title: A Kindler syndrome-associated squamous cell carcinoma treated with radiotherapy.
abstract: 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.
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Ademar
authors.initials: A
authors.lastname: Caldeira
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: William Correia
authors.initials: WC
authors.lastname: Trinca
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Thais Pires
authors.initials: TP
authors.lastname: Flores
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Andrea Barleze
authors.initials: AB
authors.lastname: Costa
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Claudio de Sá
authors.initials: Cde S
authors.lastname: Brito
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Karen Loureiro
authors.initials: KL
authors.lastname: Weigert
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Maryana Schwartzhaupt
authors.initials: MS
authors.lastname: Matos
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Carmela
authors.initials: C
authors.lastname: Nicolini
authors.affiliation: Institute of Cancer, Hospital Mãe de Deus, Orfanotrofio Str, 299, 90840-440 Porto Alegre, Brazil.
authors.forename: Fernando Mariano
authors.initials: FM
authors.lastname: Obst
keywords: CTV, clinical tumor volume; GTV, gross tumor volume; HDMLC, high definition multileaf collimator; Head and neck cancer; KS, Kindler syndrome; Kindler syndrome; NTO, normal tissue optimization; OAR, organs at risk; PRV, planning risk volume; PTV, planning tumor volume; QUANTEC, quantitative analysis of normal tissue effects in the clinic; RTOG, Therapy Oncology Group; Radiotherapy; Skin fragility and photosensitivity