title: Nasopharyngeal rhabdomyosarcoma in a patient with hypohidrotic ectodermal dysplasia syndrome.
abstract: Hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is characterized by partial or complete absence of sweat glands, hypotrichosis, hypodontia, prominent frontal ridges and chin, saddle nose, sunken cheeks, thick, everted lips, large ears and sparse hair. While association of other ectodermal dysplasia syndromes with tumors such a non-Hodgkin's lymphoma, hamartoma, keratoakanthoma, Merkel-cell cancer, squamous-cell carcinoma, syringofibroadenomatosis has been reported, association of hypohidrotic ectodermal dysplasia and a tumor has not been reported. In a five-year-old male patient admitted with nasal obstruction and nasal mass complaints, we have reported firstly an association of hypohidrotic ectodermal dysplasia and nasopharyngeal rhabdomyosarcoma.
authors.affiliation: Department of Otolaryngology, H & N Surgery, Faculty of Medicine, Yüzüncü Yil University Tip Fakültesi, 65200 Van, Turkey. hcankaya2@hotmail.com
authors.forename: Hakan
authors.initials: H
authors.lastname: Cankaya
authors.forename: Mustafa
authors.initials: M
authors.lastname: Kösem
authors.forename: Muzaffer
authors.initials: M
authors.lastname: Kiris
authors.forename: Abdurrahman
authors.initials: A
authors.lastname: Uner
authors.forename: Ahmet
authors.initials: A
authors.lastname: Metin
mesh_terms: D001706:Biopsy; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D015508:Nasal Obstruction / Q000175:diagnosis / Q000473:pathology; D009303:Nasopharyngeal Neoplasms / Q000175:diagnosis* / Q000473:pathology; D009305:Nasopharynx / Q000473:pathology; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology; D014057:Tomography, X-Ray Computed