Large penile plexiform neurofibroma in an 11-year old boy

摘要

BackgroundNeurofibromatosis is a genetically inherited disorder of the nervous system (brain and spinal cord) which mainly affects the development of nerve (neural) cell tissues, causing tumors (neurofibromas) to develop on nerves. It is the most common single gene disorder of the nervous system and inheritance is through autosomal dominance. They are usually classified into types 1 and 2, the type 1 is the commoner type and also known as superficial neurofibroma. Plexiform neurofibromas are the next most common type of tumor in individuals with type 1 neurofibroma. Plexiform neurofibromas are histologically benign tumors that are made up of a variety of cell types including neuronal axons, Schwann cells, fibroblasts, mast cells, macrophages, perineural cells and extracellular matrix materials such as collagen. They can occur in any part of the body and can grow throughout the person's lifetime, often becoming disfiguring, disabling or deadly via compression of vital structures or conversion to a malignant sarcoma or malignant peripheral nerve sheath turmor. The aim of this report is to present a large penile plexiform neurofibroma which required extensive dissection for complete excision and reconstruction of the phallus and glans penis.