Figure 1. Intraoperatory view of the mass. DOI http://dx.doi.org/10.13070/rs.en.1.1016 Date 2014-09-10 Cite as Research 2014;1:1016 License CC-BY Benign fibrous histiocytoma of the nasal vestibule: report of a case and review of literature Ioannis Mylonakis1, 2 (ioannis dot mylonakis at unipd dot it) #, Enzo Emanuelli3, Barbara Pedruzzi1, 3, Filippo Marino4, Alessandro Martini1, 3 1 Department of Neurosciences, Univerity of Padua, Italy. 2 ENT Clinic, University Hospital of Padova, Italy. 3 Endoscopic Airways Surgery Unit, University Hospital of Padova, Italy. 4 Anatomical Pathology Unit, University Hospital of Padova, Italy # : corresponding author Benign fibrous histiocytoma (BFH) is a rare connective tissue tumor arising in skin (dermatofibroma), soft tissues and bone (“deep” fibrous histiocytoma). Few cases of soft tissue BFH have been reported in the head and neck and localizations in the nasal fossa are extremely rare. We report the presence of a soft tissue BFH in the right nasal vestibule of a 54 year old male, the first well defined report in the English literature of a tumor of this kind in this particular site. Introduction Benign benign fibrous histiocytoma (BFH) was first described as a separate clinical entity in the 1960s after a lengthy process aiming towards a precise identification of fibrohistiocytic lesions and the distinction between benign and malignant variants [1]. It is now considered a benign mesenchymal lesion composed of fibroblasts and histiocytes arising in the cutaneous and non-cutaneous soft tissues, although disputes still exist on whether this is a true tumor or a reactive mass at least as for the cutaneous form [2] [3]. Confusion on the nature of fibrohistiocytic lesions also accounts for the variety of names used to describe this tumor and its variants: Dermatofibroma, sclerosing hemangioma, adult xanthogranuloma, fibroxanthoma, and nodular subepidermal fibrosis are some of the terms more commonly used in the past to identify this tumors [1]. While cutaneous BFH (dermatofibroma) usually originates in sun-exposed skin, non-cutaneous soft tissue BFH usually presents itself in subcutaneous tissues of the extremities and more rarely in visceral spaces like the retroperitoneum and pelvis; localizations in tendons are rare and visceral presentations anecdotal [3]. The “deep” soft tissue variant of the tumor seems to be relatively more frequent in young children, especially in the head and neck region [4]. BFH arising in the orbital tissues represents the most frequent primary mesenchymal tumor of the orbit in adult patients and accounts for most of the head and neck localizations that would otherwise be rare [5]. In the ENT practice BFH has been more commonly encountered in the bone and soft tissues of the oral cavity such as in the tongue, gingival or alveolar ridge, mandible, maxilla, lips, soft palate, and floor of the mouth. The nasal cavity and paranasal sinuses, larynx, trachea, temporomandibular joint, and submandibular and parotid glands are more rarely involved [5]. A malignant subtype of fibrous histiocytoma has also been described [2] [3]. Tumors of the nasal vestibule are generally rare and reports of nonepithelial variants absolutely exceptional [6] ; no definite cases of fibrohistiocytic tumors in this site are present in literature so far. Case report In February 2012 a 54 year old Italian male presented in the outpatient ambulatory of the Endoscopic Upper Airways Surgery Unit of the University Hospital of Padua with a large, nontender mass of the right nasal vestibule which he had noticed 3 months earlier and had rapidly grown in size since then. The tumor caused local discomfort and unilateral nasal obstruction. The patient referred no specific personal or professional risk factors for nasal disease apart from persistent nasal picking. In his general history there were no other significant pathologies apart from hypertension under treatment with vasodilators. On direct examination the lesion appeared as a pedunculated mass without alterations of the overlying mucosal lining, originating from the alar region of the right nostril (Fig. 1). Complete upper aerodigestive tract endoscopic examination revealed no other lesions. Topics paranasal sinus neoplasms benign fibrous histiocytoma histiocytes