Fibrofolliculoma and Trichodiscoma

 High risk of renal cell carcinoma


image Also, pulmonary cysts and pneumothoraces





Clinical Issues




• Typically present as multiple small papule(s) on face


Microscopic




• FF shows proliferation of thin, anastomosing epithelial cords
image Numerous attachments to follicular structures and epidermis

image Typically find central, dilated follicular structure

• TD is stromal-predominant lesion, may show only small/miniaturize follicle


Top Differential Diagnoses




• Tumor of follicular infundibulum
image Superficial, plate-like proliferation of bland pale to clear squamoid cells

image Anastomosing strands of epithelium present, but usually thicker than those in FF

• Inverted follicular keratosis
image Endophytic epithelial proliferation with numerous squamous eddies

image Typically shows multiple pseudohorn cysts, suggesting that these are variants of irritated seborrheic keratoses

• Basal cell carcinoma, infundibulocystic type
image Basal cell carcinoma shows areas of peripheral palisading and tumor-stromal retraction artifact

image
Clinical Photograph of Fibrofolliculoma
Clinical photograph of a patient with Birt-Hogg-Dubé syndrome shows numerous small, flesh-colored papules on the lateral cheek and neck image. (Courtesy J. Wu, MD.)


image
Fibrofolliculoma at Low Magnification
Fibrofolliculoma shows a central dilated (cystic) follicular structure with keratin debris. There are multiple thin, radiating epithelial strands attached to the overlying epidermis image.

image
Fibrofolliculoma With Central Follicular Structure
Fibrofolliculoma shows a central follicular structure with a dilated lumen and thin, radiating epithelial strands attached to both the follicle image and the epidermis image.

image
Trichodiscoma at Low Magnification
Trichodiscoma is composed mostly of fibromyxoid stroma but will often demonstrate a residual miniaturized follicular structure if multiple deeper level sections are examined.


TERMINOLOGY


Abbreviations




• Fibrofolliculoma (FF)

• Trichodiscoma (TD)


Synonyms




• Mantleoma (likely FF variant with sebaceous differentiation)


Definitions




• Closely related hamartomatous proliferations with follicular and mesenchymal differentiation

• Trichodiscoma likely represents late stage in evolution of FF showing stromal predominance


ETIOLOGY/PATHOGENESIS


Genetic Syndrome




• Multiple lesions are strongly correlated with Birt-Hogg-Dubé syndrome (BHD)
image Due to mutations in folliculin (FLCN) gene

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Fibrofolliculoma and Trichodiscoma

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