apocrine hidrocystomas

7/30/2019 Apocrine hidrocystomas

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Grand Rounds

Michael Rubin, MD

Department of Ophthalmology and

Visual ScienceThe University of Chicago

Case Presentation

• 55 year old male presents for general

ophthalmology evaluation. States that he

has no visual complaints, but that he has

had a lesion on this left eye for the past

few years that we wanted evaluated.

Medical History

• PMH: BPH, Borderline HTN

• MEDS: Finestride

• ALL: NKDA• Ocular Hx: LUL lesion

Visual Examination

• BCVA: OD: 20/30 OS: 20/30

• IOP: 16, 14

• CVF: Full OU• Pupils: 4mm to 2mm, No APD

Ocular Examination

• Adnexa: Right: MGD/BlephLeft: See Photo

• Sclera/Conj: Clear OU

• Cornea: Clear OU• AC: Deep and Quiet

• Iris: Normal OU

• Lens: 1 + NS and 1 + CC both eyes

LUL Lesion

Diagnosis

• Apocrine Hidrocystoma

Benign Adnexal Tumors

1. Holocrine Tumors

-- Glands of Zeis, Meibomian Glands

2. Eccrine Tumors3. Apocrine Tumors

--Sweat glands of Moll

4. Hair Follicle Tumors

Holocrine/Sebaceous Tumors

Benign Lesions of Sebaceous Origin

--Milia

--Acquired Sebaceous Gland Hyperplasia--Sebaceous Adenoma

Benign Lessions of Eccrine Origin

• 1. Eccrine Hidrocystoma

• 2. Syringoma

•3. Acrospiroma

Benign Lesions of Apocrine Origin

1. Apocrine Hidrocystoma

2. Cylindroma

Benign Lesions of Hair Follicle

• 1. Trichoepithelioma

• 2. Trichofolliculoma

•3. Tricholemmoma

• 4. Pilomatrixoma

Background

• Apocrine hidrocystomas are benign cystic

proliferations of the apocrine secretory glands.

• These cysts most commonly appear as solitary,

soft, dome-shaped, translucent papules or nodules and most frequently are located on the

eyelids, especially the inner canthus.

• Apocrine hidrocystomas grow slowly and usually

persist indefinitely.

Pathophysiology

• The exact stimulus for the development of

an apocrine hidrocystoma is unknown.

Plausible causes of the closely related

eccrine hidrocystoma include occlusion or blockage of the sweat duct apparatus,

which results in the retention of sweat and

a dilated cystic structure.

Predisposition

• Race: No known predilection exists for race or geographic region.

• Sex: No known gender predilection exists.

• Age: Cysts occur in adulthood, although in noparticular age group.

• History: Apocrine hidrocystomas usually areasymptomatic. No seasonal variation or familial

tendencies have been identified. Cysts tend toappear during adulthood, grow slowly, andpersist indefinitely.

Physical Characteristics

• The tumors usually occur as solitary translucent papulesor nodules. – Consistency is fluctuant cystic.

– Size varies from a few millimeters to approximately 1.5 cm.

– Tumors occasionally present as multiple lesions.• Cysts often appear tense and shiny; coloration varies

from flesh-colored to blue or black

• Tumors have a predilection for the eyelid, particularly theinner canthus. – Tumors may arise on other areas of the head, neck, and trunk.

– Tumors also have been reported to occur on the penis, in theaxillae, and in the anal region.

More Characteristics

• Walls, although translucent, are sufficiently thick thatthey seldom rupture spontaneously.

• When incised, the cyst collapses and a thin, clear,brownish or blackish fluid is released.

• Cysts are mobile with palpation and transilluminate.• The fluid color of an apocrine hidrocystoma does not

result from the presence of melanin or hemosiderin butmay result either from the Tyndall phenomenon or thepresence of lipofuscin pigment.

• Apocrine hidrocystomas are not affected by variation intemperature (unlike eccrine hidrocystomas).

Causes

• Although the origin of apocrine

hidrocystomas is not known entirely, they

are believed to be adenomatous cystic

proliferations of the apocrine glands.

Need for Histology?

• The clinical appearance of a pea-sized

cyst near the inner canthus of the eye,

which contains a thin clear or pigmented

fluid, suggests an apocrine hidrocystoma;however, histologic examination often is

required to establish a specific and

definitive diagnosis.

Histology

• On histologic exam, apocrine

hidrocystomas show large unilocular or

multilocular cystic spaces within the

dermis. Apocrine hidrocystomas are morelikely to be multilocular than the closely

related eccrine hidrocystoma.

Slide One

• Scan power view

showing multiple

cystic areas. The fluid

does not containpigment granules

though the fluid was

Slide Two

• 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. There is a

suggestion of pigmented

material in the apices of

other cells. Apocrinesnouts are prominent.

Cyst Wall

• The cyst wall is lined by apocrine-type secretory

epithelium.

• The innermost layer of the wall is composed of a

single (occasionally double) layer of cuboidal-tocolumnar-shaped cells. The nuclei of these cells

are positioned basally.

• The outer layer of cells composing the cyst wall

is formed by myoepithelial cells in which the long

axes run parallel to the cyst wall.

More Histology

• Well-organized fibrous tissue surrounds the cyst.Papillary projections extend from the secretory layer intothe cyst cavity, depicting decapitation secretion.

• The secretory cells are acid-Schiff –positive, and

occasionally contain pigment granules, which provide thebrown color of the cystic fluid. This pigment is neither melanin nor hemosiderin.

• On electron microscopy, secretory cells have numerous,dense, lysosomal-type secretory granules typical of

apocrine gland cells. They also have an increasednumber of annulate lamellae, which are unusual innormal apocrine cells.

Treatment

• Apocrine hidrocystomas can be incised

and drained.

• Electrosurgical destruction of the cyst wall

often is recommended to prevent

recurrence. Punch, scissors, or elliptical

excision also can remove tumors.

Prognosis

• Apocrine hidrocystomas grow gradually

and persist indefinitely after attaining full

size. They seldom recur after removal.

Pitfall

• If the diagnosis is made clinically without

biopsy, a cystic basal cell carcinoma of the

eyelid may be missed. Incision to observe

the egress of fluid or biopsy of suspiciouslesions excludes the possibility.

What’s This?

Syringoma

• Syringoma is a benign adnexal neoplasm

formed by well-differentiated ductal

elements.

• Its name is derived from the Greek word

syrinx, which means pipe or tube.

Appearance

Syringomas are skin-colored or yellowish,

small, dermal papules.

Sometimes, the lesions may appear

translucent or cystic.

The surface can be rounded or flat-topped.

The lesions are usually smaller than 3 mm

in diameter.

Pathophysiology

• Syringoma is a benign neoplasm that is

traditionally considered to differentiate

along eccrine lines.

• Many tumors that were traditionally

thought to be eccrine have recently been

shown to have apocrine differentiation.

Que es eso?

Eccrine Hidrocystoma

• AKA Sudoriferous or Sweat gland Cysts

• Solitary or multiple small nodules on the

eyelid

• Overlying skin is shiny and smooth

• Thought to be ductal retention cysts

•Tend to increase in size during hot andhumid weather

Histology of Eccrine Hydrocystoma

• Eccrine

Hydrocysomas are

dermal cysts lined by

double layeredcuboidal epithelium.

• Cyst in usually

translucent and fluid

filled.

What the…

Cylindroma

• The cellular origin of cylindromas is

debated by dermatopathologists.

• Cylindroma most likely is a very primitive

sweat gland tumor differentiating toward

either the eccrine line or the apocrine line.

Physical Characteristics of

Cylindroma

– Solitary lesions are firm, rubbery nodules with

pink, red, or sometimes blue coloring that

range in size from a few millimeters to several

centimeters. – The multiple form has numerous masses of

pink, red, or blue nodules, sometimes

resembling bunches of grapes or small

tomatoes (sometimes called a tomato tumor).

Histology of Cylindromas

• Note the focus of tubular differentiation. Tubulesare usually few in number or absent.

• Small subunits areseparated by glassy,eosinophilic basementmembrane material.Some of the subunits

have angulated outlines.The darker cells tend tobe found around theperiphery of the subunits.

Hair Follicle Lesions

1. Trichoepithelioma--Muliple, keratin-filled horn cysts

2. Trichofolliculoma

--Well differentiated hamartomatous lesion

--Solitary flesh-colored nodule with central umbilication

3. Trichilemmoma--Arises from outer hair sheath

--Flesh colored, nodular, or papillomatous

--Cowdens Disease: AD, Multiple lesions, acral keratosis, oralpapillomas, increased risk for thyroid and breast CA.

4. Pilomatrixoma

--Bening tumor of hair matrix origin

--Solid or cystic, mobile, subcutaneous nodule, frim irregular, oftenreddish blue.

Lesion 1: Trichoepithelioma

Lesion 2: Trichofolliculoma

Image 3: Trichilemmoma

Image 4: Pilomatrixoma

Two Lesions, One disease

Basal Cell Carcinoma

• Basal cell carcinoma (BCC) is the mostcommon malignancy in humans.

• It typically occurs in areas of chronic sun

exposure.• BCC is usually slow growing and rarely

metastasizes, but it can cause significant

local destruction and disfigurement if neglected or treated inadequately.

• Prognosis is excellent with proper therapy.

Features of Nodular BCC

• Nodular BCC: This is the most common variety of BCC.

• Nodular BCCs have some of the following features: – Waxy papule(s) with central depression

– Pearly appearance

– Erosion or ulceration – Bleeding

– Crusting

– Rolled (raised) border

– Translucency

– Telangiectases over the surface – History of bleeding with minor trauma

Other forms of BCC

• Pigmented BCC: In addition to features seen in lesions of nodular BCC, lesions of pigmented BCC contain increased brown or blackpigment and are seen more commonly in individuals with dark skin.

• Cystic BCC: Lesions of cystic BCC are translucent blue-gray cysticnodules that may mimic benign cystic lesions.

• Superficial BCC: This variety presents as scaly patches or papules

that are pink to red-brown in color, often with central clearing.Micronodular BCC: This aggressive BCC subtype has the typicalBCC distribution. It is less prone to ulceration, may appear yellow-white when stretched, and is firm to touch.

• Morpheaform and infiltrating BCC: These are aggressive BCCsubtypes with sclerotic (scarlike) plaques or papules. The border

usually is not well defined and often extends well beyond clinicalmargins.

Resident Quiz

1. What is this?

2 What sydrome predisposing to thyroid and

2. What sydrome predisposing to thyroid and

breast CA is associated with this lesion? What is

this lesion?

3. Describe this Slide…

Bibliography

• Alessi E, Gianotti R, Coggi A: Multiple apocrine hidrocystomas of the eyelids. Br J Dermatol 1997Oct; 137(4): 642-5

• Bures FA, Kotynek J: Differentiating between apocrine and eccrine hidrocystoma. Cutis 1982 Jun;29(6): 616, 619-20

• Combemale P, Kanitakis J, Dupin N: Multiple Moll's gland cysts (apocrine hidrocystomas) of theeyelids. Dermatology 1997; 194(2): 195-6

• de Eusebio E, Lopez-Bran E, Rojo S: Multiple hidrocystomas. Dermatology 1996; 193(2): 152-3

• Hashimoto K, Lever WF: Tumors of skin appendages. In: Freedberg IM, Eisen AZ, Wolff K, eds.

Fitzpatrick's Dermatology in General Medicine. Vol 1. McGraw-Hill Professional Publishing; 1999:899.

• Mackie RM: Tumors of skin appendages. In: Champion RH, Wilkinson DS, Ebling FJG, eds.Rook/Wilkinson/Ebling: Textbook of Dermatology. Vol 2. Blackwell Science Inc; 1998: 1703-6.

• Milum EA: A solitary pigmented tumor of the face. Apocrine hidrocystoma. Arch Dermatol 1991 Apr; 127(4): 572, 575

• Schleicher SM: Multiple translucent facial papules. Apocrine hidrocystoma. Arch Dermatol 1998Dec; 134(12): 1627-8, 1630-1

• Shields JA, Eagle RC Jr, Shields CL: Apocrine hidrocystoma of the eyelid. Arch Ophthalmol 1993Jun; 111(6): 866-7

• Smith JD, Chernosky ME: Apocrine hidrocystoma (cystademnoma). Arch Dermatol 1974 May;109(5): 700-2

• Ter Poorten HJ: Apocrine hidrocystoma of the right scapula. Arch Dermatol 1977 Dec; 113(12):1730

apocrine hidrocystomas

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