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Large penile plexiform neurofibroma in an 11-year old boy
Collins, Nwokoro Chingbundu; Ayodeji, Emmanuel Ezekiel; Motunrayo, Fatungase Oluwabunmi; Abayomi, Salami Babatunde; Olufemi, Ogundele Ibukunolu & Olusesan, Amosu Lukmon
Abstract
Background Neurofibromatosis 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.
Objectives
To present a huge penile plexiform neurofibroma and the mode of surgical treatment.
Methods The huge penile plexiform neurofibroma was completely excised and the penile defect resulting from the excision was repaired.
Conclusion
Plexiform neurofibromas are congenital tumors of peripheral nerve sheaths which may also develop near nerve roots deep within the
body. They are usually benign but carry a malignant potential in 5-10% of patients. Plexiform neurofibromas are commoner in the face,
chest and limbs but the index case occurred on the penile shaft.
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