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Medicaltalks almost 4 years ago
Hundreds of cutaneous neurofibromas associated with neurofibromatosis!!
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Hundreds of cutaneous neurofibromas associated with neurofibromatosis!!

Neurofibromatosis type 1 is characterized by changes in skin coloring (pigmentation) and the growth of benign tumors along nerves in the skin, brain, and other parts of the body. Cutaneous neurofibromas are benign nerve sheath tumors composed of cells of neuromesenchymal origin, including Schwann cells, fibroblasts, perineurial cells, and mast cells. Multiple cutaneous neurofibromas and their variants (ie, plexiform, dermal) may occur in the setting of neurofibromatosis. These are benign and do not carry an increased risk of malignant transformation, but they often represent a major cosmetic problem. Many have also multiple cafรฉ-au-lait spots, which are flat patches on the skin that are darker than the surrounding area. NF-1 is caused by a mutation of a gene on the long arm of chromosome 17 which encodes a protein known as neurofibromin, which is a negative regulator of the Ras oncogene signal transduction pathway. When Ras isnโ€™t regulated, it is overexpressed. These are a family of proteins that are involved in cellular signal transduction. A cascade effect occurs when ras is โ€œswitched onโ€ by incoming signals, leading to activation of other proteins, which, in turn, activate genes responsible for cell growth and differentiation, hence leading to neurofibromas. Due to their benign nature, neurofibromas should be surgically excised only when symptomatic.

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Top rated comment

What is the prognosis?

almost 4 years ago

Unfortunately only surgical excision๐Ÿ˜ž

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almost 4 years ago

As constant excision clearly isnโ€™t possible is there any sort of treatment?

almost 4 years ago

Poor prognosis...

almost 4 years ago

It's a very serious disease and the prognosis is very bad but.... Kind of looks like tones of testicles....