adnexal neoplasms

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DEVELOPMENT OF HAIR DURING EARLY GESTATION

CLASSIFICATION OF ADNEXAL NEOPLASMS

I. NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE

II. NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION

III. NEOPLASMS AND PROLIFERATIONS WITH APOCRINE DIFFERENTIATION

IV. NEOPLASMS AND PROLIFERATIONS WITH ECCRINE DIFFERENTIATION

NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE

NEOPLASMS AND PROLIFERATIONS OF FOLLICULAR LINEAGE

I. Follicular and Folliculosebaceous-apocrine Hamartomas

1. Trichofolliculoma

2. Fibrofolliculoma, perifollicular fibroma and

trichodiscoma

3. Nevus sebaceous

4. Mixed tumor (chondroid syringoma)

II. Neoplasms and Proliferations with Follicular

Germinative Differentiation• Trichoepithelioma/Trichoblastoma

III. Neoplasms and Proliferations with Matrical

Differentiation

1. Pilomatricoma2. Pilomatrical carcinoma

IV. Neoplasms and Proliferations with Follicular Sheath

(Trichilemmal) Differentiation

1. Trichilemmoma2. Trichilemmal carcinoma

V. Neoplasms and Proliferations with Superficial Follicular

(Infundibular and Isthmic) Differentiation

1. Tumor of follicular infundibulum (isthmicoma)2. Trichoadenoma (trichoadenoma of

Nikolowski)3. Proliferating pilar tumor (proliferating

follicular-cystic neoplasm)

TRICHOFOLLICULOMA

TRICHOFOLLICULOMA

It is an uncommon small benign tumor originating from the hair follicle usually in the second decade of life.

Trichofolliculoma does not represent a true neoplasm. Rather, the term indicates a group of follicularhamartomas in which fully formed follicular structuresemerge from a central dilated infundibular space.

It has an excellent prognosis.

TRICHOFOLLICULOMAWISPY VELLUS HAIRS EMERGE FROM A SKIN-COLORED

PAPULE WITH A DILATED CENTRAL PORE

TRICHOFOLLICULOMA

C/P OF TRICHOFOLLICULOMA

A small, solitary flesh-colored or whitish papule that occurs most often on the face around the nose region, scalp or upper trunk.

Sometimes the lesion have a central pore (follicularostium or punctum from which it exudes sebum).

In some cases a characteristic small tuft of white hairmay be growing from the central pore.

Rarely, the clinical presentation will be as a largenodule or cyst.

HISTO. OF TRICHOFOLLICULOMA Central cystic space with infundibular cornification and

central orthokeratin with an opening onto the epidermalsurface.

Cross-sections of vellus hair shafts are identifiable within the cyst.

Relatively well-developed and occasionally oddly formed secondary and tertiary follicles protrude in radial fashion from the central structure.

The follicles usually display isthmic differentiation, bulb,papilla and exhibit inner and outer sheath.

The entire structure, including central cyst and its associated radiating secondary and tertiary follicles, is enveloped by a well circumscribed vascularized fibrous dense stroma.

HISTO. OF TRICHOFOLLICULOMATHERE IS A CENTRAL PATULOUS FOLLICULAR INFUNDIBULUM,

FROM WHICH FULLY FORMED OR NEARLY FULLY FORMED

FOLLICLES RADIATE

HISTO. OF TRICHOFOLLICULOMA

SEBACEOUS TRICHOFOLLICULOMA

IT IS A VARIANT OF

TRICHOFOLLICULOMA

DEMONSTRATING NUMEROUS WELL

DIFFERENTIATED SEBACEOUS LOBULES

EMPTYING INTO THE CENTRAL

PRIMARY FOLLICLE AND MAY BE LARGE

AND CYSTIC.

Rx OF TRICHOFOLLICULOMA

It is wholly benign and no treatment is needed.

If a trichofolliculoma is discovered by biopsy, no further intervention is required.

Occasionally they may be removed for cosmetic reasons or if they occur in functionally sensitive areas.

TREATMENT OPTIONS INCLUDE; 1. Curettage and electrodesiccation.2. Surgical excision.

NEVUS SEBACEOUS

NEVUS SEBACEOUS (OF JADASSOHN)

Originally known as ORGANOID NEVUS because it may include components of the entire skin.

It is an uncommon type of birthmark most often found on the scalp representing a classic nevus or congenital hamartoma.

It is due to postzygotic mutation and genetic mosaicism.

Nevus sebaceous is commonly thought of as a sebaceouslesion but it is a non-neoplastic malformation that includesepidermal, follicular, sebaceous, apocrine and connectivetissue elements.

C/P OF NEVUS SEBACEOUS

In an infant or young child, nevus sebaceous presents as a solitary, yellow-orange hairless patch, often oval or linear in shape with smooth or somewhat velvety surface.

It involves the scalp (the nevus will remain hairless, as the infant’s hair grows around it) or face commonly, the neckoccasionally, and the trunk rarely.

Lesions that are linear are distributed along the lines ofBlaschko, although this may be difficult to appreciate if small.

During childhood, the nevus thickens slightly.


At adolescence, progressive thickening occurs and the surface becomes verrucous. It represents a fertile field for the development of

secondary adnexal neoplasms, commonly benign but occasionally malignant.

NEVUS SEBACEOUS

NEVUS SEBACEOUS OF FOREHEAD

NEVUS SEBACEOUS OF THE CHEEK

LINEAR NEVUS SEBACEOUS

NEVUS SEBACEOUS ON THE SCALP (A) AND CHIN (B) OF THE SAME PATIENT

TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS

SYRINGOCYSTADENOMA PAPILLIFERUM ON TOP OF NEVUS SEBACEOUS

BASAL CELL CARCINOMA ON TOP OF NEVUS SEBACEOUS


TRICHOBLASTOMA is the most common secondary proliferation. Other common secondary neoplasms include syringocystadenoma papilliferum, trichilemmoma, desmoplastic trichilemmoma, sebaceous adenoma, apocrine adenoma, and poroma.

The actual incidence of secondary BCC is less than 1%. Only rarely does secondarysebaceous carcinoma or apocrine carcinoma arise in a nevus sebaceous. Malignancy probably develops only in longstanding or neglected lesions. Most tumors occur in adults older than 40 years.

NEVUS SEBACEOUS SYNDROME Nevus sebaceous syndrome refers to the rare association of a large nevus

sebaceous with disorders of the eye, brain and skeleton.

Sebaceous naevus syndrome may result in eye tumors and the skull may be

asymmetrical. Characteristic associated neurological features may include:

1. Developmental delay.

2. Epileptic seizures, especially infantile spasms.

3. Hemiparesis or cranial nerve palsies.

4. X-ray images and ultrasound evaluation may be quite normal.

5. Various structural abnormalities may be found within the brain.

HISTO. OF NEVUS SEBACEOUS

Infantile nevus sebaceous malformed follicular units are small and

deviate little from normal.

Childhood tiny deformed immature hair follicles provide a stark

contrast to normal terminal follicles at the periphery of the biopsy.

Small sebaceous and apocrine glands may be present. The epidermis

also thickens and becomes progressively more papillated, gradually

assuming a configuration akin to an epidermal nevus.

HISTO. OF NEVUS SEBACEOUS

late adolescence there is thickening of the epidermis with acanthosis

and papillomatosis. Clusters of follicular germinative cells positioned

along the dermal–epidermal junction. The underlying follicular units

remain smallish and appear distorted but their sebaceous lobules

increase in prominence. Dilated apocrine glandular structures with

inspissated secretions are not uncommonly identifiable in the reticular

dermis. This histopathology remains stable into adulthood.

NEVUS SEBACEUSMEDIUM MAGNIFICATION REVEALS EXPANSION OF THE EPIDERMIS IN A PAPILLATED FASHION, MUCH

LIKE THE PATTERN OF AN EPIDERMAL NEVUS.

THIS IS ACCOMPANIED BY ADNEXAL MALFORMATION, WITH ENLARGED SEBACEOUS

LOBULES AND CLUSTERS OF FOLLICULAR GERMINATIVE CELLS POSITIONED ALONG THE

DERMAL–EPIDERMAL JUNCTION.

NEVUS SEBACEUSPAPILLOMATOUS EPIDERMAL

HYPERPLASIA, WITH BASKET WEAVE

HYPERKERATOSIS AND HYPERTROPHIC

SEBACEOUS GLANDS.

NEVUS SEBACEUSSEBACEOUS GLAND OPENING INTO THE

EPIDERMIS.

HISTO. OF NEVUS SEBACEOUS Over time, some patients develop exaggerated verrucous epidermal

hyperplasia much like a verruca vulgaris.

Secondary neoplasms are common, and trichoblastoma,

trichilemmoma and syringocystadenoma are frequently observed. The

strong association between syringocystadenoma and nevus sebaceus

warrants careful inspection any time the diagnosis of

syringocystadenoma is made, to find out whether a nevus sebaceus can

be found in the same specimen.

Rx OF NEVUS SEBACEOUS Conservativecomplete excision in late childhood represents a commonlyutilized

approach due to; 1. Nevus sebaceous becomes increasingly verrucous and unsightly over time.2. The risk for the development of a secondary benign neoplasm, such as trichoblastoma or

syringocystadenoma, as it is relatively high.3. The risk of development of secondary carcinoma although it is low. 4. Lesions within the scalp may be difficult to follow clinically.

For facial lesions, consideration should be given to excision during childhood, beforethe development of secondary verrucous alteration, at a time when the risk ofscarring is reduced compared to adulthood.

The excisional margins can be minimal. Removal by shave or laser ablation is usually not successful.

TRICHOEPITHELIOMA

TRICHOEPITHELIOMA

Trichoepithelioma is a benign cutaneous neoplasms that originates from germinative cells of hair follicles.

It occurs as a sporadic non-familial form or as multipletrichoepitheliomas that is transmitted as an autosomaldominant trait. Lesions first appear in childhood and gradually increase in number with age.

For many, in the current dermatopathologic terms, trichoepithelioma represents a variant of trichoblastoma.

MULTIPLE TRICHOEPITHELIOMAS

ASYMPTOMATIC, MULTIPLE, FIRM, TRANSLUCENT

PAPULES INVOLVED THE MIDFACE ESPECIALLY THE

NASOLABIAL FOLDS.

MULTIPLE FAMILIAL TRICHOEPITHELIOMAS

NUMEROUS SKIN-COLORED PAPULES AND NODULES ON

THE MIDFACE .

C/P OF TRICHOEPITHELIOMA

CLINICAL VARIANTS OF TRICHOEPITHELIOMA:

1. Solitary trichoepithelioma

2. Multiple Trichoepitheliomas either isolated or in association with

Brooke-Spiegler Syndrome.

3. Desmoplastic trichoepithelioma.

4. Trichoblastoma.


1. SOLITARY TRICHOEPITHELIOMA occurs more

commonly than multiple trichoepitheliomas usually

presents as a firm small (usually less than one

centimeter), skin-colored, yellow or pink dome-

shaped shiny papule or nodule on the face (lesions

have a predilection for the nose) or upper trunk.

C/P OF TRICHOEPITHELIOMA2. MULTIPLE TRICHOEPITHELIOMAS are yellowish-pink,

translucent dome-shaped papules distributed

symmetrically on the nasolabial areas, cheeks, eyelids,

forehead and upper lip occasionally occur on the neck and

the upper trunk.

The lesions may cause disfigurement because of

involvement of the face.

Often inherited as an autosomal dominant trait, the

papules first appear during childhood and grow slowly for

years and usually gradually increase in number with age.


BROOKE-SPIEGLER SYNDROME, is a rare genetic conditionresults in predisposition to three types of benign skinappendage tumor.

1. Trichoepitheliomas: with lesional density is always greatest in the central face is associated with a risk of secondary BCC.

2. Cylindromas: solitary or multiple tumors on the scalp may transform into cylindrocarcinoma.

3. Spiradenomas: painful nodules on head, neck and trunk may transform into spiradenocarcinoma.


3.DESMOPLASTIC TRICHOEPITHELIOMA represents a

variant of trichoepithelioma with exaggerated stromal

sclerosis (desmoplasia).

Commonly presents as a shiny firm skin colored to

erythematous annular lesion with a central dimple usually

solitary on the cheek of a woman. Mostly does not exceed

1 cm in diameter.

DESMOPLASTIC TRICHOEPITHELIOMA

PRESENT ON THE CHEEK OF A WOMAN

C/P OF TRICHOEPITHELIOMA4. TRICHOBLASTOMA: is very rare and tend to be larger and more

deeply located (in the deep dermis and subcutaneous tissue) thanclassical trichoepithelioma which is more superficial.

Presents as a small well circumscribed, solitary of skin-colored to brown or blue-black papules or nodules, usually 1-2 cm in diameter. They most commonly occur on the face and scalp of adults around 40-50 years of age.

Trichoblastoma is the most common neoplasm developed in pre-existing nevus sebaceus.

It may occasionally co-exist with BCC. Some trichoblastoma may also transform into the malignant counterpart TRICHOBLASTICCARCINOMA, which can be locally aggressive and have the potential to spread and metastasize.

TRICHOBLASTOMA ON TOP OF NEVUS SEBACEOUS

HISTO. OF TRICHOEPITHELIOMA As they are benign neoplasms relative symmetry, circumscription

and a lack of cytologic atypicality. Histological features are multiple dermal nodules of basaloid cells

surrounded by fibrous stroma (similar to BCC). Prominence of follicular germinative basaloid cells that have scant

cytoplasm and dark staining nuclei and show striking peripheralpalisading with surrounding fibrocytic stroma that varies in degree.They are often arranged as small clusters or as reticulate and cribriform cords.

Small cornifying cystic spaces containing laminated keratin (horncysts) and a lining of pinkish squamous cells (keratinocytes), reflecting concurrent infundibular or isthmic differentiation, are also apparent.

HISTO. OF TRICHOEPITHELIOMA

In classic trichoepithelioma, the fibrocytic stroma is obvious and constitutes as much as half of the cellularity of the lesion or may be arrayed as either small or large nodules with only scantintervening sclerotic stroma.

A distinctive feature is the papillary mesenchymal bodies (foci of fibroblastic bulbar differentiation resembling abortive follicularpapillae).

There may be tiny collections of mature sebocytes, reflecting concurrent sebaceous differentiation, and (apocrine) ductaldifferentiation may also be noted.

TRICHOEPITHELIOMATHIS “CLASSIC” TRICHOEPITHELIOMA IS COMPOSED MOSTLY OF

CLUSTERS OF FOLLICULAR GERMINATIVE CELLS BUT ALSO

SHOWS SUPERFICIAL FOLLICULAR DIFFERENTIATION, WITH

SMALL KERATINIZING CYSTIC SPACES.

NOTE THAT CLEFTS WITHIN THE PROLIFERATION ARE

BETWEEN STROMAL ELEMENTS, IN CONTRAST TO THE CLEFTS

BETWEEN TUMOR AND STROMA THAT ARE CHARACTERISTIC OF

BASAL CELL CARCINOMA.

HISTOLOGY OF TRICHOEPITHELIOMA

WELL-DEMARCATED NODULES CONSIST OF SMALL

BASALOID CELLS THAT ARE ARRANGED IN A

PALISADE-LIKE PATTERN; MULTIPLE HORN CYSTS

WITH A FULLY KERATINIZED CENTER SURROUNDED

BY BASALOID CELLS LIE FREE IN THE FIBROUS

STROMA.

HISTOLOGY OF TRICHOEPITHELIOMA

THE CYSTIC SPACES CONTAIN KERATIN.

NOTICE THE LACK OF MITOTIC FIGURES OR

APOPTOTIC BODIES.

DESMOPLASTIC TRICHOEPITHELIOMA

IT IS COMPOSED OF THIN STRANDS OF

BASALOID (FOLLICULAR GERMINATIVE) CELLS,

ARRAYED IN SCLEROTIC STROMA.

MUCH LIKE CONVENTIONAL

TRICHOEPITHELIOMA, THERE ARE FOCI OF

SUPERFICIAL FOLLICULAR KERATINIZATION.

HIGH-POWERED VIEW OF DESMOPLASTIC

TRICHOEPITHELIOMANOTICE THE CLUSTER OF SQUAMOUS CELLS

SURROUNDING A SMALL CYSTIC AREA CONTAINING

KERATIN. INTERVENING STROMA IS MARKEDLY FIBROUS.

Rx OF TRICHOEPITHELIOMA Trichoblastoma is a benign condition so no treatment may be indicated but follow-up

for the possibility of secondary BCC.

Individual lesions may be removed surgically but scarring may occur.

Multiple facial trichoepitheliomas can be cosmetically disabling and because of the number of lesions, conventional excision is not indicated.

Other ablative approaches have been employed with some success but partialdestruction of the tumor is usually followed by regrowth. These include;

1. Electrodesiccation

2. Laser3. Dermabrasion

4. Cryotherapy

LASER SURGERY OF MULTIPLE TRICHOEPITHELIOMAS

IMMEDIATELY POST-PROCEDURE WITH

THE CO2 LASER AND

ELECTRODESICCATION OF LARGER

NODULES

AFTER TWO SESSIONS WITH CO2

LASER SURGERY

AFTER THREE YEAR FOLLOW-UPBEFORE TREATMENT

NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION

NEOPLASMS AND PROLIFERATIONS WITH SEBACEOUS DIFFERENTIATION

1. SEBACEOUS GLAND HYPERPLASIA

2. SEBACEOUS ADENOMA

3. SEBACEOUS EPITHELIOMA AND SEBACEOMA

4. SEBACEOUS CARCINOMA

SEBACEOUS GLAND HYPERPLASIA

SEBACEOUS GLAND HYPERPLASIA (SGH)

SGH is relatively common and does not

represent a true neoplasm. It represents

benign enlargement of sebaceous glands

usually seen on the forehead or cheeks of

the middle-aged and elderly.

SEBACEOUS HYPERPLASIA

C/P OF SEBACEOUS GLAND HYPERPLASIA

Presents with single or multiple, small, shiny, yellowish,up to 5 mm in diameter papules, usually on the central orupper face and sometimes on the upper trunk.

Commonly, the clinical lesions display a centralumbilication that corresponds to a central follicularinfundibular ostium.

There are often telangiectasia best seen using dermoscopy.

C/P OF SEBACEOUS GLAND HYPERPLASIA

Sebaceous hyperplasia may be more prevalent in immunosuppressed patients: for example, in a patient following organ transplantation.

Juxta-clavicular beaded lines - variant of sebaceous hyperplasia characterized by papulesarranged in parallel rows resembling 'strands ofbeads'.

SEBACEOUS HYPERPLASIASKIN COLORED TO YELLOWISH PAPULES WITH A CENTRAL

DELL

SEBACEOUS HYPERPLASIA

SEBACEOUS HYPERPLASIANUMEROUS LESIONS OF SEBACEOUS HYPERPLASIA IN A SOLID

ORGAN TRANSPLANT RECIPIENT RECEIVING LONG-TERM

CYCLOSPORINE

HISTO. OF SEBACEOUS GLAND HYPERPLASIA

The sebaceous gland in SGH shows normal

morphology.

The enlarged numerous sebaceous lobules usually

circumferentially surround a central infundibulum.

Sebaceous lobules composed of mature sebocytes

with a thin rim of seboblasts.

Rx OF SEBACEOUS GLAND HYPERPLASIA

MEDICAL:

• Topical retinoid for long-term application may be beneficial.

• Oral isotretinoin may be utilized for patients with extensive disfiguring lesions but these

may recur when treatment is stopped.

SURGICAL: If treatment is desired, lesions can be removed or diminished by;

1. Shave excision

2. Light electrosurgical destruction

3. Cryotherapy

4. Laser ablation

SEBACEOUS ADENOMA/SEBACEOMA

SEBACEOUS ADENOMA/SEBACEOMA Sebaceous adenoma is superficial benign

neoplasms in which there is a proliferation of

mature sebocytes and seboblasts.

Sebaceoma is deeply seated benign neoplasms

in which there is a proliferation of mature

sebocytes and seboblasts but seboblasts

predominates.

C/P OF SEBACEOUS ADENOMA/SEBACEOMA

It presents clinically as solitary or multiple yellowish papules or nodules, usually less than a centimeter in diameter, distributed on the head or neck and sometimes on the upper trunk.

Superficial sebaceous adenomas are usually relatively small and papular, while sebaceomas may present as a deep nodule.

Multiple sebaceous neoplasms, especially cystic ones, may serve as a presenting sign of the MUIR–TORRE SYNDROME.

SEBACEOUS ADENOMAS ON THE FACE IN A

PATIENT WITH MUIR-TORRE SYNDROME

A, EXTERNAL APPEARANCE OF THE LEFT EYE. NOTE A YELLOWISH-PINK WARTY

GROWTH ARISING FROM THE ANTERIOR LAMELLA OF THE LEFT UPPER EYELID. B, MULTIPLE YELLOW NODULAR LESIONS INVOLVING THE CENTRAL FOREHEAD,

NOSE, AND ADJACENT CHEEK AREA (ARROW).

MUIR-TORRE SYNDROME Is a rare inherited autosomal dominant condition in which there are

sebaceous skin neoplasms in association with internal cancer. The most common organ involved is the gastrointestinal tract, with

almost one half of patients having colonic adenocarcinoma. The second most common site is cancer of the genitourinary tract.

The sebaceous neoplasms include:

1. Sebaceous adenomas

2. Sebaceous epitheliomas

3. Sebaceous carcinomas Other skin lesions that may arise in affected family members

include:

1. Multiple Keratoacanthoma

2. Squamous cell carcinoma

3. Multiple follicular cysts

HISTO. OF SEBACEOUS ADENOMA/SEBACEOMA

SEBACEOUS ADENOMA is usually superficial containing mature sebocytic cells and basaloid seboblastic cells but sebocytes usually predominate. Seboblasts comprise a thin multilayer of cells at the periphery of each lobule that may show mitotic figures.

SEBACEOMA is usually more deeply situated, often involving the deep reticular dermis and sometimes the superficialhypodermis. The lesions are lobulated sharply circumscribedand seboblasts predominate and sebocytic differentiation may be only focal and remain the minor proportion.

SEBACEOUS ADENOMAIT SHOWS MATURE SEBOCYTIC CELLS

SURROUNDED BY A RIM OF BASALOID

SEBOBLASTIC CELLS.

SEBACEOMATHIS HIGH-MAGNIFICATION VIEW SHOWS A

BACKGROUND OF BASALOID SEBOBLASTIC CELLS

PUNCTUATED BY SCATTERED MATURE SEBOCYTES

WITH COARSELY VACUOLATED CYTOPLASM. THE

NEOPLASM WAS LARGE BUT SHARPLY

CIRCUMSCRIBED AT LOW MAGNIFICATION.

SEBACEOMAFREQUENT MITOSES CAN BE SEEN IN THIS

TUMOR, THOUGH CYTOLOGICAL ATYPIA IS

LACKING

Rx OF SEBACEOUS ADENOMA/SEBACEOMA

Complete surgical excision of the lesion is recommended to

exclude the possibility of BCC with sebaceous differentiation or

sebaceous carcinoma.

NEOPLASMS AND PROLIFERATIONS WITH APOCRINE AND ECCRINE DIFFERENTIATION

NEOPLASMS AND PROLIFERATIONS WITH APOCRINE AND ECCRINE DIFFERENTIATION It is difficult to determine whether sweat gland tumors are of eccrine or

apocrine differentiation. The precise diagnosis of sweat gland lesions is most likely to be made histologically rather than clinically.

Benign Neoplasms and Proliferations may be of both apocrine or eccrine Differentiation

1. Syringoma

2. Poroma

3. Hidradenoma

4. Adnexal adenocarcinoma

NEOPLASMS AND PROLIFERATIONS WITH APOCRINE DIFFERENTIATION

1. Apocrine adenoma

2. Spiradenoma

3. Cylindroma

4. Apocrine adenocarcinomas

NEOPLASMS AND PROLIFERATIONS WITH ECCRINE DIFFERENTIATION

1. Eccrine nevus (hamartoma)

2. Syringofibroadenoma

3. Papillary adenoma

4. Papillary adenocarcinoma

SYRINGOMA

SYRINGOMA

Syringoma represents a benign adnexal

neoplasm with mostly ductal (syringeal)

differentiation usually found on the upper

cheeks and lower eyelids of young adults.

SYRINGOMA

C/P OF SYRINGOMA Asymptomatic small, firm, skin-colored to yellowish papule

commonly multiple and symmetric may be eruptive. They first appear during adolescence and further increase

during adult life and remain indefinitely. Prone to occur in the periorbital area, especially the lower

eyelids sometimes, lesions involve the upper trunk, favoring the ventral surface, or genital skin.

The lesions are removed from women more commonly than men.

There are an association with Down syndrome. Clear cellsyringomas are associated with diabetes mellitus.

SYRINGOMA

COBBLESTONE APPEARANCE OF THE

SKIN UNDER THE EYES TYPICAL OF

MULTIPLE SYRINGOMAS.

SYRINGOMAS ON THE NECK MULTIPLE SKIN-COLORED, FIRM, SMALL

LESIONS. THESE BENIGN LESIONS TYPICALLY

APPEAR NEAR THE EYELIDS, BUT THEY CAN

OCCUR LOWER ON THE FACE.

SYRINGOMAS

C/P OF SYRINGOMA

ERUPTIVE SYRINGOMAS which is less common

appear as a crop of multiple lesions typically on the

chest, neck or lower abdomen but may involve the

extremities, including the palms and soles.

SYRINGOMA

SYRINGOMASMULTIPLE SKIN-COLORED TO PINK, SMOOTH PAPULES

ON THE NECK AND UPPER CHEST. THE LESIONS FAVOR

THE VENTRAL SURFACE OF THE TRUNK.

ERUPTIVE SYRINGOMA

ERUPTIVE SYRINGOMAS

SYRINGOMAS IN THE GENITAL AREAS

HISTO. OF SYRINGOMA The lesion is circumscribed and is usually confined to the

superficial dermis. Epithelial cells with dark stained nuclei and pale eosinophilic

cytoplasm forming nests and tubules with surrounding scleroticstroma.

Depending upon the exact plane of section, the nests of a syringoma vary in shape, and some nests may assume a characteristic morphology that resembles a comma or a tadpole.

Tubular areas exhibit ductular differentiation with central luminalined by a compact eosinophilic cuticle.

Usually syringoma lacks keratinization or follicular differentiation.

HISTOPATHOLOGY OF SYRINGOMA

DUCTULAR STRUCTURES (1) AND MORE

SOLID APPEARING NESTS AND STRANDS

(2) THAT ARE PRESENT WITHIN THE

DERMIS.

SYRINGOMATHIS SUPERFICIAL ADNEXAL NEOPLASM

CONSISTS OF NESTS OF CELLS WITH PALE

CYTOPLASM POSITIONED WITHIN SCLEROTIC

STROMA. MANY NESTS SHOW CENTRAL

DUCTAL DIFFERENTIATION WITH A COMPACT

EOSINOPHILIC CUTICLE.

HISTOLOGY OF SYRINGOMA

HISTOLOGY OF SYRINGOMA

SHOWING TYPICAL "TADPOLE/ COMMA

SHAPED" EPITHELIAL TUMOR STRAND

Rx OF SYRINGOMA As it is a benign neoplasm and in most cases are left alone.

If they become cosmetically disturbing, destructivetreatment options may be employed.

Although there is a risk of scarring treatment options include;

1. Trichloroacetic acid

2. Cryotherapy

3. Punch excision

4. Electrosurgical destruction

5. Dermabrasion

6. Laser ablation especially in disseminated lesions

TREATMENT OF SYRINGOMA

SYRINGOMA BEFORE ELECTROSURGERY SYRINGOMA AFTER ELECTROSURGERY

POROMA

POROMA

Poroma represents a group of benign adnexal

neoplasms with poroid differentiation (derived

from cells of the terminal sweat duct and

connected to the epidermis).

Poroma can be a proliferation of either

apocrine or eccrine lineage

POROMA

C/P OF POROMA Poromas usually present as solitary skin-colored to reddish

papules, plaques or nodules and may resemble pyogenic granuloma but may also appear as a pigmented lesion.

Most lesions are asymptomatic, but minor pain can accompany some lesions

They can appear on any cutaneous surface but those with eccrine differentiation are most commonly found on the palms, soles and scalp (with or without an associated nevussebaceus).

When sessile vascular plaques surrounded by thin groove appear on the palms and soles.

POROMATOSIS: Rarely, multiple poromas develop, either in an acral or in a widespread distribution.

HISTO. OF POROMA Circumscribed proliferation of compact cuboidal “poroid” cells with

small monomorphous nuclei and scanty eosinophilic cytoplasm. The narrow ducts are often lined by a row of cuboidal cells. According to extension it may be;

1. Intra-epidermal poroma: nests of cells with tubular differentiation are confined to the surface epidermis.

2. Juxta-epidermal poroma: In continuity with epidermis but also involve the superficial dermis.

3. Intra-dermal poroma: confined wholly within the dermis.

HISTOCHEMISTRY: shows positive carcinoembryonic antigen (CEA) immunostaining labels the luminal surface of both apocrine and eccrine ducts.

OUTLINES OF THE 3 VARIANTS OF POROMA

INTRAEPIDERMAL POROMA JUXTA-EPIDERMAL POROMA INTRADERMAL POROMA

JUXTAEPIDERMALPOROMA

PRESENTS HISTOPATHOLOGICALLY IN

CONTINUITY WITH THE EPIDERMIS,

CREATING A SEBORRHEIC KERATOSIS-

LIKE PROFILE.

POROMA, JUXTA-EPIDERMAL PATTERN

THIS POROMA DISPLAYS A PATTERN COMMON TO BOTH

ECCRINE AND APOCRINE POROMA.

THERE ARE INTER-ANASTOMOSING CORDS COMPOSED

OF SMALL COMPACT “POROID” CELLS, AND THE

INTERVENING STROMA IS HIGHLY VASCULARIZED AND

RESEMBLES GRANULATION TISSUE.

INTRADERMAL POROMA SHOWS A SHARPLY CIRCUMSCRIBED BORDER

AT SCANNING MAGNIFICATION. HIGHLY

VASCULARIZED AND FOCALLY SCLEROTIC

STROMA IS ALSO EVIDENT.

INTRADERMAL POROMA AT HIGHER MAGNIFICATION, DUCTAL

DIFFERENTIATION IS CONSPICUOUS.

Rx OF POROMA

Poroma is a benign adnexal neoplasm and thus treatment is

optional.

Superficial lesions may be treated by shave or electrosurgical

destruction.

Superficial or deeper lesions may also be treated with simple

surgical excision.

REFERENCES

Bolognia 3rd Ed.

http://dermnetnz.org

Google Images

globalskinatlas.com

adnexal neoplasms

Health & Medicine