Is neurofibroma a tumor?

summary

Neurofibromatosis is an autosomal dominant genetic disease, which is caused by the abnormal development of neural crest cells due to gene defects, resulting in multi system damage. According to clinical manifestations and gene location, neurofibromatosis was divided into type I (NFI) and type II (NF Ⅱ). The main features are skin milk coffee spots and peripheral nerve multiple neurofibroma. Is neurofibroma a tumor? Let's talk about it

Is neurofibroma a tumor?

In almost all cases, milk coffee spots were found on the skin at birth, with different shapes and sizes, irregular edges and no protruding skin surface, and most of them occurred on the non exposed parts of the trunk; More than 6 skin milk coffee spots > 5mm before puberty (more than 15mm after puberty) have high diagnostic value, and systemic and axillary freckles are also one of the characteristics.

Dermatofibroma and fibrochondroma occur in childhood, mainly in the trunk and face skin, but also in the extremities. Most of them are pink in color, and the number is variable. They can be several thousand in size and vary in size. Most of them are the size of sesame, mung bean to orange, and are soft in texture; Soft tumor is fixed or pedicled, soft and elastic to touch; The neurofibroma of superficial epidermal nerve is like a bead like nodule, which can move and cause pain, tenderness, radiation pain or paresthesia; Plexiform neurofibroma is a diffuse neurofibroma of the nerve trunk and its branches, often accompanied by a large number of hyperplasia of skin and subcutaneous tissue, causing diffuse hypertrophy of the region or limbs, known as neurofibromatous elephantiasis.

Intracranial tumors acoustic neuroma is the most common, bilateral neuroma is the main feature of NF Ⅱ, often combined with meningeal meningioma, multiple meningioma, glioma, ependymoma, meningocele and hydrocephalus, spinal nerve posterior root neurilemmoma, optic nerve, trigeminal nerve and posterior group of cranial nerve can occur, a few cases may have mental retardation, memory impairment and seizures;

matters needing attention

The disease is an autosomal dominant disease, and 50% of the children may have the disease, so sterilization should be considered. At the same time, we should pay attention to self-monitoring. If the tumor is found to increase rapidly in a short time, there may be malignant transformation. Surgical treatment is needed when severe complications occur, such as intracranial tumor, hemorrhage and intestinal obstruction caused by gastrointestinal involvement, or visceral pressure caused by huge retroperitoneal neurofibroma.