Rabin J M, Hirschfield L, Badlani G H
Department of Obstetrics and Gynecology, Long Island Jewish Campus for Albert Einstein College of Medicine, New Hyde Park, New York.
Urology. 1991 Dec;38(6):563-6. doi: 10.1016/0090-4295(91)80181-6.
Patients with type IX Ehlers-Danlos syndrome have a tendency for development of diverticulae of the urinary bladder, and these often recur after surgical excision. We report on a patient with this syndrome in whom an extensively infiltrating transitional cell carcinoma developed in a diverticulum. To our knowledge, this is the first reported case of malignancy developing in a patient with type IX Ehlers-Danlos syndrome. The highly aggressive nature of the neoplasm is discussed in relation to the dampened desmoplastic response to the tumor, observed in this patient.
IX型埃勒斯-当洛综合征患者有发生膀胱憩室的倾向,且这些憩室在手术切除后常复发。我们报告了1例患有该综合征的患者,其憩室内发生了广泛浸润性移行细胞癌。据我们所知,这是首例报道的IX型埃勒斯-当洛综合征患者发生恶性肿瘤的病例。本文结合该患者中观察到的对肿瘤的促结缔组织增生反应减弱,讨论了该肿瘤的高度侵袭性。