title: Benign follicular tumors.
abstract: Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Oscar
authors.initials: O
authors.lastname: Tellechea
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: José Carlos
authors.initials: JC
authors.lastname: Cardoso
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: José Pedro
authors.initials: JP
authors.lastname: Reis
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Leonor
authors.initials: L
authors.lastname: Ramos
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Ana Rita
authors.initials: AR
authors.lastname: Gameiro
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: Inês
authors.initials: I
authors.lastname: Coutinho
authors.affiliation: Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.
authors.forename: António Poiares
authors.initials: AP
authors.lastname: Baptista
mesh_terms: D049309:Acanthoma / Q000473:pathology; D000236:Adenoma / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005497:Follicular Cyst / Q000473:pathology; D006201:Hair Diseases / Q000145:classification / Q000473:pathology*; D018859:Hair Follicle / Q000473:pathology*; D006801:Humans; D008297:Male; D018295:Neoplasms, Basal Cell / Q000473:pathology; D012878:Skin Neoplasms / Q000145:classification / Q000473:pathology*; D013577:Syndrome