High risk of renal cell carcinoma
Also, pulmonary cysts and pneumothoraces
Clinical Issues
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Typically present as multiple small papule(s) on face
Microscopic
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FF shows proliferation of thin, anastomosing epithelial cords
Numerous attachments to follicular structures and epidermis
Typically find central, dilated follicular structure
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TD is stromal-predominant lesion, may show only small/miniaturize follicle
Top Differential Diagnoses
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Tumor of follicular infundibulum
Superficial, plate-like proliferation of bland pale to clear squamoid cells
Anastomosing strands of epithelium present, but usually thicker than those in FF
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Inverted follicular keratosis
Endophytic epithelial proliferation with numerous squamous eddies
Typically shows multiple pseudohorn cysts, suggesting that these are variants of irritated seborrheic keratoses
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Basal cell carcinoma, infundibulocystic type
Basal cell carcinoma shows areas of peripheral palisading and tumor-stromal retraction artifact
TERMINOLOGY
Abbreviations
Synonyms
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Mantleoma (likely FF variant with sebaceous differentiation)
Definitions
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Closely related hamartomatous proliferations with follicular and mesenchymal differentiation
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Trichodiscoma likely represents late stage in evolution of FF showing stromal predominance
ETIOLOGY/PATHOGENESIS
Genetic Syndrome