28.11.2020

Classification and manifestations of neurofibromas

Neurofibroma are benign tumors developing from Schwann cells, which are classified as accessory cells of nervous tissue. They are included in the structure of connective tissue and perineurial. Often presents numerous soft tissue structures situated in the subcutaneous tissue. However, the formation of single novoobrazovany, as well as their location in the spinal roots, the soft tissues of various organs.

Due to the growth of this tumor destructive neural sheath. Rarely a benign tumor into a malignant transformirovalsya.

In the ratio of 1:1 occurs in childhood and in the adult organism, and the same ratio and crouches on the gender criterion. In an adult organism arises in 20-30 years.

Often recorded in the elderly, and single tumors and more young.

In magadanlag bodies observed mostly solitary (single) neurofibromas.

Classification

In accordance with a clearly demarcated tumor borders of the tumor determines the forms of:

  • Encapsulated. Is formed in the thickness of large neural trunks. Capsule formation is the sheath of the nerve bundle. Because of the chaotic sprawl of pirovolakis delimited by bundles of collagen, nerve becomes fusiform. Often a favorite location to perform soft tissue, mediastinum and retroperitoneal region. It is clearly defined dense node having a homogeneous fibrous stroenie gray-whitish or grayish color. Such tumors are rarely large quantities (not more than 40 mm).
  • Diffuse (plexiform). Formed in the small nerves in the form of a large number of nodules. It is characterized by a fuzzy boundary. Localization of subcutaneous and cutaneous, but, in some embodiments, is defined in the intermuscular spaces. It is formed elongated limacidae. Sometimes join pigmentosaa and receptors similar to the Taurus Meissner and Vater-Pacini.

Histologically, neurofibromas are divided into:

  • Myxoid, consisting of a large amount of mucin.
  • Plexiform. The basis of it – a large number raznoformatnyh nerve fibers, which are decorated in a matrix of spindle cells, collagen fibers, basophils tissue and mucin.
  • In the composition of the structure similar to tactile corpuscles.
  • Pigment. Perceived outwardly protruding dermatofibrosarcoma.
Causal factors

With all their old primary identification of causal factors to date not fully understood:

On the growth of neoplastic growths is affected by:

  • restructuring in hormonal levels at puberty, during pregnancy or in the post-Natal period;
  • trauma or severe pathology of internal organs;
  • excision of various tumors (neurofibroma, swanna, neuromas) for diagnostic or therapeutic purpose;
  • after conducting some physical treatments;
  • as a result of fractures or correction of different forms of scoliosis;
  • after the massage, in the treatment of neuromuscular disorders.
Symptomatic picture

The pathogenetic mechanism is not fully understood:

  • I type. Formed due to mutation of the gene NF1 located in the region q11.2. 17 chromosome;
  • Type II is a mutation of the 22nd chromosome (region q).

In 50% of cases pathology becomes hereditary (autosomal dominant transmission). In other embodiments, the tumor acts as a result of a spontaneous mutation.

  • type V. M. Riccardi. This later onset of the disease and also develops as the previous type. It is extremely rare.

Of a tumor characterized by slow growth and painless syndrome, therefore, symptomatic presentation depends on the size and location.

The localization in the spine, the patient may complain of pain syndrome during spinal narokoroko. In the plural form due to compression latest developing radiculopathy (sensitivity, mikaboshi, radionovna blunt or acute pain). When large-focal lesions occurs myelopathy with hypocholesterolaemia and a decrease in the amplitude of arbitrary motor acts, pelvic disorders and spasserovannye.

The localization in the internal organs occur functional disorders of the latter.

A large tumor can cause permanent pain with irrationalism in the course of the affected nerve trunk.

In 80% of cases the skin has spot light brown staining, and in the armpits of the tumor similar to freckles. A large number of them – is a sign of neurofibromatosis.

The mixed type has a joint course with tumors of the Central nervous system, developing in the 20-30 years of age.

Type II mostly occurs accompanied by an acoustic neuroma on both sides.

Plexiform can take a giant sized vsledstvie excessive reproduction of cells of the subcutaneous tissue and connective tissue.

Plexiform forms in early childhood presented periorbital swelling.

Diagnosis

Pathology is diagnosed on the basis of:

  • external inspection;
  • Ultrasound, CT and MRI;
  • evaluation of family history (genetic predisposition).
Therapeutic measures

Because of the purity of current treatment is mainly symptomatic.

Excision of the tumor surgically is indicated for:

  • the progression of neurological disorders;
  • the compression of adjacent tissues and organs, as well as when pain syndrome;
  • suspected malignancy;
  • giant quantities;
  • the presence of cosmetic indications.

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