Trichoepithelioma is regarded as a poorly differentiated hamartoma of
the hair germ.
Although most
classifications include this as a separate entity Ackerman and his
colleagues have suggested that such tumours should be grouped together
with trichoblastomas and use the term "trichoblastoma" for all tumours
showing predominant hair germ differentiation.
Site:
Commonly located on the head and neck region. Solitary- Nose, upper lip
and cheeks. Multiple- Central part of the face, trunk, neck & scalp.
Clinical presentation:
Skin coloured papules. May be
solitary or multiple. Papules may coalesce to form plaques. Rare
presentation is a linear form.
Multiple tumours (epithelioma
adenoides cysticum) inherited as autosomal dominant.
Brooke-Spiegler
syndrome: Multiple trichoepitheliomas and cylindromas.
Microscopic features :
Well circumscribed dermal tumour ; Islands, nests and cords of
uniform basaloid cells ; Cells are set in a variably cellular fibrous stroma ; Epithelial structures resemble hair papillae or abortive hair
follicle ; Small keratocysts (infundibular differentiation) lined by
stratified squamous epithelium ; Retraction of stroma from adjacent
dermis ; Foci of calcification are often present ; Few mitotic figures
and apoptotic bodies.
Immature
trichoepithelioma: Typically exhibits no horn cysts, displays
fewer primitive hair structures, and lacks the adenoidal growth patterns
of the tumour lobules which are usually present in the classical
trichoepitheliomas.
Differential diagnosis :
1.
Basal cell carcinoma;
Distinction from some types
of basal cell carcinoma has been based on the paucity of mitoses and
apoptotic bodies, lack of retraction between stroma and epithelium and
presence of primitive follicles in trichoepithelioma and differences in
CD34 and bcl2 expression. However, there are some tumours in which this
distinction cannot be reliably made.
2.
Microcystic adnexal carcinoma ;
4.
Trichoadenoma ;
5.Trichoblastoma ;
ImageLink(Dr
Weems) CaseLink
Clinical image(DermAtlas):
IMAGE1
;
IMAGE2
DESMOPLASTIC TRICHOEPITHELIOMA:
Desmoplastic
trichoepithelioma is a benign neoplasm considered to have follicular
differentiation. Its sweat gland- or sebaceous-lines of differentiation
have been also reported.
The cells in
desmoplastic trichoepithelioma are suggested to be in close association
with the basal cells in the outer root sheath.
Diagnosis of this
lesion is important because it may be mistaken for
Microcystic adnexal
carcinoma
clinically and
histologically.
This condition should
not be diagnosed on shave, curettage or punch biopsies.
Deep biopsy is necessary to identify subcutaneous involvement.
Clinical presentation:
Presents as a solitary , small indurated often depressed plaque on the
face.
Microscopic features:
Symmetrical well-circumscribed ; Lesions are usually located
in the mid and upper dermis ; Tumour consists of cords and small nests
of uniform basaloid cells in a dense fibrotic stroma ; Superficially,
small horn cysts and keratin granulomas may be present ; No ductal
differentiation.
Differential diagnosis:
1.Syringoma - Usually periorbital and multiple lesions ; Narrow strands of tumour cells ;
Horn cysts, foreign body granuloma or calcifications are rare.
2.Morpheic basal cell
carcinoma - Form clefts between the nests and the stroma ; Coexisting
nodular basal cell carcinoma; Mitoses and apoptotic bodies are common;
Foreign body granulomas and ruptured keratinous cysts are rare.
3.Microcystic
adnexal carcinoma:
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4.Metastatic breast
carcinoma
Image
Link(Dr Weems)
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