Type IX Ehlers-Danlos syndrome: bladder diverticula with transitional cell carcinoma.
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.
Rabin
J M
JM
Department of Obstetrics and Gynecology, Long Island Jewish Campus for Albert Einstein College of Medicine, New Hyde Park, New York.
Hirschfield
L
L
Badlani
G H
GH
D002295:Carcinoma, Transitional Cell / Q000150:complications* / Q000453:epidemiology; D004240:Diverticulum / Q000150:complications* / Q000453:epidemiology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D001745:Urinary Bladder Diseases / Q000150:complications* / Q000453:epidemiology; D001749:Urinary Bladder Neoplasms / Q000150:complications* / Q000453:epidemiology