Clinical Remission of Xeroderma Pigmentosum-Associated Squamous Cell Carcinoma with Isotretinoin and Chemotherapy: Case Report

Abstract

We report the case of a 7-year old boy with xeroderma pigmentosum and a large squamous cell carcinoma of the cheek. He received a combination of isotretinoin (1 mg/kg/day) and chemotherapy for a period of 3 months and showed complete remission of the tumor. Treatment modalities of malignancies in xeroderma pigmentosum are reviewed and discussed in relation to the literature. The advantages of our protocol were emphasized because of the rapid improvement in a short time with minimal side effects.

Retinoic acid effects on thyroid function of female rats

“…Retinoids and their active metabolites, including retinoic acid (RA), are essential for morphogenesis, differentiation, and homeostasis (Giguere 1994;Means and Gudas 1995;Ross et al 2000), and are effective therapeutic and chemopreventive agents for several cancers (Lippman and Meyskens 1987;Saad et al 1999;Li et al 2000). Retinoid effects are mediated by two types of nuclear receptors: retinoic acid receptor -RAR and retinoid X receptor -RXR, which in turn are divided in three subtypes: α, β and γ (Giguere et al 1987;Giguere 1994;Chambon 1996).…”

Anestesia em paciente com Xeroderma Pigmentoso: relato de caso

“…A profilaxia baseia-se principalmente na proteção do paciente contra a luz solar, através do uso de protetores solares, óculos e roupas que impeçam a penetração dos raios ultravioleta 16 . A cirurgia e a eletrocoagulação, assim como o emprego do 5-fluorouracil, dos retinóides e a imunoterapia são algumas opções de tratamento disponíveis, mas nenhuma delas é completamente eficaz, além da ocorrência dos diversos efeitos colaterais, devendo-se, portanto, considerar os benefícios do seu uso [17][18][19][20] . A excisão completa dos tumores malignos deve ser realizada sempre.…”

“…Prevention is primarily based on protecting patients against sunlight with sun protectors, glasses and clothes preventing UV rays penetration 16 . Surgery and electrocoagulation, in addition to 5-FU, retinoids and immunotherapy are some available options, but none of them is fully effective, and the presence of several side-effects may limit the benefits of such therapies [17][18][19][20] . Malignant tumors should always be totally excised.…”

Anaesthetic Management of Siblings having Xeroderma Pigmentosum: A Rare Presentation

“…Prophylaxis is based primarily on the patient's protection from sunlight, through the use of sun screens, sunglasses and clothes that prevent the penetration of ultraviolet rays [9]. Surgery and electro coagulation, as well as the use of 5-fluorouracil, retinoids and immunotherapy are few treatment options are available, but none is completely effective [10][11][12][13][14].…”