ABC of Sweat Gland TumorsABC of Sweat Gland Tumors

Deba P Sarma, MDDeba P Sarma, MD

Lakeside Hospital, Omaha, NELakeside Hospital, Omaha, NE

Sweat gland anatomySweat gland anatomy

Duct

Gland

Duct: Pore Acrosyringium Syringium Straight part Cylinder Spiral part

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 papilliferum SyringomaSyringoma HidrocystomaHidrocystoma PoromaPoroma CylindromaCylindroma SpiradenomaSpiradenoma

Glandular:Glandular: HidradenomaHidradenoma

Malignant:Malignant: AdenocarcinomaAdenocarcinoma

Syringocystadenoma papilliferum

Epidermis shows acanthosis and papillomatosis. Cystic invaginations with papillary projections extend downward from the epidermis. The papillary projections are lined by two layers of cuboidal and columnar epithelial cells. The stroma is infiltrated by a numerous plasma cells.

Syringocystadenoma papilliferumSyringocystadenoma papilliferum

Syringocystadenoma papilliferum (SP) is a benign Syringocystadenoma papilliferum (SP) is a benign adnexal tumor, most commonly located on the scalp adnexal tumor, most commonly located on the scalp or face, which frequently arises from a nevus or face, which frequently arises from a nevus sebaceus (NS).sebaceus (NS).

Epidermis shows acanthosis and papillomatosis. Cystic invaginations with papillary projections extend downward from the epidermis. The papillary projections are lined by two layers of cuboidal and columnar epithelial cells. Luminal cells may show decapitation secretion.The stroma is infiltrated by a numerous plasma cells.Malformed sebaceous glands and hair structures may be present.

Syringoma

Epidermis is normal. Upper dermis shows numerous small epithelial ducts embedded in sclerotic stroma. The walls of the ducts are lined by two layers of cuboidal or flat epithelial cells. Ductal lumen contains eosinophilic, amorphous debris. Some ducts have elongated tails of epithelial cells that produce a comma-shaped or tadpole appearance.

Syringoma is a benign adnexal neoplasm formed by Syringoma is a benign adnexal neoplasm formed by well-differentiated ductal elements of sweat gland.well-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) generalized associated with Down syndrome, (3) generalized multiple 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 1-3 appearing at puberty as symmetrical multiple 1-3 mm clustered lesions in the upper cheeks and lower mm clustered lesions in the upper cheeks and lower eyelids.eyelids.

Other sites include axilla, chest, abdomen, genital Other sites include axilla, chest, abdomen, genital skin.skin.

Eruptive syringomas are more common in African Eruptive syringomas are more common in African Americans and Asians.Americans and Asians.

Pathology: Epidermis is normal. Upper dermis shows Pathology: Epidermis is normal. Upper dermis shows numerous small epithelial ducts embedded in numerous small epithelial ducts embedded in sclerotic stroma. The walls of the ducts are lined by sclerotic stroma. The walls of the ducts are lined by two layers of cuboidal or flat epithelial cells. Ductal two layers of cuboidal or flat epithelial cells. Ductal lumen contains eosinophilic, amorphous debris. Some lumen contains eosinophilic, amorphous debris. Some ducts have elongated tails of epithelial cells that ducts have elongated tails of epithelial cells that produce a comma-shaped or tadpole appearance. produce a comma-shaped or tadpole appearance. Keratinous cysts are commonly seen in the Keratinous cysts are commonly seen in the subepidermal location.subepidermal location.

Tumor does not extend into subcutis. Tumor does not extend into subcutis.

Syringoma

Poroma

Poroma shows intraepidermal nests of small monotonous Poroma shows intraepidermal nests of small monotonous polygonal cells with low mitotic activity. The tumor cells polygonal cells with low mitotic activity. The tumor cells generally demonstrate direct downward growth into the dermis generally demonstrate direct downward growth into the dermis as interconnected basaloid proliferations. The intraepidermal as interconnected basaloid proliferations. The intraepidermal nests of basaloid cells are smaller than the adjacent nests of basaloid cells are smaller than the adjacent keratinocytes and show intercellular bridges. There are foci of keratinocytes and show intercellular bridges. There are foci of maturation towards ducts characterized by lumen formation 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 Poroma is a benign adnexal tumor arising from sweat gland (eccrine and apocrine) duct.sweat gland (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 C. Poroma (Compound poroma), the most common typetype

HistologyHistology

Poroma shows intraepidermal nests of small Poroma shows intraepidermal nests of small monotonous polygonal cells with low mitotic activity. monotonous polygonal cells with low mitotic activity.

The tumor cells generally demonstrate direct The tumor cells generally demonstrate direct downward growth into the dermis as interconnected downward growth into the dermis as interconnected basaloid proliferations. basaloid proliferations.

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

There are foci of maturation towards ducts There are foci of maturation towards ducts characterized by lumen formation surrounded by characterized by lumen formation surrounded by eosinophilic material over small epithelial cells.eosinophilic material over 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 fluid, not keratin. Eccrine or apocrine type epithelial cells may suggest the origin.

Hidrocystoma

Cylindroma

Lobules of epithelial cells arranged in a jigsaw or mosaic Lobules of epithelial cells arranged in a jigsaw or mosaic pattern. Prominent red basement membrane-like structure pattern. Prominent red basement membrane-like structure encircles the tumor lobules. Each lobule shows a peripheral encircles the tumor lobules. Each lobule shows a peripheral lining by dark basaloid cells and an inner larger and paler zone lining by dark basaloid cells and an inner larger and paler zone of cells. of cells.

CylindromaCylindroma

Clinical: Sex: mostly female. Location: mostly scalp. Clinical: Sex: mostly female. Location: mostly scalp. Slow-growing, sometimes painful solitary pink or red dermal Slow-growing, sometimes painful solitary pink or red dermal

nodule averaging 1 cm in size. Familial cases are associated nodule averaging 1 cm in size. Familial cases are associated with multiple tumors. Such cases may also be associated with with multiple tumors. Such cases may also be associated with facial trichoepitheliomas, and eccrine spiradenomas, called facial trichoepitheliomas, and eccrine spiradenomas, called autosomal dominant Brooke-Spiegler syndrome (familial autosomal dominant Brooke-Spiegler syndrome (familial cylindromatosis or turban tumor syndrome).cylindromatosis or 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’. -Non-encapsulated dermal tumor not connected to the -Non-encapsulated dermal tumor not connected to the

overlying epidermis. overlying epidermis. -Composed of numerous lobules of epithelial cells arranged in -Composed of numerous lobules of epithelial cells arranged in

a jigsaw or mosaic pattern. a jigsaw or mosaic pattern. -Prominent red basement membrane-like structure encircles -Prominent red basement membrane-like structure encircles

the tumor lobules. the tumor lobules. -Each lobule shows a peripheral lining by dark basaloid cells -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. -Nodular deposits of red material within the lobules as well as -Nodular deposits of red material within the lobules as well as

focal well-formed ducts.focal well-formed ducts. -This is a common adnexal tumor of eccrine origin. -This is a common adnexal tumor of eccrine origin.

Spiradenoma

Well-circumscribed or encapsulated dermal nodule composed of Well-circumscribed or encapsulated dermal nodule composed of small dark basaloid and large pale epithelial cells within a vascular small dark basaloid and large pale epithelial cells within a vascular stroma. Low-power view resembles a lymph node. Stroma contains stroma. Low-power view resembles a lymph node. Stroma contains appreciable number of lymphocytes. Cuboidal epithelial cells form appreciable number of lymphocytes. Cuboidal epithelial cells form compacted cords with occasional ductal lumen formation with compacted cords with occasional ductal lumen formation with eosinophilic cuticle.Hyalinized matrix around the epithelial cords eosinophilic cuticle.Hyalinized matrix around the epithelial cords may resemble that of cylindroma.may resemble that of cylindroma.

SpiradenomaSpiradenoma

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

of the body during 2of the body during 2ndnd to 4 to 4thth decade. decade. Multiple tumors may be part of Brooke-Spiegler Multiple tumors may be part of Brooke-Spiegler

syndrome.syndrome.

Pathology:Pathology: Well-circumscribed or encapsulated dermal nodule Well-circumscribed or encapsulated dermal nodule

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

Low-power view resembles a lymph node.Low-power view resembles a lymph node. Stroma contains appreciable number of lymphocytes.Stroma contains appreciable number of lymphocytes. Cuboidal epithelial cells form compacted cords with Cuboidal epithelial cells form compacted cords with

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

Hyalinized matrix around the epithelial cords may Hyalinized matrix around the epithelial cords may resemble that of cylindroma.resemble that of cylindroma.

Hidradenoma

Well circumscribed, un-encapsulated solid and cystic lobular Well circumscribed, un-encapsulated solid and cystic lobular dermal tumor, 50% connected to the epidermis. Biphasic dermal tumor, 50% connected to the epidermis. Biphasic cellular pattern: areas of round, fusiform, polygonal squamoid cellular pattern: areas of round, fusiform, polygonal squamoid cells with eosinophilic cytoplasm and cells with clear cytoplasm. cells with eosinophilic cytoplasm and cells with clear cytoplasm. Duct-like structures, cystic change, focal apocrine change, Duct-like structures, cystic change, focal apocrine change, squamous eddies, goblet cells etc may be present.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, slow-growing solid or cystic dermal nodule, 1-2 Solitary, slow-growing solid or cystic dermal nodule, 1-2

cm.cm.

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

Biphasic cellular pattern: areas of round, fusiform, Biphasic cellular pattern: areas of round, fusiform, polygonal squamoid cells with eosinophilic cytoplasm polygonal squamoid cells with eosinophilic cytoplasm and cells with clear cytoplasm.and cells with clear cytoplasm.

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

Stroma is fibrovascular, collagenous or hyalinized.Stroma is fibrovascular, collagenous or hyalinized. Tumor ‘budding’ from the periphery to the surrounding Tumor ‘budding’ from the periphery to the surrounding

dermis should be considered as a low-grade malignant dermis should be considered as a low-grade malignant tumor.tumor.

Diffuse nuclear anaplasia, necrosis and tumor giant cells Diffuse nuclear anaplasia, necrosis and tumor giant cells maymay

suggest malignancy.suggest malignancy.

Malignant:Malignant:Eccrine carcinomaEccrine carcinoma

(Syringomatous carcinoma, porocarcinoma, (Syringomatous carcinoma, porocarcinoma, mucinous carcinoma, papillary carcinoma, mucinous carcinoma, papillary carcinoma, mucoepidermoid carcinoma, microcystic adnexal mucoepidermoid carcinoma, microcystic adnexal carcinoma etc)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 , features suggestive of (poroma, syringoma, cyindroma, suggestive of (poroma, syringoma, cyindroma, hidradenoma, chondroid syringoma, etc)hidradenoma, chondroid syringoma, etc)

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