Clinical manifestations of neurofibroma
Almost all cases of skin symptoms can be seen at birth. The shape of milk and coffee spots on the skin is not easy to get to school. Skin tends to occur in unexposed parts of the trunk. There is more than one milk coffee spot before puberty, which has high diagnostic value. Freckles on the whole body and armpits are also one of the characteristics. The large black pigmentation often suggests that the cluster neurofibroma is located in the midline, suggesting that spinal cord tumor dermatofibroma and fibrochondroma are mostly pink in childhood and mainly distributed in the trunk. Moreover, you can also see on your face and face that the number of skin on your limbs is also changeable, and the size can reach thousands of kinds, often from citrus to sesame and mung beans. Soft and elastic, pedicled, superficial neurofibroma is like a movable pearl sister, which can cause pain, tenderness, radiation pain or abnormal sensation. Plexus neurofibroma is often accompanied by massive proliferation of skin and subcutaneous tissue. The second is to cause diffuse hypertrophy in this area or limb, which is called neurofibromatosis.
Symptoms of nervous system: About 50% patients have symptoms of nervous system, which are mainly caused by compression of central or peripheral nerve tumors, followed by gliosis, vascular hyperplasia, skeletal malformation, intracranial tumors, and acoustic neuromas on one or both sides. Most of them can occur in trigeminal nerve and posterior cranial nerve, but they can be combined with multiple meningiomas, gliomas, ependymomas and hydrocephalus. In a few cases, mental retardation, memory disorder and seizures may occur. One or more neurofibromas, meningiomas, etc. It can occur on any plane of the spinal cord, and can be complicated with spinal cord malformation. Peripheral nerve tumors can involve peripheral nerves of the whole body, especially cauda equina, and are distributed in a beaded shape along the nerve trunk. Generally, there are no obvious symptoms, such as sudden growth or severe pain, which can be malignant.
Eye symptoms: fibrochondroma or plexiform neurofibroma can be seen in the upper eyelid, and the orbit touches the mass and the exophthalmos beats. The light shows that the iris has millet-like orange nodules, which are hamartomas, which can increase with age and are unique to neurofibromas. Gray tumor can be seen in the fundus. Optic keratoma with nipple protrusion can lead to exophthalmos and vision loss.
Other common symptoms are congenital bone dysplasia, such as scoliosis, kyphosis, asymmetric defect and depression of skull. Direct compression of tumor can also cause bone changes, such as enlargement of internal auditory canal caused by acoustic neuroma, enlargement of intervertebral foramen caused by spinal neuroma, bone destruction, overgrowth of long bone and sternum, hyperosteogeny of limbs, curvature of spine and pseudoarthrosis, which are more common in adrenal gland, cardiopulmonary digestive tract and mediastinum.
The main feature of neurofibromatosis is bilateral acoustic neuroma, which is often combined with meningioma, astrocytoma and spinal heel neurilemmoma.
Neurofibroma examination
X-rays can find all kinds of skeletal deformities. Myelography CT and MRI are helpful to find brainstem evoked potentials in central nervous system tumors and have important diagnostic value for acoustic neuroma. The genetic analysis department decides the type of mutation.
Treatment of neurofibroma
There is no specific therapy at present. For optic neuroma, acoustic neuroma and other intracranial and intraspinal tumors, surgery should be performed to relieve the compression. Some patients with epilepsy can be treated with antiepileptic drugs, and some patients can be treated with radiotherapy.
The treatment of neurofibroma is now divided into early drug intervention and late plastic surgery.
Childhood neurofibroma is relatively sensitive to drug treatment, which can effectively prevent the tumor from continuing to grow and expand, and as some doctors say, it can't be treated when it grows up.
The late treatment of neurofibroma is mainly aimed at the deformity caused by the tumor itself. Some patients have bone deformities, and the bone deformities should be repaired first, and then the soft tissue deformities should be repaired. In the treatment of such patients, we must be highly alert to massive bleeding, and embolization or other treatments to reduce bleeding must be performed before operation.