adnexal tumors sweat gland origin皮肤附属器肿瘤汗腺起源课件

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 Syringoma Syringoma Hidrocystoma Hidrocystoma Poroma Poroma Cylindroma Cylindroma Spiradenoma Spiradenoma 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 n n Syringocystadenoma papilliferum (SP) is a benign adnexal Syringocystadenoma papilliferum (SP) is a benign adnexal tumor, most commonly located on the scalp or face, which tumor, most commonly located on the scalp or face, which frequently arises from a nevus sebaceus (NS).frequently arises from a nevus 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. n n Syringoma is a benign adnexal neoplasm formed by wellSyringoma is a benign adnexal neoplasm formed by well -differentiated ductal elements of sweat gland.-differentiated ductal elements of sweat gland. n n 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. n n Syringomas are common lesions, mostly in female, Syringomas are common lesions, mostly in female, appearing at puberty as symmetrical multiple 1-3 mm appearing at puberty as symmetrical multiple 1-3 mm clustered lesions in the upper cheeks and lower eyelids.clustered lesions in the upper cheeks and lower eyelids. n n Other sites include axilla, chest, abdomen, genital skin.Other sites include axilla, chest, abdomen, genital skin. n n Eruptive syringomas are more common in African Eruptive syringomas are more common in African Americans and Asians.Americans and Asians. n n Pathology: Epidermis is normal. Upper dermis shows Pathology: Epidermis is normal. Upper dermis shows numerous small epithelial ducts embedded in sclerotic numerous small epithelial ducts embedded in sclerotic stroma. The walls of the ducts are lined by two layers of stroma. The walls of the ducts are lined by two layers of cuboidal or flat epithelial cells. Ductal lumen contains cuboidal or flat epithelial cells. Ductal lumen contains eosinophilic, amorphous debris. Some ducts have eosinophilic, amorphous debris. Some ducts have elongated tails of epithelial cells that produce a comma-elongated tails of epithelial cells that produce a comma- shaped or tadpole appearance. Keratinous cysts are shaped or tadpole appearance. Keratinous cysts are commonly seen in the subepidermal monly 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 polygonal Poroma shows intraepidermal nests of small monotonous polygonal cells with low mitotic activity. The tumor cells generally demonstrate cells with low mitotic activity. The tumor cells generally demonstrate direct downward growth into the dermis as interconnected basaloid direct downward growth into the dermis as interconnected basaloid proliferations. The intraepidermal nests of basaloid cells are smaller proliferations. The intraepidermal nests of basaloid cells are smaller than the adjacent keratinocytes and show intercellular bridges. There than the adjacent keratinocytes and show intercellular bridges. There are foci of maturation towards ducts characterized by lumen formation are 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 n n Poroma is a benign adnexal tumor arising from sweat gland Poroma is a benign adnexal tumor arising from sweat gland (eccrine and apocrine) duct.(eccrine and apocrine) duct. n n Location: Mostly foot and hand.Location: Mostly foot and hand. n n May be painful.May be painful. n n 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 type C. Poroma (Compound poroma), the most common type HistologyHistology n n Poroma shows intraepidermal nests of small monotonous Poroma shows intraepidermal nests of small monotonous polygonal cells with low mitotic activity. polygonal cells with low mitotic activity. n n The tumor cells generally demonstrate direct downward The tumor cells generally demonstrate direct downward growth into the dermis as interconnected basaloid growth into the dermis as interconnected basaloid proliferations. proliferations. n n The intraepidermal nests of basaloid cells are smaller than The intraepidermal nests of basaloid cells are smaller than the adjacent keratinocytes and show intercellular bridges. the adjacent keratinocytes and show intercellular bridges. n n There are foci of maturation towards ducts characterized by There are foci of maturation towards ducts characterized by lumen formation surrounded by eosinophilic material over lumen formation surrounded by eosinophilic material over small epithelial cells.small epithelial cells. n n 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 pattern. Lobules of epithelial cells arranged in a jigsaw or mosaic pattern. Prominent red basement membrane-like structure encircles the tumor Prominent red basement membrane-like structure encircles the tumor lobules. Each lobule shows a peripheral lining by dark basaloid cells and lobules. Each lobule shows a peripheral lining by dark basaloid cells and an inner larger and paler zone of cells. an inner larger and paler zone of cells. Cylindroma Cylindroma Clinical: Sex: mostly female. Location: mostly scalp. Clinical: Sex: mostly female. Location: mostly scalp. Slow-growing, sometimes painful solitary pink or red dermal nodule Slow-growing, sometimes painful solitary pink or red dermal nodule averaging 1 cm in size. Familial cases are associated with multiple averaging 1 cm in size. Familial cases are associated with multiple tumors. Such cases may also be associated with facial tumors. Such cases may also be associated with facial trichoepitheliomas, and eccrine spiradenomas, called autosomal trichoepitheliomas, and eccrine spiradenomas, called autosomal dominant Brooke-Spiegler syndrome (familial cylindromatosis or dominant Brooke-Spiegler syndrome (familial cylindromatosis or turban 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. -Non-encapsulated dermal tumor not connected to the overlying -Non-encapsulated dermal tumor not connected to the overlying epidermis. epidermis. -Composed of numerous lobules of epithelial cells arranged in a jigsaw -Composed of numerous lobules of epithelial cells arranged in a jigsaw or mosaic pattern. or mosaic pattern. -Prominent red basement membrane-like structure encircles the tumor -Prominent red basement membrane-like structure encircles the tumor lobules. lobules. -Each lobule shows a peripheral lining by dark basaloid cells and an -Each lobule shows a peripheral lining by dark basaloid cells and an inner larger and paler zone of cells. inner larger and paler zone of cells. -Nodular deposits of red material within the lobules as well as focal -Nodular deposits of red material within the lobules as well as focal well-formed ducts.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 small dark Well-circumscribed or encapsulated dermal nodule composed of small dark basaloid and large pale epithelial cells within a vascular stroma. Low-power basaloid and large pale epithelial cells within a vascular stroma. Low-power view resembles a lymph node. Stroma contains appreciable number of view resembles a lymph node. Stroma contains appreciable number of lymphocytes. Cuboidal epithelial cells form compacted cords with lymphocytes. Cuboidal epithelial cells form compacted cords with occasional ductal lumen formation with eosinophilic cuticle.Hyalinized occasional ductal lumen formation with eosinophilic cuticle.Hyalinized matrix around the epithelial cords may resemble that of cylindroma.matrix around the epithelial cords may resemble that of cylindroma. SpiradenomaSpiradenoma n n Clinical:Clinical: n n Painful, solitary dermal tumor in the skin of upper half of Painful, solitary dermal tumor in the skin of upper half of the body during 2the body during 2nd nd to 4 to 4th th decade. decade. n n Multiple tumors may be part of Brooke-Spiegler syndrome.Multiple tumors may be part of Brooke-Spiegler syndrome. n n Pathology:Pathology: n n Well-circumscribed or encapsulated dermal nodule Well-circumscribed or encapsulated dermal nodule composed of small dark basaloid and large pale epithelial composed of small dark basaloid and large pale epithelial cells within a vascular stroma.cells within a vascular stroma. n n Low-power view resembles a lymph node.Low-power view resembles a lymph node. n n Stroma contains appreciable number of lymphocytes.Stroma contains appreciable number of lymphocytes. n n Cuboidal epithelial cells form compacted cords with Cuboidal epithelial cells form compacted cords with occasional ductal lumen formation with eosinophilic cuticle.occasional ductal lumen formation with eosinophilic cuticle. n n Hyalinized matrix around the epithelial cords may resemble Hyalinized matrix around the epithelial cords may resemble that of cylindroma.that of cylindroma. Hidradenoma Well circumscribed, un-encapsulated solid and cystic lobular dermal Well circumscribed, un-encapsulated solid and cystic lobular dermal tumor, 50% connected to the epidermis. Biphasic cellular pattern: areas tumor, 50% connected to the epidermis. Biphasic cellular pattern: areas of round, fusiform, polygonal squamoid cells with eosinophilic cytoplasm of round, fusiform, polygonal squamoid cells with eosinophilic cytoplasm and cells with clear cytoplasm. Duct-like structures, cystic change, focal and cells with clear cytoplasm. Duct-like 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. n n Middle age and elderly, FM.Middle age and elderly, FM. n n Solitary, slow-growing solid or cystic dermal nodule, 1-2 cm.Solitary, slow-growing solid or cystic dermal nodule, 1-2 cm. n n Well circumscribed, un-encapsulated solid and cystic lobular Well circumscribed, un-encapsulated solid and cystic lobular dermal tumor, 50% connected to the epidermis.dermal tumor, 50% connected to the epidermis. n n Biphasic cellular pattern: areas of round, fusiform, polygonal Biphasic cellular pattern: areas of round, fusiform, polygonal squamoid cells with eosinophilic cytoplasm and cells with clear squamoid cells with eosinophilic cytoplasm and cells with clear cytoplasm.cytoplasm. n n 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. n n Stroma is fibrovascular, collagenous or hyalinized.Stroma is