cutaneous adnexal tumors of sweat gland origin

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ABC of Sweat Gland Tumors ABC of Sweat Gland Tumors

Deba P Sarma, MDDeba P Sarma, MD

CUMCCUMC

Jan 25, 2011Jan 25, 2011



Sweat gland anatomySweat gland anatomy

Duct

Gland

Duct:Pore

Acrosyringium

Syringium

Str aight par t

Cylinder

Spir al par t

Gland:

Coiled glands



Duct:

Pore: Opening through epidermis

Acrosyringium: Acro (end or top)+ syringium (tube or duct)

Syringium: Tube or duct

Straight (Cylinder) part of the duct

Spiral part of the duct

Gland: Coiled glands



Duct:

Pore: Poroma

Acrosyringium: Syringocystadenoma papilliferum

Syringium: Syringoma

Straight part of the duct: Cylindroma

Spiral part of the duct: Spiradenoma

Gland: Hidradenoma



Sweat gland tumorsSweat gland tumors

Benign: AdenomaBenign: Adenoma

DuctalDuctal

GlandularGlandular

MixedMixed

Malignant: AdenocarcinomaMalignant: Adenocarcinoma

DuctalDuctal

GlandularGlandular

MixedMixed



Sweat gland tumorsSweat gland tumors

Benign:Benign:

Ductal:Ductal:Syringocystadenoma papilliferumSyringocystadenoma papilliferumSyringomaSyringomaHidrocystomaHidrocystoma

PoromaPoromaCylindromaCylindromaSpiradenomaSpiradenoma

Glandular:Glandular:HidradenomaHidradenoma

Malignant:Malignant: Adenocarcinoma Adenocarcinoma



Syringocystadenoma papilliferum

Epidermis shows acanthosis and papillomatosis. Cysticinvaginations with papillary projections extend downward fromthe epidermis. The papillary projections are lined by two layersof cuboidal and columnar epithelial cells. The stroma isinfiltrated by a numerous plasma cells.



Syringocystadenoma papilliferumSyringocystadenoma papilliferum

Syringocystadenoma papilliferum (SP) is a benign adnexalSyringocystadenoma papilliferum (SP) is a benign adnexaltumor, most commonly located on the scalp or face, whichtumor, most commonly located on the scalp or face, whichfrequently arises from a nevus sebaceus (NS).frequently arises from a nevus sebaceus (NS).

Epidermis shows acanthosis and papillomatosis. Cystic

invaginations with papillar y pr ojections extend downward f r om the epidermis. The papillar y pr ojections are lined by two layer s of

cuboidal and columnar epithelial cells. Luminal cells may show

decapitation secretion.

The str oma is infiltrated by a numer ous plasma cells.

Malformed sebaceous glands and hair structures may be

present.



Syringoma is a benign adnexal neoplasm formed bySyringoma is a benign adnexal neoplasm formed bywellwell--differentiated ductal elements of sweat gland.differentiated ductal elements of sweat gland.

Four variants of syringoma : (1) localized form, (2)Four variants of syringoma : (1) localized form, (2)associated with Down syndrome, (3) generalizedassociated with Down syndrome, (3) generalizedmultiple and eruptive syringomas, and (4) familial.multiple and eruptive syringomas, and (4) familial.

Syringomas are common lesions, mostly in female,Syringomas are common lesions, mostly in female,appearing at puberty as symmetrical multiple 1appearing at puberty as symmetrical multiple 1--3 mm3 mmclustered lesions in the upper cheeks and lower eyelids.clustered lesions in the upper cheeks and lower eyelids.

Other sites include axilla, chest, abdomen, genital skin.Other sites include axilla, chest, abdomen, genital skin. Eruptive syringomas are more common in AfricanEruptive syringomas are more common in African Americans and Asians. Americans and Asians.

Pathology: Epidermis is normal. Upper dermis showsPathology: Epidermis is normal. Upper dermis showsnumerous small epithelial ducts embedded in scleroticnumerous small epithelial ducts embedded in scleroticstroma. The walls of the ducts are lined by two layers ofstroma. The walls of the ducts are lined by two layers ofcuboidal or flat epithelial cells. Ductal lumen containscuboidal or flat epithelial cells. Ductal lumen contains

eosinophilic, amorphous debris. Some ducts haveeosinophilic, amorphous debris. Some ducts haveelongated tails of epithelial cells that produce a commaelongated tails of epithelial cells that produce a comma--shaped or tadpole appearance. Keratinous cysts areshaped or tadpole appearance. Keratinous cysts arecommonly seen in the subepidermal location.commonly seen in the subepidermal location.Tumor does not extend into subcutis.Tumor does not extend into subcutis.

Syringoma



Poroma

Poroma shows intraepidermal nests of small monotonous polygonalPoroma shows intraepidermal nests of small monotonous polygonalcells with low mitotic activity. The tumor cells generally demonstratecells with low mitotic activity. The tumor cells generally demonstratedirect downward growth into the dermis as interconnected basaloiddirect downward growth into the dermis as interconnected basaloidproliferations. The intraepidermal nests of basaloid cells are smallerproliferations. The intraepidermal nests of basaloid cells are smallerthan the adjacent keratinocytes and show intercellular bridges. Therethan the adjacent keratinocytes and show intercellular bridges. Thereare foci of maturation towards ducts characterized by lumen formationare foci of maturation towards ducts characterized by lumen formation

surrounded by eosinophilic material over small epithelial cells.surrounded by eosinophilic material over small epithelial cells.



PoromaPoroma

Poroma is a benign adnexal tumor arising from sweat glandPoroma is a benign adnexal tumor arising from sweat gland(eccrine and apocrine) duct.(eccrine and apocrine) duct.

Location: Mostly foot and hand.Location: Mostly foot and hand.

May be painful.May be painful.

Poroma are three types:Poroma are three types:

A. Intraepidermal poroma (Hidroacanthoma simplex) A. Intraepidermal poroma (Hidroacanthoma simplex)B. Intradermal poroma (Dermal duct tumor)B. Intradermal poroma (Dermal duct tumor)

C. Poroma (Compound poroma), the most common typeC. Poroma (Compound poroma), the most common type



HistologyHistology

Poroma shows intraepidermal nests of small monotonousPoroma shows intraepidermal nests of small monotonouspolygonal cells with low mitotic activity.polygonal cells with low mitotic activity. The tumor cells generally demonstrate direct downwardThe tumor cells generally demonstrate direct downward

growth into the dermis as interconnected basaloidgrowth into the dermis as interconnected basaloidproliferations.proliferations.

The intraepidermal nests of basaloid cells are smaller thanThe intraepidermal nests of basaloid cells are smaller thanthe adjacent keratinocytes and show intercellular bridges.the adjacent keratinocytes and show intercellular bridges.

There are foci of maturation towards ducts characterized byThere are foci of maturation towards ducts characterized bylumen formation surrounded by eosinophilic material overlumen formation surrounded by eosinophilic material oversmall epithelial cells.small epithelial cells.

Differential diagnosis: Basal cell carcinoma,Differential diagnosis: Basal cell carcinoma,tricholemmoma, seborrheic keratosistricholemmoma, seborrheic keratosis



Mostly in the eyelids. Dermal cyst lined by cuboidal ductal epithelium

of sweat gland containing f luid, not keratin. Eccr ine or apocr ine type

epithelial cells may suggest the or igin.

Hidrocystoma



Cylindroma

Lobules of epithelial cells arranged in a jigsaw or mosaic pattern.Lobules of epithelial cells arranged in a jigsaw or mosaic pattern.Prominent red basement membraneProminent red basement membrane--like structure encircles the tumorlike structure encircles the tumorlobules. Each lobule shows a peripheral lining by dark basaloid cellslobules. Each lobule shows a peripheral lining by dark basaloid cells

and an inner larger and paler zone of cells.and an inner larger and paler zone of cells.



CylindromaCylindroma

Clinical: Sex: mostly female. Location: mostly scalp.Clinical: Sex: mostly female. Location: mostly scalp.SlowSlow--growing, sometimes painful solitary pink or red dermal nodulegrowing, sometimes painful solitary pink or red dermal noduleaveraging 1 cm in size. Familial cases are associated with multipleaveraging 1 cm in size. Familial cases are associated with multipletumors. Such cases may also be associated with facialtumors. Such cases may also be associated with facialtrichoepitheliomas, and eccrine spiradenomas, called autosomaltrichoepitheliomas, and eccrine spiradenomas, called autosomaldominant Brookedominant Brooke--Spiegler syndrome (familial cylindromatosis orSpiegler syndrome (familial cylindromatosis orturban tumor syndrome).turban tumor syndrome).

Pathologic features:Pathologic features:--Presence of numerous scalp lesions is called turban tumor.Presence of numerous scalp lesions is called turban tumor.--NonNon--encapsulated dermal tumor not connected to the overlyingencapsulated dermal tumor not connected to the overlyingepidermis.epidermis.--Composed of numerous lobules of epithelial cells arranged in a jigsawComposed of numerous lobules of epithelial cells arranged in a jigsawor mosaic pattern.or mosaic pattern.--Prominent red basement membraneProminent red basement membrane--like structure encircles the tumorlike structure encircles the tumorlobules.lobules.--Each lobule shows a peripheral lining by dark basaloid cells and anEach lobule shows a peripheral lining by dark basaloid cells and aninner larger and paler zone of cells.inner larger and paler zone of cells.--Nodular deposits of red material within the lobules as well as focalNodular deposits of red material within the lobules as well as focalwellwell--formed ducts.formed ducts.--This is a common adnexal tumor of eccrine origin.This is a common adnexal tumor of eccrine origin.



Spiradenoma

WellWell--circumscribed or encapsulated dermal nodule composed of small darkcircumscribed or encapsulated dermal nodule composed of small darkbasaloid and large pale epithelial cells within a vascular stroma. Lowbasaloid and large pale epithelial cells within a vascular stroma. Low--powerpowerview resembles a lymph node. Stroma contains appreciable number ofview resembles a lymph node. Stroma contains appreciable number oflymphocytes. Cuboidal epithelial cells form compacted cords withlymphocytes. Cuboidal epithelial cells form compacted cords with

occasional ductal lumen formation with eosinophilic cuticle.Hyalinizedoccasional ductal lumen formation with eosinophilic cuticle.Hyalinizedmatrix around the epithelial cords may resemble that of cylindroma.matrix around the epithelial cords may resemble that of cylindroma.



SpiradenomaSpiradenoma

Clinical:Clinical: Painful, solitary dermal tumor in the skin of upper half ofPainful, solitary dermal tumor in the skin of upper half of

the body during 2the body during 2ndnd to 4to 4thth decade.decade.

Multiple tumors may be part of BrookeMultiple tumors may be part of Brooke--Spiegler syndrome.Spiegler syndrome.

Pathology:Pathology: WellWell--circumscribed or encapsulated dermal nodulecircumscribed or encapsulated dermal nodule

composed of small dark basaloid and large pale epithelialcomposed of small dark basaloid and large pale epithelialcells within a vascular stroma.cells within a vascular stroma.

LowLow--power view resembles a lymph node.power view resembles a lymph node.

Stroma contains appreciable number of lymphocytes.Stroma contains appreciable number of lymphocytes. Cuboidal epithelial cells form compacted cords withCuboidal epithelial cells form compacted cords with

occasional ductal lumen formation with eosinophilic cuticle.occasional ductal lumen formation with eosinophilic cuticle.

Hyalinized matrix around the epithelial cords may resembleHyalinized matrix around the epithelial cords may resemblethat of cylindroma.that of cylindroma.



Hidradenoma

Well circumscribed, unWell circumscribed, un--encapsulated solid and cystic lobular dermalencapsulated solid and cystic lobular dermaltumor, 50% connected to the epidermis. Biphasic cellular pattern: areastumor, 50% connected to the epidermis. Biphasic cellular pattern: areasof round, fusiform, polygonal squamoid cells with eosinophilic cytoplasmof round, fusiform, polygonal squamoid cells with eosinophilic cytoplasmand cells with clear cytoplasm. Ductand cells with clear cytoplasm. Duct--like structures, cystic change, focallike structures, cystic change, focal

apocrine change, squamous eddies, goblet cells etc may be present.apocrine change, squamous eddies, goblet cells etc may be present.



HidradenomaHidradenoma

Location: mostly head and neck, limbs, or any site.Location: mostly head and neck, limbs, or any site. Middle age and elderly, F>M.Middle age and elderly, F>M. Solitary, slowSolitary, slow--growing solid or cystic dermal nodule, 1growing solid or cystic dermal nodule, 1--2 cm.2 cm.

Well circumscribed, unWell circumscribed, un--encapsulated solid and cystic lobularencapsulated solid and cystic lobulardermal tumor, 50% connected to the epidermis.dermal tumor, 50% connected to the epidermis.

Biphasic cellular pattern: areas of round, fusiform, polygonalBiphasic cellular pattern: areas of round, fusiform, polygonalsquamoid cells with eosinophilic cytoplasm and cells with clearsquamoid cells with eosinophilic cytoplasm and cells with clearcytoplasm.cytoplasm.

DuctDuct--like structures, cystic change, focal apocrine change,like structures, cystic change, focal apocrine change,squamous eddies, goblet cells etc may be present.squamous eddies, goblet cells etc may be present.

Stroma is fibrovascular, collagenous or hyalinized.Stroma is fibrovascular, collagenous or hyalinized. Tumor budding from the periphery to the surrounding dermisTumor budding from the periphery to the surrounding dermis

should be considered as a lowshould be considered as a low--grade malignant tumor.grade malignant tumor. Diffuse nuclear anaplasia, necrosis and tumor giant cells mayDiffuse nuclear anaplasia, necrosis and tumor giant cells may

suggest malignancy.suggest malignancy.



Malignant:Malignant:Eccrine carcinomaEccrine carcinoma

(Syringomatous carcinoma, porocarcinoma, mucinous(Syringomatous carcinoma, porocarcinoma, mucinouscarcinoma, papillary carcinoma, mucoepidermoidcarcinoma, papillary carcinoma, mucoepidermoidcarcinoma, microcystic adnexal carcinoma etc)carcinoma, microcystic adnexal carcinoma etc)

Very rare dermal infiltrating carcinoma. Very rare dermal infiltrating carcinoma.

Exclude metastatic carcinoma first.Exclude metastatic carcinoma first.



Acceptable Diagnosis: Acceptable Diagnosis:

A. A. Benign adnexal tumor of sweat gland originBenign adnexal tumor of sweat gland origin, features, featuressuggestive of (poroma, syringoma, cyindroma, hidradenoma,suggestive of (poroma, syringoma, cyindroma, hidradenoma,chondroid syringoma, etc)chondroid syringoma, etc)

B.B. Maignant adnexal tumor of sweat gland originMaignant adnexal tumor of sweat gland origin, features, featuressuggestive of (porocarcinoma, syringoadenocarcinoma,suggestive of (porocarcinoma, syringoadenocarcinoma,papillary eccrine adenocarcinoma etc)papillary eccrine adenocarcinoma etc)

cutaneous adnexal tumors of sweat gland origin

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