apocrine hidrocystomas
TRANSCRIPT
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Grand Rounds
Michael Rubin, MD
Department of Ophthalmology and
Visual ScienceThe University of Chicago
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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.
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Medical History
• PMH: BPH, Borderline HTN
• MEDS: Finestride
• ALL: NKDA• Ocular Hx: LUL lesion
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Visual Examination
• BCVA: OD: 20/30 OS: 20/30
• IOP: 16, 14
• CVF: Full OU• Pupils: 4mm to 2mm, No APD
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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
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LUL Lesion
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Diagnosis
• Apocrine Hidrocystoma
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Benign Adnexal Tumors
1. Holocrine Tumors
-- Glands of Zeis, Meibomian Glands
2. Eccrine Tumors3. Apocrine Tumors
--Sweat glands of Moll
4. Hair Follicle Tumors
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Holocrine/Sebaceous Tumors
Benign Lesions of Sebaceous Origin
--Milia
--Acquired Sebaceous Gland Hyperplasia--Sebaceous Adenoma
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Benign Lessions of Eccrine Origin
• 1. Eccrine Hidrocystoma
• 2. Syringoma
•3. Acrospiroma
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Benign Lesions of Apocrine Origin
1. Apocrine Hidrocystoma
2. Cylindroma
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Benign Lesions of Hair Follicle
• 1. Trichoepithelioma
• 2. Trichofolliculoma
•3. Tricholemmoma
• 4. Pilomatrixoma
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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.
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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.
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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.
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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.
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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).
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Causes
• Although the origin of apocrine
hidrocystomas is not known entirely, they
are believed to be adenomatous cystic
proliferations of the apocrine glands.
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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.
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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.
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Slide One
• Scan power view
showing multiple
cystic areas. The fluid
does not containpigment granules
though the fluid was
dark.
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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.
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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.
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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.
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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.
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Prognosis
• Apocrine hidrocystomas grow gradually
and persist indefinitely after attaining full
size. They seldom recur after removal.
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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.
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What’s This?
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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.
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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.
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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.
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Que es eso?
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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
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Histology of Eccrine Hydrocystoma
• Eccrine
Hydrocysomas are
dermal cysts lined by
double layeredcuboidal epithelium.
• Cyst in usually
translucent and fluid
filled.
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What the…
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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.
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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).
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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.
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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.
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Lesion 1: Trichoepithelioma
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Lesion 2: Trichofolliculoma
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Image 3: Trichilemmoma
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Image 4: Pilomatrixoma
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Two Lesions, One disease
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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.
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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
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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.
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Resident Quiz
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1. What is this?
2 What sydrome predisposing to thyroid and
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2. What sydrome predisposing to thyroid and
breast CA is associated with this lesion? What is
this lesion?
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3. Describe this Slide…
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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