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Volume 21, Number 6, 2016

A Kindler syndrome-associated squamous cell carcinoma treated with radiotherapy

Ademar Caldeira, William Correia Trinca, Thais Pires Flores, Andrea Barleze Costa, Claudio de Sá Brito, Karen Loureiro Weigert, Maryana Schwartzhaupt Matos, Carmela Nicolini, Fernando Mariano Obst

Summary:

Kindler syndrome1,2 is a genetic disorder mainly characterized by increased skin fragility and photosensitivity,3,4 making the use of treatments based on radiation difficult or even prohibited. Thus, cases reporting Kindler syndrome patients treated with radiotherapy are rare. In this study, we report clinical outcomes and care provided for a rare case of a Kindler syndrome patient submitted to radiotherapy. Diagnosed with squamous cell carcinoma involving the buccal mucosa, the patient was exclusively treated with radiotherapy, with 70 Gy delivered on the PTV with the Volumetric Modulated Arc technique. The patient's reaction regarding control of the lesion is relevant compared to patients not affected by the syndrome. We noticed acute reactions of the skin and buccal mucosa after few radiotherapy sessions, followed by a fast reduction in the tumor volume. The efficacy of radiotherapy along with multidisciplinary actions allowed treatment continuity, leading to a complete control of the lesion and life quality improvement and showed that the use of radiotherapy on Kindler syndrome patients is possible.

Signature: Rep Pract Oncol Radiother, 2016; 21(6) : 532-536


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Indexed in: EMBASE®, the Excerpta Medica database, the Elsevier BIOBASE (Current Awareness in Biological Sciences) and in the Index Copernicus.

http://www.sciencedirect.com/science/journal/15071367/19/2