Nasopharyngeal rhabdomyosarcoma in a patient with hypohidrotic ectodermal dysplasia syndrome

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...

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Bibliographic Details
Published in:Auris, nasus, larynx nasus, larynx, 2002-07, Vol.29 (3), p.313-316
Main Authors: Çankaya, Hakan, Kösem, Mustafa, Kiris, Muzaffer, Üner, Abdurrahman, Metin, Ahmet
Format: Article
Language:English
Subjects:
Biopsy
Child, Preschool
Diagnosis, Differential
Ectodermal Dysplasia - diagnosis
Ectodermal Dysplasia - pathology
Fetal rhabdomyoma
Humans
Hypohidrotic ectodermal dysplasia syndrome
Male
Nasal Obstruction - diagnosis
Nasal Obstruction - pathology
Nasopharyngeal mass
Nasopharyngeal Neoplasms - diagnosis
Nasopharyngeal Neoplasms - pathology
Nasopharynx - pathology
Rhabdomyosarcoma
Rhabdomyosarcoma - diagnosis
Rhabdomyosarcoma - pathology
Skin - pathology
Tomography, X-Ray Computed
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Summary: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.
ISSN:0385-8146
1879-1476
DOI:10.1016/S0385-8146(02)00005-6