Tricholemmoma

 Benign clear cell adnexal proliferation with external root sheath differentiation



• Subtype: Desmoplastic tricholemmoma




Etiology/Pathogenesis




• Some cases are associated with Cowden syndrome (PTEN hamartoma syndrome)
image Multiple tricholemmomas, hamartomas, and visceral tumors including breast and thyroid carcinomas

image Mutations in PTEN gene on 10q23.31

• Some have considered TL to be related to HPV infection (“tricholemmal verrucae”), but not well-accepted


Clinical Issues




• Relatively common tumors

• Usually occur in adults, although Cowden syndrome patients present at earlier age

• Most occur on face, especially nose and upper lip


Microscopic




• Lobular proliferation of mature squamoid cells with pale- to clear-staining cytoplasm
• Peripheral palisading of basaloid cells

• Cells are surrounded by thickened, glassy-appearing basement membrane

• Multiple broad connections to epidermis and follicles

• Desmoplastic tricholemmoma

image Variant with prominent desmoplastic stroma

image Typically in center of tumor, surrounded by conventional-appearing TL


Top Differential Diagnoses




• Tumor of follicular infundibulum

• Clear cell acanthoma

• Tricholemmal carcinoma

• Clear cell squamous cell carcinoma in situ (clear cell Bowen disease)

• Clear cell basal cell carcinoma

image
Clinical Photograph of Multiple Tricholemmomas
Multiple tricholemmomas (TLs) are shown on and around the nose of a patient with Cowden syndrome. (Courtesy B. Hall, MD.)


image
Superficial Tricholemmoma
This is a small, superficial tricholemmoma on the eyelid showing multifocal epidermal attachments image. The tumor is composed of pale- to clear-staining epithelioid cells.

image
Superficial Tricholemmoma
Higher magnification of a superficial TL shows a lobular proliferation of small, pale- to clear-staining image adnexal cells. Note the thickened basement membrane surrounding the tumor image.

image
High Magnification of Tricholemmoma Basement Membrane
High magnification of the periphery of a TL shows bland-appearing small cells with pale to clear-staining cytoplasm and small nucleoli. The peripheral cells show palisading, and there is a thickened peripheral basement membrane image.


TERMINOLOGY


Abbreviations




• Tricholemmoma (TL)

• Desmoplastic tricholemmoma (DTL)


Synonyms




• Trichilemmoma


Definitions




• Benign clear cell adnexal proliferation with external root sheath differentiation


ETIOLOGY/PATHOGENESIS


Unknown in Most Cases




• Some have considered TL to be related to HPV infection (“tricholemmal verrucae”)
image Not generally accepted, and most PCR studies for HPV have been negative


Genetic




• Some cases are associated with Cowden syndrome (PTEN hamartoma syndrome)
image Characterized by multiple tricholemmomas, hamartomas, and visceral tumors including breast and thyroid carcinomas

image Mutations in PTEN, a tumor suppressor gene, on 10q23.31


CLINICAL ISSUES


Epidemiology




• Incidence
image Relatively common tumors

• Age
image Usually adults, although Cowden syndrome patients present at earlier age


Site




• Most occur on face, especially nose and upper lip


Presentation




• Small papular lesion
image Usually flesh-colored

image Often clinically mimics basal cell carcinoma or verruca


Treatment




• Surgical approaches
image Usually not necessary, as these are benign tumors

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Tricholemmoma

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