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患有着色性干皮病患者的口腔内不寻常癌症和意外结果:抗肿瘤治疗的警示。

Unusual intraoral cancer with unexpected outcome in a patient with xeroderma pigmentosum: An alert for antineoplastic treatment.

机构信息

Department of Dentistry, State University of Maringá, Maringá, PR, Brazil.

Department of Dentistry, State University of Maringá, Maringá, PR, Brazil.

出版信息

Oral Surg Oral Med Oral Pathol Oral Radiol. 2020 Jan;129(1):e1-e11. doi: 10.1016/j.oooo.2019.09.017. Epub 2019 Oct 12.

Abstract

Xeroderma pigmentosum (XP) is a rare autosomal disorder characterized by extreme sensitivity to ultraviolet radiation. DNA repair mechanisms are impaired, and minimal sun exposure can lead to the development of cutaneous neoplasms in very young patients. Intraoral carcinomas are uncommon and, when present, are located mainly at the tongue tip. We report an unprecedented case of squamous cell carcinoma (SCC) in the floor of mouth of a 23-year-old woman with XP. The patient was referred to the oncologist, and 2 months after surgical resection, she underwent a single session of chemotherapy plus radiotherapy. However, she died 73 hours after undergoing her first chemotherapy session. Considering the unexpected outcome of this case, we also investigated possible exacerbated adverse effects of antineoplastic treatments (especially cisplatin-based chemotherapy) in patients with XP and reviewed the main characteristics of the disease, especially cases with oral manifestations reported in the literature.

摘要

着色性干皮病(XP)是一种罕见的常染色体疾病,其特征为对紫外线辐射极度敏感。DNA 修复机制受损,即使极少量的阳光暴露也可导致非常年轻的患者发生皮肤肿瘤。口腔内的癌瘤并不常见,而当存在时,主要位于舌腹。我们报告了一例 XP 患者发生的、前所未有的口底鳞状细胞癌(SCC)病例。该患者被转介至肿瘤科医生,在接受手术切除后 2 个月,她接受了单次化疗加放疗。然而,在进行第一次化疗后 73 小时,她死亡。鉴于该病例的意外结局,我们还调查了 XP 患者中抗肿瘤治疗(尤其是基于顺铂的化疗)可能加剧的不良反应,并回顾了该疾病的主要特征,尤其是文献中报告的口腔表现病例。

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