open access

Vol 21, No 6 (2016)
Published online: 2016-11-01
Submitted: 2015-12-02
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A Kindler syndrome-associated squamous cell carcinoma treated with radiotherapy

Ademar Caldeira, William Correia Trinca, Thais Pires Flores, Andrea Barleze Costa, Claudio de Brito, Karen Loureiro Weigert, Maryana Schwartzhaupt Matos, Carmela Nicolini, Fernando Mariano Obst
DOI: 10.1016/j.rpor.2016.07.004
·
Rep Pract Oncol Radiother 2016;21(6):532-536.

open access

Vol 21, No 6 (2016)
Published online: 2016-11-01
Submitted: 2015-12-02

Abstract

Kindler syndrome[[ce:cross-refs id="crfs0005" refid="bib0125 bib0130"]]1,2 is a genetic disorder mainly characterized by increased skin fragility and photosensitivity,[[ce:cross-refs id="crfs0010" refid="bib0135 bib0140"]]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[[ce:hsp sp="0.25"/]]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.

Abstract

Kindler syndrome[[ce:cross-refs id="crfs0005" refid="bib0125 bib0130"]]1,2 is a genetic disorder mainly characterized by increased skin fragility and photosensitivity,[[ce:cross-refs id="crfs0010" refid="bib0135 bib0140"]]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[[ce:hsp sp="0.25"/]]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.

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Keywords

KS; GTV; CTV; PTV; HDMLC; OAR; PRV; QUANTEC; NTO; RTOG; Kindler syndrome; Head and neck cancer; Radiotherapy; Skin fragility and photosensitivity

About this article
Title

A Kindler syndrome-associated squamous cell carcinoma treated with radiotherapy

Journal

Reports of Practical Oncology and Radiotherapy

Issue

Vol 21, No 6 (2016)

Pages

532-536

Published online

2016-11-01

DOI

10.1016/j.rpor.2016.07.004

Bibliographic record

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

Keywords

KS
GTV
CTV
PTV
HDMLC
OAR
PRV
QUANTEC
NTO
RTOG
Kindler syndrome
Head and neck cancer
Radiotherapy
Skin fragility and photosensitivity

Authors

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

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