Giant plexiform neurofibroma in a patient with neurofibromatosis type I.
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder primarily affecting the development and growth of nerve cell tissues. Plexiform neurofibroma is considered an uncommon skin tumor. The involvement of the genitourinary tract or the lower limb is rare, with bladder, upper urinary tract and genital involvement reported in decreasing order of frequency. The management of patients with plexiform neurofibroma is not well defined and aiming mostly at controlling symptoms. We present a case of a giant genitourinary plexiform neurofibroma associated with lower limb gigantism in a patient with NF1.
Rallis
Efstathios
E
Department of Dermatology, Army General Hospital, Athens, Greece. efrall@otenet.gr
Ragiadakou
Dimitra
D
D002081:Buttocks / Q000473:pathology*; D004200:Diseases in Twins; D006801:Humans; D007866:Leg / Q000473:pathology*; D008297:Male; D009361:Neoplasm Invasiveness; D018318:Neurofibroma, Plexiform / Q000235:genetics / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology*; D012611:Scrotum / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology*; D014565:Urogenital Neoplasms / Q000235:genetics / Q000473:pathology*; D055815:Young Adult