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与范科尼贫血相关的外阴鳞状细胞癌:一例报告。

Squamous cell vulvar carcinoma associated with Fanconi's anemia: a case report.

作者信息

Carvalho J P, Dias M L Nogueira, Carvalho F M, Del Pilar Estevez Diz M, Petito J W

机构信息

Departments of Gynecology and Pathology, Clinical Oncology Service and Radiotherapy Service, University of São Paulo School of Medicine, São Paulo, Brazil.

出版信息

Int J Gynecol Cancer. 2002 Mar-Apr;12(2):220-2. doi: 10.1046/j.1525-1438.2002.01090.x.

Abstract

Fanconi's anemia (FA) is a rare autosomal recessive syndrome associated with a strong predisposition to cancer, particularly squamous cell carcinoma (SCC) of various organs. A few cases of lower genital tract neoplasia have been described. We present a 14-year-old black girl with an advanced squamous cell vulvar carcinoma treated with cisplatin chemotherapy plus radiation therapy. The patient died because of fungal sepsis. Polymerase chain reaction (PCR) was positive to human papillomavirus (HPV)-16. Vulvar carcinoma is a very rare condition in teenagers, but the association of Fanconi's anemia and SCC of many sites is common. Vulvar carcinoma when associated with Fanconi's anemia is a great treatment challenge.

摘要

范可尼贫血(FA)是一种罕见的常染色体隐性综合征,与患癌易感性密切相关,尤其是各器官的鳞状细胞癌(SCC)。已有少数下生殖道肿瘤形成的病例报道。我们报告了一名14岁的黑人女孩,患有晚期外阴鳞状细胞癌,接受了顺铂化疗加放射治疗。患者因真菌败血症死亡。聚合酶链反应(PCR)检测显示人乳头瘤病毒(HPV)-16呈阳性。外阴癌在青少年中非常罕见,但范可尼贫血与多个部位的鳞状细胞癌相关的情况很常见。范可尼贫血合并外阴癌是一个巨大的治疗挑战。

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