of the foot. May occur anywhere. Most commonly occur on the sole or the side of the foot. May occur anywhere. Bleeds with slight trauma Bleeds with slight trauma Frequent cup-shaped shallow depression from which the tumor grows Frequent cup-shaped shallow depression from which the tumor grows Benign – simple excision Benign – simple excision Eccrine poromatosis Eccrine 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 longstanding eccrine poromas (50%) Most arise from longstanding eccrine poromas (50%) Clinically similar Clinically similar May also manifest as a blue or black nodule, plaque or ulcerated tumor May also manifest as a blue or black nodule, plaque or ulcerated tumor M=F, avg 70 yrs. M=F, avg 70 yrs. Distribution: Legs 30%, feet 20%, face 12%, thighs 8% If metastatic, 70% mortality Mohs 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 Syringoma 1. Firm intradermal or subcutaneous nodule, most commonly located on the nose or cheeks % involving the head and neck 3. Felt to be of eccrine origin Malig. Chon. Syringoma 1. Malignant mixed tumor of the skin 2. Most occur on extremities. Reported on face, scalp, back, buttocks 3. Grow rapidly. Metastasis more the 50% 4. Aggressive surgical excision, Adjuvant radiation therapy w/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 eccrine sweat gland tumor Classified as an eccrine sweat gland tumor Flesh colored or reddish, nodular protruding mass. May be solid or cystic. Flesh colored or reddish, nodular protruding mass. May be solid or cystic. Location: Anywhere. Most common site is the head Location: Anywhere. Most common site is the head 20% c/o pain on pressure Multiple lesions reported Women 2x > men Extirpation is TOC

Clear cell hidradenoma (nodular hidradenoma)

Scan power view. Note: epidermal connections are not usually present. Circumscribed nodular architecture. Clear cell hidradenoma (nodular hidradenoma)

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

Arrows point to some of the mucus producing cells. Clear cell hidradenoma (nodular hidradenoma)

Malignant clear cell hidradenoma (hidradenocarcinoma) Extremely rare Extremely rare Presents as a solitary nodule Presents as a solitary nodule Lower extremity 32.9 %, upper extremity 27.6 %, trunk 11.9 %, head 26.3 % Lower extremity 32.9 %, upper extremity 27.6 %, trunk 11.9 %, head 26.3 % Metastasis occurs 60% Metastasis occurs 60% Tx wide local excision, radiation and chemotherapy Tx wide local excision, radiation and chemotherapy

Eccrine spiradenoma Solitary, 1cm, deep-seated nodule. Most frequently seen on the ventral surface. Solitary, 1cm, deep-seated nodule. Most frequently seen on the ventral surface. Skin-colored, blue or pink with normal overlying skin. Skin-colored, blue or pink with normal overlying skin. Paroxysmal pain Especially upper half of the body Multiple lesions, linear pattern may be seen

Eccrine spiradenoma Benign clinical course Benign clinical course TX: Simple excision TX: Simple excision DDX may include: DDX may include: A - angiolipoma A - angiolipoma N - neuroma N - neuroma G - glomus tumor G - glomus tumor E L – leiomyoma L – leiomyoma

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

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

Papillary eccrine adenoma Uncommon benign lesion. Uncommon benign lesion. Dermal nodules, most common on extremities of black patients. Dermal nodules, most common on extremities of black patients. Tendency to recur. Tendency to recur. Treatment: Complete surgical excision Treatment: 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 solitary, hyperkeratotic nodule or plaque involving the extremities Most presentations are a solitary, hyperkeratotic nodule or plaque involving the extremities Characteristic marker of Schopf syndrome Characteristic marker of Schopf syndrome  Hydrocystomas of the eyelids, hypotrichosis, hypodontia, and nail abnormalities

cylindroma Other names: Dermal eccrine cylindroma, Spiegler’s tumor, turban tumor, and tomato tumor. Other names: Dermal eccrine cylindroma, Spiegler’s tumor, turban tumor, and tomato tumor. Pinkish to blue, solitary (usually), firm rubbery nodule on scalp or face. Pinkish to blue, solitary (usually), firm rubbery nodule on scalp or face. Women chiefly affected Women chiefly affected Slow growing. Rarely undergo malignant degeneration Slow growing. Rarely undergo malignant degeneration Treatment :excision Treatment :excision

cylindroma Dominantly inherited form: 1. Numerous rounded masses of various sizes on the scalp 2. Appears soon after puberty 3. Resembles bunches of grapes or small tomatoes

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

Sweat gland carcinoma Eccrine carcinoma 1. No characteristic clinical appearance 2. High incidence of metastatic spread Mucinous eccrine carcinoma 1. Commonly a round, elevated, reddish, and sometimes ulcerated mass 2. Usually head and neck (75%) 3. Slow growth, asymptomatic 4. 11% incidence of metastasis 5. Local excision

Aggressive digital papillary adenocarcinoma Aggressive malignancy involving the digit between the nail bed and the distal interphalangeal joint spaces in most cases. Aggressive malignancy involving the digit between the nail bed and the distal interphalangeal joint spaces in most cases. Presents as a solitary nodule Presents as a solitary nodule 50% recurrence rate. 50% metastasis. 50% recurrence rate. 50% metastasis. All patients should have CXR All patients should have CXR Complete excision TOC Complete excision TOC Amputation may be required. Amputation may be required.

Microcystic Adnexal Carcinoma (sclerosing sweat duct carcinoma) A very slow-growing plaque or nodule. Occurs most commonly on the upper lip of women. Central face. A very slow-growing plaque or nodule. Occurs most commonly on the upper lip of women. Central face. Perineural infiltration is common and may be extensive Perineural infiltration is common and may be extensive TOC Mohs. TOC Mohs. No reports of metastases No reports of metastases

Microcystic adnexal carcinoma

1.Poorly circumscribed. 2.Ducts. 3.Tumor islands 4.*deeper than wide*

APOCRINE GLANDS

ceruminoma Rare apoeccrine tumor that rarely becomes malignant Rare apoeccrine tumor that rarely becomes malignant Firm nodular mass in the EAC Firm nodular mass in the EAC Ulceration and crusting may occur Ulceration and crusting may occur Obstruction if large. Obstruction if large. Questionable true entity Questionable true entity Treatment - excision Treatment - excision

Hidradenoma papilliferum Benign solitary tumor Benign solitary tumor Almost exclusively on the vulva Almost exclusively on the vulva Bleeding, ulceration, discharge, itching and pain Bleeding, ulceration, discharge, itching and pain Firm nodule Firm nodule excision excision

Syringadenoma papilliferum (syringocystadenoma papilliferum) Most commonly develops in a nevus sebaceous of Jadassohn Most commonly develops in a nevus sebaceous of Jadassohn Scalp or face Scalp or face Firm rose red papules in groups, with vesicle-like inclusions. Firm rose red papules in groups, with vesicle-like inclusions. May simulate MC May simulate MC Transition to carcinoma is rare Excision is advised

Syringoma papilliferum

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

Apocrine hidrocystoma/cystadenoma (apocrine retention cyst) Solitary, dome- shaped, smooth- surfaced translucent nodule. Bluish or brownish. Solitary, dome- shaped, smooth- surfaced translucent nodule. Bluish or brownish. Benign tumor Benign tumor Occurs chiefly on the face. Penile shaft- median raphe cyst. Occurs chiefly on the face. Penile shaft- median raphe cyst. TX: Simple excision TX: 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 Rare Rare Axilla is the most common site Axilla is the most common site May be seen in the nipple, vulva and EAC May be seen in the nipple, vulva and EAC May originate from aberrant mammary glands May originate from aberrant mammary glands Widespread metastases may occur Widespread metastases may occur

HAIR FOLLICLE NEVI AND TUMORS

Pilomatricoma (calcifying epithelioma of Malherbe) Asymptomatic, solitary, deeply seated firm nodule, covered with normal or pink skin. Asymptomatic, solitary, deeply seated firm nodule, covered with normal or pink skin.  Stretching may show “tent sign” Most commonly on the face, neck or arms Most commonly on the face, neck or arms Derived from hair matrix cells Derived from hair matrix cells Clinical DDX is impossible Clinical DDX is impossible TX: Simple excision TX: Simple excision Familial patterns do occur Familial patterns do occur  Multiple in Rubinstein-Taybi and Gardner syndrome Malignant variety exist. Rare. Not aggressive. Malignant variety exist. Rare. Not aggressive.

pilomatricoma

Well circumscribed. Basophilic and eosinophic areas in continuity.

pilomatricoma Crowded basophilic cells blend into eosinophilic ‘ghost cells’ Ghost cells

Trichofolliculoma Benign, highly structured adenoma of the pilosebaceous unit Benign, highly structured adenoma of the pilosebaceous unit Small dome-shaped nodule on the face or scalp Small dome-shaped nodule on the face or scalp A small wisp of fine, immature hairs protrude from a central pore A small wisp of fine, immature hairs protrude from a central pore Simple excisional bx Simple excisional bx

Multiple follicles opening on central cystic space

Trichoepithelioma (epithelioma adenoides cysticum, multiple familial trichoepitheliomas) Occur as multiple cystic and solid nodules typically on the face.  Nodules are small, rounded, smooth, shiny, slightly translucent and firm.  Flesh colored or slightly reddish  Slightly depressed center  Often grouped and symmetrical Benign Benign

Solitary trichoepithelioma Solitary trichoepithelioma  Nonhereditary  Mostly on face Giant solitary trichoepithelioma Giant solitary trichoepithelioma  May reach several cm  Mostly on thigh and perianal Desmoplastic trichoepithelioma Desmoplastic trichoepithelioma  Difficult to differentiate from morphea-like BCC  Solitary or multiple on 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 cells Benign neoplasms of follicular germinative cells Asymptomatic Asymptomatic Scalp and face. Scalp and face. Surgical excision Surgical excision  Tend to ‘shell out’

Trichilemmoma and Cowden’s disease (multiple hamartoma syndrome) Benign neoplasm of outer root sheath of the hair follicle Benign neoplasm of outer root sheath of the hair follicle Small solitary papule on the face, esp nose and cheeks. Small solitary papule on the face, esp nose and cheeks. Multiple lesions are a marker for Cowden’s syndrome. Multiple lesions are a marker for Cowden’s syndrome. Cowden’s syndrome 87% of patients with Cowden’s develop tricholemmomas. 38% develop malignancies  Breast 25-36%  Thyroid 7%  Colon adenocarcinoma Tumor 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 areas Sun exposed areas Face and ears Face and ears Slow growing epidermal papule, indurated plaque or nodule with tendency to ulcerate Slow growing epidermal papule, indurated plaque or nodule with tendency to ulcerate Surgical excision Surgical excision

Trichodiscoma and fibrofolliculoma Trichodiscoma Hundreds of flat or dome-shaped, 2-4 mm skin-colored asymptomatic papules occuring on face, trunk and extremities. Hundreds of flat or dome-shaped, 2-4 mm skin-colored asymptomatic papules occuring on face, trunk and extremities. Autosomal dominant trait Autosomal dominant trait Controversial entity Controversial entity Fibrofoliculoma 2-4 mm. Solitary, more commonly multiple. Scattered over the face, trunk and extremities Birt – Hogg – Dube syn.  Renal CA. assoc.  Abdominal CT.

Slits in collagen

Proliferating trichilemmal cyst Large exophytic neoplasms Large exophytic neoplasms Almost exclusively confined to scalp and back of neck Almost exclusively confined to scalp and back of neck May ulcerate May ulcerate Assoc. with nevus sebaceous Assoc. with nevus sebaceous Metastasis may occur Metastasis may occur Most respond to surgical excision Most respond to surgical excision

Dermoid cyst Congenital in origin Congenital in origin Chiefly along lines of cleavage Chiefly along lines of cleavage Result from improper embryologic development Result from improper embryologic development Potential for intracranial communication Potential for intracranial communication CT or MRI scan is required to rule this out prior to BX over cranial cleavage planes CT or MRI scan is required to rule this out prior to BX over cranial cleavage planes Freely mobile and not attached to the skin Freely mobile and not attached to the skin

Pilonidal cyst Midline hairy patch or pit in the sacral region with a sinus orifice in the bottom, or a cyst beneath it Midline hairy patch or pit in the sacral region with a sinus orifice in the bottom, or a cyst beneath it Usually becomes symptomatic during adolescence Usually becomes symptomatic during adolescence Treatment: Opening cyst widely, debriding it, and packing it with silver nitrate crystals Treatment: Opening cyst widely, debriding it, and packing it with silver nitrate crystals SCC has been reported to arise from chronic inflammatory pilonidal disease SCC has been reported to arise from chronic inflammatory pilonidal disease

Pilonidal sinus

Steatocystoma simplex Noninheritable counterpart to the more familiar steatocystoma multiplex Noninheritable counterpart to the more familiar steatocystoma multiplex Face limbs or chest Face limbs or chest Simple excision Simple excision

Steatocystoma multiplex Multiple, small, yellowish, cystic nodules 2-6 mm in diameter. Multiple, small, yellowish, cystic nodules 2-6 mm in diameter.  Contain a syrup-like, yellowish, odorless oily material  Principally on the upper anterior trunk, upper arms, axillae and thighs  Lesions may be generalized High familial tendency hence likely an autosomal dominant inheritance. High familial tendency hence likely an autosomal dominant inheritance. Tx- excision of individual lesions Tx- excision of individual lesions Incision and expression or aspiration Incision and expression or aspiration

Steatocystoma multiplex

Steatocystoma Corrugated cyst wall. Cyst wall with mature sebaceous lobules

Eruptive vellus hair cysts Yellowish to reddish brown, small papules of the chest and proximal extremities Yellowish to reddish brown, small papules of the chest and proximal extremities Autosomal dominant inheritance Autosomal dominant inheritance Disseminated lesions reported Disseminated lesions reported

Milia White keratinous cysts, 1-4 mm White keratinous cysts, 1-4 mm Chiefly on the face esp under eyes Chiefly on the face esp under eyes May occur in great numbers May occur in great numbers Occur in up to 50 % of newborns Occur in up to 50 % of newborns Primarily develop without a predisposing condition Primarily develop without a predisposing condition Can develop in inflammatory conditions and skin diseases such as epidermolysis bullosa, pemphigus, bullous pemphigoid, PCT, herpes zoster, contact dermatitis, and after prolonged use of NSAIDS Can develop in inflammatory conditions and skin diseases such as epidermolysis bullosa, pemphigus, bullous pemphigoid, PCT, herpes zoster, contact dermatitis, and after prolonged use of NSAIDS

milia Variants include MEM (multiple eruptive milia) Variants include MEM (multiple eruptive milia)  May be familial. AD  MEP (milia en plaque) – many milia, post auricular. Tx Tx  incision and expression  Tretinoin and minocycline for MEP

milia

Pseudocyst of the auricle Fluctuant, tense, noninflammatory swelling of the upper ear Fluctuant, tense, noninflammatory swelling of the upper ear Believed to be assoc. with trauma Believed to be assoc. with trauma Tx – drainage Tx – drainage Intralesional steroids Intralesional steroids

Cutaneous columnar cysts Four types of cyst that occur in the skin are lined by columnar epithelium 1.) Branchiogenic cyst  Small solitary lesions just above the sternal notch 2.) Thyroglossal duct cysts  Anterior aspect of the neck  Malignancies reported 1%

Cutaneous columnar cysts 3.) Cutaneous ciliated cysts  Usually located on the legs of females  Perineum vulva and foot regions 4.) Median raphe cyst  Developmental defects lying in the ventral midline of the penis, usually on the glans  Surgical intervention is standard therapy

The End