Kozarek R A, Sanowski R A
J Clin Gastroenterol. 1981 Jun;3(2):171-4. doi: 10.1097/00004836-198106000-00012.
Fanconi's anemia, an inherited disorder characterized by bone marrow aplasia, peripheral pancytopenia, and multiple congenital defects, has a association with certain types of malignancies. These include acute myelomonocytic leukemia, benign and malignant liver tumors, and squamous carcinomas of various organs. We report a patient with esophageal carcinoma complicating Fanconi's anemia, summarize the various tumors associated to date with the syndrome, and discuss possible mechanisms of malignant transformation. This high incidence of malignancy must be appreciated for early diagnosis and appropriate therapy.
范科尼贫血是一种以骨髓再生障碍、外周血细胞减少和多种先天性缺陷为特征的遗传性疾病,与某些类型的恶性肿瘤有关。这些肿瘤包括急性粒单核细胞白血病、良性和恶性肝肿瘤以及各器官的鳞状癌。我们报告了一例患有范科尼贫血并发食管癌的患者,总结了迄今为止与该综合征相关的各种肿瘤,并讨论了恶性转化的可能机制。必须认识到这种高恶性肿瘤发生率以便进行早期诊断和适当治疗。