Trichilemmal carcinoma was originally
described by Headington as a "histologically invasive, cytologically atypical
clear cell neoplasm of adnexal keratinocytes which is in continuity with the
epidermis and /or follicular epithelium."
The outer root sheath consists of cells with clear vacuolated cytoplasm
due to presence of abundant glycogen. The trichilemmal carcinoma is a malignant
tumour of the outer hair sheath.
Clinical presentation:
A slow growing tumour which shows predilection for sun- exposed hair
bearing skin.
Clinically, the tumour presents as a pale tan or reddish papule, indurated
plaque or nodule. The tumour size is usually between 0.4 and 2.0 cms.
Sites:
The usual sites include
scalp, face, trunk, upper extremities.
Microscopic Images:

Microscopic features:
Tumour shows wide range of growth patterns (solid,
lobular, trabecular) ; tumour lobules are sharply circumscribed by a
hyaline, PAS(+) membrane, and infiltrates with a pushing border ; lobules consist of large tumour cells with PAS reactive and diastase
sensitive clear or pale eosinophilic cytoplasm ; stain for mucin is negative
; foci of pilar-type keratinization and peripheral palisading of cells with subnuclear vacuolization
; some dyskeratotic cells & numerous mitotic figures ; actinic damage
; superficial ulceration; in some cases tumour shows pagetoid
spread ; others infiltrate into
the deep dermis.
Immunohistochemistry:
Immunocytochemistry reveals positivity for cytokeratin and negativity for
CEA and EMA.
Clinical course:
Despite an aggressive growth it has an indolent clinical course. No cases
with recurrence or metastasis have been reported in the literature.
Differential diagnosis:
Clear cell carcinomas of the skin.
Benign tumours like
trichilemmoma and hidradenoma
lack the infiltrative
growth pattern and cytological atypia.
Trichilemmal carcinoma and malignant proliferating trichilemmal tumour are
two separate entities. The latter, usually arises in a preexisting
trichilemmal cyst and has metastatic potential. |