Appendageal skin tumours:

Introduction:
Appendageal tumours are neoplasms whose differentiation is toward one or more of
the adnexal structures of the skin. Depending on their presumed origin, adnexal
tumours are categorized into those with apocrine and eccrine, foliicular and se
baceous differentiation.
WHO histological classification of appendageal tumours
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Tumours with apocrine and eccrine differentiation
Malignant tumours
Tubular carcinoma 8211/3
Microcystic adnexal carcinoma 8407/3
Porocarcinoma 8409/3
Spiradenocarcinoma 8403/3
Malignant mixed tumour 8940/3
Hidradenocarcinoma 8400/3
Mucinous carcinoma 8480/3
Digital papillary carcinoma 8408/3
Adenoid cystic carcinoma 8200/3
Apocrine carcinoma 8401/3
Paget disease of breast 8540/3
Extramammary Paget disease 8542/3
Benign tumours
Hidrocystoma 8404/0
Syringoma 8407/0
Poroma 8409/0
Syringofibroadenoma 8392/0
Hidradenoma 8402/0
Spiradenoma 8403/0
Cylindroma 8200/0
Tubular adenoma 8211/0
Tubular papillary adenoma 8263/0
Syringocystadenoma papilliferum 8406/0
Hidradenoma papilliferum 8405/0
Mixed tumour (chondroid syringoma) 8940/0

Tumours with follicular differentiation

Malignant tumours
Pilomatrical carcinoma 8110/3
Proliferating tricholemmal tumour 8103/1
Benign tumours
Trichoblastoma 8100/0
Pilomatricoma 8110/0
Tricholemmoma 8102/0
Multiple tricholemmomas 8102/0
Trichofolliculoma 8101/0
Fibrofolliculoma / trichodiscoma 8391/0
Tumours with sebaceous differentiation
Sebaceous carcinoma 8410/3
Sebaceous adenoma 8410/0
Sebaceoma 8410/0
Cystic sebaceous tumour 8410/0
Histogenesis
The origin of most adnexal neoplasms is unknown The most clear-cut evidence of d
ifferentiation is in follicular neoplasms, where such signs as follicular papill
ae and germs (as in the trichoblastomas) or trichohyaline granules (as are focal
ly found in pilomatricoma and in matrical carcinomas) can occur.
Clearcut apocrine differentiation, in which decapitation secretion of co
lumnar cells that have brightly eosinophilic cytoplasmic granules is also specif
ic. However, there is a marked similarity between eccrine and apocrine ducts. Al
so, the columnar cells of eccrine secretory coils can resemble poorly differenti
ated apocrine secretory cells. Hence, neoplasms with ductular differentiation of
ten have debatable histogenesis .
Pathology
Diagnostic criteria of adnexal carcinomas :
> Irregular borders, asymmetry at scanning magnification
> Horizontal orientation
> Markedly irrregular aggregates of epithelial cells
> Necrosis en masse
> Infiltration of the dermis or subcutis without the interposition of densely
fibrotic stroma
> Mitoses frequent, can be atypical
> Stroma irregular, often scant, sometimes myxoid
> Nuclei pleomorphic. Some neoplasms with monomorphous nuclei, e.g. microcystic
adnexal carcinoma, are exceptions.
Diagnostic criteria of benign epithelial adnexal neoplasms :
> Symmetric and smooth bordered at scanning magnification
> Vertically oriented with respect to the surface of the skin
> Aggregates of epithelial cells uniform
> No necrosis en masse (with the excetion of poroma)
> Mitoses variable, but typical
> Densely fibrotic stroma, rich in fibrocytes in the case of trichogenic
> Neoplasms forming a blunt, rounded interface with the native dermis. An
exception is poroma, which has vascular, myxoid stroma.
> Nuclei monomorphous; rare exceptions include atypical squamous
nuclei in poromas.
Tumours with apocrine and eccrine differentiation
Cylindroma
Synonyms
Cylindrospiradenoma , spiradenocylindroma
Definition
Cylindroma is a relatively undifferentiated benign adnexal neoplasm with a mosai
c microscopical pattern. Cylindroma commonly occurs as a hybrid with spiradenoma
, an event that has been referred to as cylindrospiradenoma or spiradenocylindro
ma.
Cylindromas may be solitary or multiple, arising on a sporadic basis or as part
of Brooke-Spiegler syndrome. There is no sex predilection.
The etiology is unknown. A link to chromosome 9 seems likely for multiple spirad
enomas and cylindromas in the context of the Brooke-Spiegler syndrome, as the ge
ne has been mapped to 9p21.
Cylindromas are typically smooth, domeshaped hairless red-brown papules and nodu
les. Extensive scalp involvement can create clinical morphology resembling a hea
dpiece (â turban tumourâ ).
Histopathology
Cylindroma is a mostly dermal and sometimes subcutaneous neoplasm with a multino
dular, circumscribed pattern at scanning magnification. Individual nodules are c
omposed of mosaic nests of undifferentiated basaloid cells with small darkly-sta
ining nuclei and scant cytoplasm; The nests of cylindroma are commonly surrounde
d by a rim of densely eosinophilic PAS-positive basement membrane material.
Prognosis and predictive factors
Simple excision is usually curative. Malignant transformation is extremely
uncommon.
Hidrocystoma
Hidrocystomas are cystic proliferations of the sweat glands. They have either ap
ocrine or eccrine differentiation, with the majority being of apocrine nature. A
pocrine hidrocystomas are cystic adenomas that arise from the apocrine secretory
 coil, while eccrine hidrocystomas represent retention cysts of the eccrine cyst
duct.
Hidrocystomas have a predilection for the face and neck, mainly the periorbital
area.
Etiology
The exact cause of hidrocystomas is not known. They have been reported to be exa
cerbated with high temperatures and to completely disappear with cold weather an
d atropine therapy . There is an increased incidence of hidrocystomas in hyperth
yroid patients, perhaps related to hyperhidrosis.
They normally present as solitary lesions. Hidrocystomas usually affect middle-a
ged or older individuals although rare examples have been described in children
and adolescents; both sexes are equally affected.
Histopathology
Hidrocystomas can be uni or multilocular and are usually lined by a double layer
of epithelium. The inner layer contains large columnar cells with eosinophilic
cytoplasm which has luminal decapitation secretion, while the outer layer is fla
t and composed of myoepithelial cells. The term â papillary apocrine gland cystâ has
been applied for hidrocystomas with papillary projections of epithelium into the
lumen .
Immunoprofile
Hidrocystomas express epithelial membrane antigen (EMA) and lysozyme in the cell
s of the cyst wall; carcinoembryonicantigen (CEA) decorates the luminal cells .
The pattern of cytokeratin expression is variable ,there is expression of cytoke
ratins 7,8,18,19 in the luminal cell layer and cytokeratins 1,5,10,14 in the bas
al and luminal cell layers. Smooth muscle actin (SMA) is present in the basal la
yer . Human milk fat globulin 1 (HMFG) is expressed by the apocrine sweat gland
only . S-100 protein is positive in the secretory portion of normal eccrine glan
ds and in the myoepithelial cells of apocrine glands.
Prognosis and predictive factors
Complete excision is usually curative.