Contents
 

Adnexal tumors



6  Skin tumors

6.4  Adnexal tumors

Introduction:

Adnexal tumors are divided into tumors arising from the follicular epithelium, from sweat and sebaceous glands. These tumours are usually benign. Their clinical appearance is often not diagnostic.

6.4.1  Tumors of the hair follicle

Introduction:

Signs of follicular differentiation: hair matrix, hair papillae, hair germs

6.4.1.1  Hair follicle nevus

Clinical signs:

  • location: head, neck
  • area of hypertrichosis

Histology:

Group of normal vellus hairs.

6.4.1.2  Aberrant follicle

Sometimes hair follicle may appear on a skin, where normaly no hair follicles are present (palm).

Pictures

Aberrant follicle of the palm:
Aberrant follicle (palm, sole), HE 20x (4198)
  [zoomify]

6.4.1.3  Trichofolliculoma

Histology:

Cystic tumor, lined by squamous epithelium, usually communicating with the surface. Within the wall of the cyst there are small, malformed, well differentiated follicles with hair papilla. The tumor is surrounded by fibrous tissue rich in fibroblasts.

Variant of this tumor with many sebaceous glands present as well is called sebaceous trichofolliculoma.

6.4.1.4  Trichoadenoma

Clinical signs:

  • rare solitary tumor
  • location: face
  • size: up to 15 mm

Histology:

Groups of cysts, filled with keratin; granular layer is usually absent; epithelial strands connect the cysts; nests of keratinocytes.

6.4.1.5  Dilated pore of Winer

Clinical signs:

  • comedo-like benign tumor, rare
  • location: face

Histology:

Dilated open follicular cystic cavity filled with keratin with irregularly proliferating rete ridges. The lesion is sometimes pigmented.

6.4.1.6  Pilar sheath acanthoma

Clinical signs:

  • papule, usually on the upper lip
  • age: adults

Histology:

Dilated follicular infundibulum filled with keratin, acanthotic proliferation of the follicular epithelium; cells are regular, lesion is benign.

6.4.1.7  Trichoepithelioma, superficial trichoblastoma

Clinical signs:

  • usually solitary papules, location: face
  • multiple papules of the face in hereditary form, around the eyes, nasolabial folds
  • tiny pearly papules
  • color: skin color or yellowish

Pictures

Trichoepithelioma, face:
Trichoepithelioma, CLINIC (930)

Histology:

The tumor consists of basophilic epithelium with peripheral palisading. Mitotic activity is usually minimal. The slits between tumorous nests and the stroma, but rather between the stroma and uninvolved dermis. Focal differentiation into hair bulbs with stromal reaction (and differentiation into the papilla). The tumor doesn's usually reach depper than into 2/3 of the dermis (this differs from trichoblastoma).

6.4.1.8  Trichoblastoma

Histology:

Large tumor (reaching at least into half of the dermis) with variable structure (many types were described).

The tumor is characterized by proliferation of basaloid cells with focal differentiation into hair follicle, especially by formation of hair papilla (epithelial and mesenchymal parts). Mitotic activity is present, but the tumor is well demarcated, stroma is in contact with the epithelium (as opposed to bazalioma).

6.4.1.9  Desmoplastic trichoepithelioma

Clinical signs:

  • small, centrally umbilicated tumor
  • location: face

Histology:

Small nests and branching cords of squamous epithelium, small keratinous cysts, foci of keratinization, small calcifications and ossifications. Stroma is collagenous. No retraction arteracts are present.

EMA is positive (positive in microadnexal carcinoma, negative in basal cell carcinoma), stromelysin 3 is negative (positive in BCC and microadnexal carcinoma).

Pictures

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 40x (5480)

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 40x (4704)
  [zoomify]

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 20x (4230)

Desmoplastic trichoepithelioma, HE 20x (4231)
  [zoomify]

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 20x (5294)

Desmoplastic trichoepithelioma near basalioma:
Desmoplastic trichoepithelioma, HE 20x (5011)
  [zoomify]

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 20x (5023)
  [zoomify]

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 20x (5296)

Desmoplastic trichoepithelioma:
Desmoplastic trichoepithelioma, HE 20x (5091)
  [zoomify]

6.4.1.10  Tricholemmoma

Clinical signs:

  • solitary tricholemmoma is a small papule, several mm in size
  • location: usually the face
  • multiple tricholemmomas are found in Cowden's disease

Histology:

Nests or larger solid tumors of pale, glycogen containing epithelium; peripheral palisading, usually thick basement membrane.

6.4.1.10.1  Desmoplastic tricholemmoma

Histology:

Variant of the tricholemmoma, where multiple, irregular tumorous nests of pale epithelium with palisading grow between dermal collagen.

Pictures

Desmoplastic tricholemmoma:
Desmoplastic tricholemmoma, HE 20x (1954)

6.4.1.11  Trichomatricoma (pilomatrixoma)

Clinical signs:

  • firm, roundish, solitary subcutaneous nodule
  • location: variable, especially the upper extremities or the face
  • size: 5 – 30 mm
  • age: any age, common in children

Histology:

Nodule consisting of dark, basophilic cells, often of high mitotic activity and pale, eosinophilic areas of keratin and necrotic cells with eosinophilic cytoplasm and lost nucleus (ghost cells).

Dystrophic calcifications and inflammatory reaction are common, sometimes ossificatinons can be found.

6.4.1.11.1  Trichomatrical carcinoma

Introduction:

Rare malignant variant of pilomatricoma.

Pictures

Malignant pilomatricoma of sacral area. Infiltrative tumor after repeated local resections:
Trichomatricoma, malignant, HE 40x (5036)
  [zoomify]
Trichomatricoma, malignant, HE 40x (5037)
  [zoomify]

6.4.1.12  Folliculosebaceous hamartoma

Histology:

Intradermally located tumor, consisting of centrally located mesenchymal part (fat, blood vessels, fibrous tissue), surrounded by malformed follicles with prominent sebaceous glands.

6.4.1.13  Tumor of the follicular infundibulum

Clinical signs:

Tumor located usually on the face, may be multiple (face, neck, trunk).

Histology:

Shallow acanthosis of pale, interconnected keratinocytes without atypia, PAS positive. The tumor is well circumscribed.

Pictures

Tumor of the follicular infundibulum:
Tumor of the follicular infundibulum, HE 40x (5473)

6.4.1.14  Trichodiscoma

Clinical signs:

  • papules cca 5 mm of size
  • lokalization: especially the face
  • age: over 50y
  • multiple in Birt-Hogg-DubĂ© syndrome (+ kidney tumors and pulmonary cysts)

Histology:

Fine collagenous bundles with some mucin and sebaceous glands in various combinations. This tumor is close to fibrofolliculoma, where epithelial part is more prominent.

6.4.2  Tumors of the sweat glands

Introduction:

Signs of sweat gland differentiation: tubules and ducts, secretion, sometimes papillary formations; eccrine or apocrine secretion.

6.4.2.1  Eccrine nevus

Clinical signs:

  • papular lesions or areas of hyperhidrosis
  • very rare

Histology:

Groups of normal or enlarged eccrine sweat glands.

6.4.2.1.1  Eccrine angiomatous hamartoma

Histology:

Eccrine glands, blood vessels and sometimes nerve fibres.

6.4.2.2  Cylindroma

Clinical signs:

  • location: usually scalp, face
  • elastic, roundish tumors
  • pink or skin colored papules
  • often multiple

Pictures

Cylindroma and trichoepitheliomas of the back, familial Brooke-Spiegler syndrome
Cylindroma, CLINIC (693)

Cylindroma and trichoepitheliomas of the ear, familial Brooke-Spiegler syndrome
Cylindroma, CLINIC (694)

Histology:

Intraepidermal tumor consisting of (usually) multiple islands of tumorous cells, which are surrounded by hyaline sheath and fit together like pieces of a jigsaw puzzle. The hyaline sheaths and hyaline droplets within the tumor are PAS positive.

The tumor consists of two kinds of cells: smaller, on the periphery of the nests (with narrower cytoplasm), and larger, more centrally located, with pale nucleus and eosinophilic cytoplasm.

The tumor can contain tiny tubules.

Pictures

Cylindroma:
Cylindroma, HE 40x (2017)

Cylindroma:
Cylindroma, HE 20x (13946)

6.4.2.3  Spiradenoma

Clinical signs:

  • intradermal nodule, size up to 2 cm, rarely more
  • multiple, linearly arranged forms exist
  • age: usualy young adults
  • nodules are tender, sometimes painful
  • localization: variable

Histology:

Intradermal tumorouus nodules, sharp circumscription, in scanning magnification may resemble lymphnodes. Bands of tumorous cells with small, dark nuclei on the periphery and larger, pale nuclei in the centre. No hyaline sheats around the bands are present. In addition tubules, PAS positive hyaline granules and scattered lymphocytes are present.

Sometimes this tumor is mixed with cylindroma. In some tumors the cells form small rosettes.

Eccrine and apocrine variants exist.

6.4.2.4  Nodular hidradenoma

Histology:

Well circumscribed tumor, consisting of one or more tumorous nodes, solid, cystic, tubular.

Solid areas contain several kinds of cells: pale (or sometimes clear) cells and dark (with eosinophilic cytoplasm), sometimes squamoid and mucinous cells are present as well. Cystic areas usually contain eosinophilic amorphous material. The lining is flat. Tubules are lined by cubic or cylindric epithelium.

Mitotic activity is present even in benign tumors, the diagnostic criteria of malignity are cellular polymorphism and invasive growth.

Clear cell hidradenoma consists of pale or clear cells.

Pictures

Nodular hidradenoma:
Hidradenoma, nodular, HE 40x (2021)

Another case of nodular hidradenoma:
Hidradenoma, nodular, clear cell, HE 20x (4439)

Solid hidradenoma:
Solid hidradenoma, HE 40x (12406)

Solid hidradenoma:
Solid hidradenoma, HE 60x (12407)

Solid hidradenoma:
Nodular hidradenoma, HE 60x (10991)

Solid hidradenoma:
Hidradenoma, nodular, HE 40x (13021)

Solid and papillary hidradenoma:
Hidradenoma, solid and papillary, HE 40x (12683)

Cystic and nodular hidradenoma:
Hidradenoma, cystic, HE 40x (2682)

Cystic hidradenoma, focally with clear cells:
Hidradenoma, nodular, clear cell, HE 20x (4225)
  [zoomify]

Another case of nodular hidradenoma:
Nodular hidradenoma, HE 40x (10033)

Another case of hidradenoma with pale cells:
Hidradenoma, nodular, clear cell, HE 20x (4671)
  [zoomify]

Clear cell hidradenoma:
Clear cell nodular hidradenoma, HE 60x (6391)

Clear cell hidradenoma:
Hidradenoma, nodular, clear cell, HE 60x (12682)

Clear cell hidradenoma:
Hidradenoma, nodular, clear cell, HE 40x (13034)

Clear cell hidradenoma:
Clear cell myoblastoma, HE 60x (12014)

6.4.2.4.1  Cystic nodular hidradenoma

Histology:

Variant of nodular hidradenoma with prevalence of cysts (solid structures are present as well, as opposed to apocrine hidrocystoma).

6.4.2.4.2  Apocrine hidradenocarcinoma

Clinical signs:

  • large nodule
  • location: almost anywhere
  • age: over 50
  • rare

Histology:

Large tumor with focal apocrine differentiation, infiltrative growth, mitotic activity, focal necroses. Malignant conterpart of solid hidradenoma.

6.4.2.5  Syringoma

Clinical signs:

  • location: periorbital skin, neck, genital; more common in women
  • tiny, smooth nodules
  • color: skin color or pink
  • variants: linear, eruptive
  • clinically may resemble eczematous dermatitis (specimens of syringoma come under this diagnosis to pathology sometimes)

Pictures

Eruptive syringomas, face:
Syringoma, CLINIC (762)

Syringoms, face:
Syringoma, CLINIC (927)

Syringomas, lower eyelid:
Syringoma, CLINIC (928)

Syringomas, eyelids:
Syringoma, Macro (3971)

Syringoma, Macro (3972)

Syringoma, Macro (3973)

Another case of periocular syringoma:
Periocular syringoma, CLINIC (5820)

Periocular syringoma, CLINIC (5821)

Histology:

The tumor consists of many tiny cellular nests and ducts, containing PAS positive material. The lining is flat or two-layered. Focal keratinisation is often present. Small cellular nests are assymmetric, of tadpole shape. The tumor is usually small, limited to the upper dermis.

6.4.2.6  Papillary eccrine adenoma

Clinical signs:

  • well circumscribed intradermal tumor
  • papule, size about 1 cm
  • location: extremities

Histology:

Intradermal tumor, consisting of small cysts (some cysts are of middle size), filled by eosinophilic debris. Two layered epithelial lining (at least focally), small papillary projections. Cuboidal cells, no squamos differentiation (no granular layer, inner surface of the cysts is uneven). No mitotic activity.

6.4.2.7  Apocrine nevus

Clinical signs:

  • location: thorax, especially axilla

Histology:

Groups of normal or enlarged sweat glands.

Pictures

Apocrine nevus:
Nevus, apocrine, HE 20x (4890)
  [zoomify]

6.4.2.8  Mammary gland anal polyp
  • anal polyp

Histology:

Fibrous stroma and ectopic breast tissue; positive reaction to estrogen receptors.

6.4.2.9  Chondroid syringoma

Clinical signs:

  • firm subcutaneous nodules
  • size up to 2 cm

Histology:

Roundish tumor, benign, often separated from surrounding dermis by a cleft. Also called mixed tumor of the skin (usually contains areas of chondroid appearance). Two forms are recognized: tubular and branching (apocrine) and form with small tubular lumina (probably eccrine). The tumor cells are CEA positive.

Apocrine variant is more common; epithelium is high, basophilic, two layered epithelium; forming duct-like and cystic structures and epithelial nests. The outer layer of the ducts is vimentin positive. Stroma contains mucin and often is of chondroid appearance. Epithelium sometimes shows decapitation secretion.

Eccrine variant consists of roundish tubules lined with single layer of epithelial cells. Stroma contains mucin; chondroid changes are less apparent than in apocrine type.

6.4.2.10  Syringocystadenoma papilliferum

Clinical signs:

  • location: face, sometimes elsewhere
  • papule or sometimes several, linearly arranged papules
  • inborn malformation
  • enlarges during puberty

Histology:

Invagination, lined in the depth with apocrine epithelium of two layers of cells with apocrine secretion. Near to the surface the lining changes into squamous epithelium. Many papillary projections extend into the lumen.

The dermis contains a dense inflammatory infilrate with many plasma cells.

Sometimes malformed follicles and sebaceous glands are found near the lesion. Vulgar verruca is sometimes present as well.

6.4.2.11  Hidradenoma papilliferum

Clinical signs:

  • location: perigenital and perianal region, in women only (esp. on labia maiora)
  • size: several milimeters

Histology:

Well circumscribed, sometimes encapsulated cystic apocrine adenoma, located within the dermis. The tumor contains tubules, cysts and especially many papillary projections, covered by one row of columnar cells.

6.4.2.12  Tubular apocrine adenoma

Clinical signs:

  • location: variable
  • size: 1 – 2 cm or more

Histology:

Apocrine glands, cysts and tubules; sometimes focal papillary structures; sometimes communication with the infundibula.

Pictures

Tubular apocrine adenoma:
Adenoma, tubular apocrine, HE 40x (4840)
  [zoomify]

Tubular apocrine adenoma:
Adenoma, tubular apocrine, HE 60x (14024)

Adenoma, tubular apocrine, HE 60x (14025)

Tubular apocrine adenoma, in superficial part there is syringocystadenoma papilliferum; deep tubular part with myoepithelial cells positive for smooth muscle actin:
Tubular apocrine adenoma, HE 40x (6051)

Tubular apocrine adenoma, SMA 40x (6052)

6.4.2.13  Poroma

Introduction:

Eccrine poroma is a tumor of sweat glands, consisting mostly of basaloid cells growing in solid pattern. Focally small ducts can be found. Three variants of this tumor are recognized: eccrine poroma with tumorous nests broadly connected to the epidermis, hidroacanthoma simplex (with clonal grow limited to the epidermis) and dermal duct tumor (dermal form with no connection to the epidermis). Malignant poromas (porocarcinomas) are rare.

6.4.2.13.1  Eccrine poroma

Clinical signs:

  • location: soles (2/3 of all cases), hands, fingers and other locations
  • age: usually adults
  • papule, size up to 20 mm, usually solitary

Histology:

The tumor is located within the dermis, but communicates with the epidermis by broad bands of tumorous epithelium. the cells are basaloid: roundish, regular, paler than normal keratinocytes. The borders between the tumor and the epidermis is usually easy to recognize. Small ducts, small foci of keratinisation and areas of regression with nuclear pyknosis are usually present.

6.4.2.13.2  Hidroacanthoma simplex

Histology:

Clonal variant of the eccrine poroma, where basaloid, pale (glycogen), round cells form intraepidermal nests. The epidermis is usually acanthotic. Differential diagnosis of this tumor against clonal seborrhoic keratosis is difficult (duct formation, location —  seborroic keratoses do not appear on soles).

6.4.2.13.3  Dermal duct tumor

Histology:

One or more round dermal nodules composed of basaloid, regular, pale cells. Focally tiny ducts with eosinophilic cuticle and keratinisation (squamous eddies) are formed. The tumor may be partially cystic.

6.4.2.13.4  Poroma, malignant (porocarcinoma)

Clinical signs:

  • location: extremities (legs, feet)
  • age: adult
  • nodule, plaque, ulcerated tumor
  • multiple cutaneous metastases may develop

Histology:

Poroma cells, cellular polymorphism, mitotic activity, hyperchromatic nuclei, irregular tumorous nodules.

6.4.2.13.5  Syringofibroadenoma

Clinical signs:

  • hyperkeratotic papule
  • location: extremity
  • size: up to several centimeters
  • rare tumor

Histology:

Anastomosing epithelial strands of basaloid cells communicating with the epidermis and with occassional formation of lumina. Eccrine and apocrine variants exist.

Pictures

Syringofibroadenoma:
Eccrine syringofibroadenoma, HE 40x (4826)
  [zoomify]

Reactive syringofibromatosis? Probably reactive change of the amputation stump:
Reactive syringofibromatosis, HE 20x (4971)
  [zoomify]

Syringofibroadenomatous proliferation, palm:
Syringofibroadenoma, palm, HE 20x (13687)

6.4.2.14  Intraepidermal epithelioma of Borst Jadassohn

Histology:

Nests of intraepidermal tumorous cells, originally described as intraepidermal basalioma, are now considered to be clonal seborrhoic keratosis or intraepidermal poroma (hidroacanthoma simplex).

Pictures

Intraepidermal epithelioma of Borst Jadassohn:
Intraepidermal epithelioma Borst-Jadassohn, HE 20x (1942)

Intraepidermal epithelioma Borst-Jadassohn, HE 40x (1943)

Intraepidermal epithelioma, shave biopsy, lesion of unknown biological properties:
Intraepidermal epithelioma Borst-Jadassohn, fragmented shave biopsy, HE 40x (3626)

Verruca seborrhoica with Borst-Jadassohn phenomenon:
Verruca seborrhoica, Borst-Jadassohn phenomenon, HE 60x (11012)

Verruca seborrhoica with Borst-Jadassohn phenomenon:
Verruca seborrhoica, clonal, HE 40x (12570)

6.4.2.15  Microcystic adnexal carcinoma

Clinical signs:

  • location: head (upper lip, chin, cheek)
  • deep invasion, local aggresivity
  • no metastases

Histology:

Large, deeply infiltrating tumor, consisting of epithelial nests, ducts, glandular structures. Sometimes focal hair follicle differentiation. Mitoses are rare. Perineural spread is sometimes present.

6.4.2.16  Adenoid cystic carcinoma

Clinical signs:

  • very rare in the skin
  • reccurences occur often, but metastases are rare

Histology:

Masses of small, regular, basophilic cells form cribriform structures and small cystic spaces filled with mucin. These spaces are lined by cuboid or flattened epithelium. Infiltrative growth, low or none mitotic activity, sometimes perineural spread.

Resembles adenoid basalioma, but shows no peripheral palisading on the periphery of the tumorous nests. The cells of adenoid cystic carcinoma are positive for carcinoembryonal antigen and S100 (basalioma cells are negative).

6.4.2.17  Eccrine carcinoma

Clinical signs:

  • localization: face, sometimes elsewhere
  • skin nodule, growing slowly at first, later infiltrative
  • may ulcerate
  • rare

Histology:

Infiltrative growing tumor, consisting of irregular nests of atypical cells. Focally the differentiation towards eccrine ducts is present. Variable desmoplasia.

Pictures

Eccrine carcinoma:
Eccrine carcinoma, HE 40x (6252)

6.4.2.18  Mucinous carcinoma of the skin

Clinical signs:

  • rare tumor
  • location: face, esp. eyelids, but sometimes elsewhere
  • slow growth
  • size: up to several centimeters
  • metastatic rate: 15%

Histology:

Small groups of epithelial cells surrounded by large amounts of mucin. Small, sometimes signet ring cells.

6.4.2.19  Fibroadenoma

Introduction:

Mammary-like glands can be found in the skin along so called milk line from axilla to external genital. Tumors analogic to breast tumors can rarely arise from these glands.

Pictures

Fibroadenoma, axilla (isolated finding, v.s. of the mammary type glands):
Fibroadenoma, HE 60x (13884)

6.4.3  Sebaceous tumors

Introduction:

Benign and malignant processes of sebaceous epithelium (hyperplasia, benign and malignant tumors). Processes, in which (cytologically) normal sebaceous glands form a part of complex tumor are mentioned elsewhere (sebaceous nevus, folliculosebaceous hamartoma etc.)

6.4.3.1  Ectopic sebaceous glands

Clinical signs:

  • location: lip, buccal mucosa — Fordyce's spot
  • location: areola mammae — Montgomery's tubercle

Pictures

Ektopic sebaceous glands, lips:
Ectopic sebaceous glands, CLINIC (6356)

Histology:

Normal sebaceous glands not connected to any hair follicle.

Pictures

Fordyce spot (lip):
Fordyce's spot, lip, HE 40x (4877)

Montgomery glands hypertrophy, areola mammae:
Hypertrophy of Montgomery glands, areola mammae, HE 60x (13327)

Another case of Montgomery gland, areola mamme:
Montgomery tubercle, HE 60x (13944)

6.4.3.2  Nevus sebaceus (of Jadassohn)

Introduction:

Tissue malformation, mentioned in the chapter on organoid nevi and tissue malformations

6.4.3.3  Sebaceous hyperplasia

Clinical signs:

  • location: face (forhead, face)
  • persons of middle age
  • one or several soft papules, size less than 3 mm, often with central dimple

Histology:

The lesion consists of hyperplastic, enlarged sebaceous gland and the duct leading to the surface. Sebaceous epithelium is mature. If immature, basaloid cells are present, than they are located on the periphery of the lobules, and their number is low.

6.4.3.3.1  Sebaceous hyperplasia of the penis

Pictures

Papules of the dorsum of the penis, caused by hyperplastic sebaceous glands:
Hyperplasia, sebaceous, dorsum penis, HE 40x (3565)

Hyperplasia, sebaceous, HE 40x (4710)

6.4.3.4  Sebaceous adenoma

Clinical signs:

  • location: face, scalp
  • papule or nodule of yellowish color
  • adenoma sebaceum is something else, namely angiofibroma in tuberous sclerosis

Histology:

Resembles a group of enlarged sebaceous glands; immature basaloid cells are on the periphery.

6.4.3.5  Sebaceoma, sebaceous epithelioma

Clinical signs:

  • slowly growing papules

Histology:

Tumor with focal sebaceous differentiation; more or less mature sebaceous cells are mixed randomly with basophilic, immature sebaceous epithelia. Scattered mitoses may be present.

Pictures

Sebaceoma:
Sebaceoma, HE 40x (2736)

Another case (small, roundish tumor of the nose):
Sebaceoma, HE 40x (5488)

Sebaceoma, Ki67 20x (5487)

Sebaceoma, probably malignant (sebaceous carcinoma):
Sebaceoma, sebaceous carcinoma, HE 20x (4602)
  [zoomify]

Sebaceoma:
Sebaceoma, HE 60x (13444)

Sebaceous carcinoma:
Sebaceous carcinoma, HE 60x (12251)

Sebaceous carcinoma, PAS 40x (12302)

Sebaceoma:
Sebaceoma, HE 60x (13786)

Sebaceoma:
Sebaceoma, HE 60x (13787)

6.4.3.6  Muir-Torre syndrome

Clinical signs:

Association of mulitple sebaceous tumors (sebaceous hyperplasias, sebaceous adenomas and carcinomas) and/or keratoacanthomas with visceral carcinomas (often of the colon).

6.4.4  Combined adnexal tumor

Histology:

Benign, roundish tumor consisting of areas showing areas of eccrine (or apocrine) and trichoepithelial differentiation.

6.4.5  Cowden syndrome

Clinical signs:

  • inheritance: autosomal dominant, chromosomal aberration on 10q23
  • variable expression
  • multiple hamartomas
  • danger of malignant transformation (skin, breast, thyroid)
  • age: manifestation starts at second or third decade

Histology:

  • Skin and mucosal lesions:
    • trichilemmomas, inverted follicular keratoses, tumors of follicular infundibulum of the face
    • acral keratoses (dorsal aspect)
    • palmoplantar keratosis
    • papillomatous papules of the face (6 papules, out of it 3 trichilemmomas)
    • mucous membranes: fibromas, lingua scrotalis
  • Main criteria:
    • carcinoma of the breast (ductal carcinoma)
    • carcinoma of the thyroid gland (follicular carcinoma)
    • makrocephaly
    • Lhermite-Duclos disease (hamartomas of the cerebellum)
    • endometrial carcinomas
  • Minor criteria:
    • thyroid gland disorders
    • low IQ
    • hamartomas of the GIT
    • fibrocystic disease of the breast
    • lipomas, fibromas
    • genitourinary tumors (Grawitz)

Pictures

Cowden syndrome:
Cowden's disease, HE 20x (4441)



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