What does a plexiform neurofibroma look like?

What does a plexiform neurofibroma look like?

These are benign tumors that develop along a nerve on or under the skin. They may look like small lumps, bumps, or nodules. Cutaneous neurofibromas may develop at any time of life, but also seem to increase in number during times of hormone changes, such as during puberty or, for women, during pregnancy.

Where do plexiform neurofibromas appear?

Plexiform neurofibromas are the most complex type of neurofibroma and can grow within long portions of one nerve or bundles of nerves. They may be deep inside the body and identified only with medical imaging scans—for example, an MRI scan—or they can be near the skin surface and on the arms, legs, head, and neck.

How common is plexiform neurofibroma?

Plexiform neurofibromas occur in up to 30% of cases of NF-1, most frequently in the craniomaxillofacial region.

Do plexiform neurofibromas grow?

Plexiform neurofibromas can grow. They are more likely to grow in childhood but they can also grow in adult years. There is no evidence that they get worse with puberty.

When do axillary freckles appear?

The majority of affected children will show axillary or inguinal freckling by the age of 2, and by 11 years most children will have 2 or more neurofibromata of any type or 1 plexiform neurofibroma (slow-growing tumours that involve multiple nerves and fascicles).

Why do cafe au lait spots appear?

Café au lait spots are caused by an increase in melanin content, often with the presence of giant melanosomes. A significant increase in melanocyte density is noted in the café au lait macules of patients with NF1 compared with patients who have isolated café au lait macules without NF1 involvement.

Is plexiform neurofibroma curable?

How are plexiform neurofibromas treated? Until recently, the only known effective treatment for plexiform neurofibromas has been surgery. Approximately 75% of patients who undergo a complete removal of the tumor without causing significant neurologic impairment or dysfunction are cured.

Are plexiform neurofibromas?

Nerve tumors known as neurofibromas are benign or non-cancerous tumors that grow on nerves throughout the body. While they are seen in people without NF1, the presence of two or more of these tumors should raise the suspicion of NF1. There are three major types of neurofibroma: cutaneous, spinal and plexiform.

What causes plexiform neurofibroma?

Neurofibromatosis is caused by genetic defects (mutations) that either are passed on by a parent or occur spontaneously at conception. The specific genes involved depend on the type of neurofibromatosis: NF1 . The NF1 gene is located on chromosome 17.