کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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3167987 | 1199387 | 2009 | 5 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: A 19-year follow-up of a patient with type 3 ectrodactyly–ectodermal dysplasia–clefting syndrome who developed non-Hodgkin lymphoma A 19-year follow-up of a patient with type 3 ectrodactyly–ectodermal dysplasia–clefting syndrome who developed non-Hodgkin lymphoma](/preview/png/3167987.png)
The ectrodactyly–ectodermal dysplasia–clefting (EEC) syndrome is characterized by ectrodactyly, ectodermal dysplasia, and clefting. The development of a malignancy with EEC syndrome is very rare. Here we present follow-up on a Turkish boy with EEC syndrome type 3 who developed malignant lymphoma with high expression of p63. He had chronic renal failure due to recurrent urinary infections caused by ureterovesical reflux. Cervical, diffuse, large, B-cell non-Hodgkin lymphoma with high expression of p63 was diagnosed, and the patient died at 19 years of age. The transcription factor p63 is a key regulator of ectodermal, orofacial, and limb development. Mutations in the p63 gene can cause syndromes of ectodermal dysplasia, ectrodactyly, and orofacial clefting. Malignant lymphoma is a very rare complication of EEC syndrome. We suggest that p63 gene mutation analysis should be performed in every EEC syndrome patient with the possibility of developing malignant tumors.
Journal: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology - Volume 108, Issue 3, September 2009, Pages e91–e95