Neurofibroma

Description:

Neurofibroma — a benign tumor from elements of an endoperineurium, schwannian cells; it is connected with covers of peripheral nerves in skin, hypodermic fatty tissue, soft tissues, a mediastinum, with roots of a spinal cord.

Frequency of development of a tumor — 1 on 3 — 4 thousand population. The neurofibromatosis of the I type is shown since the neonatality period to 5-year age. The neurofibromatosis of the II type is shown at the age of 10 — 20 years. Family cases are diagnosed for 40 — 50% of patients.

Neurofibroma symptoms:

Tumors are localized on a trunk, extremities, is more rare on a face and a neck.
Clinical options of a neurofibromatosis of the I type (according to V. V. Mordovtseva, 1995):
• with domination of neurofibromas and a small nevus pigmentosus — large and small like freckles;
• with a large nevus pigmentosus and not numerous neurofibromas;
• with a nevus pigmentosus like freckles in combination with large spots without or with single neurofibromas;
• the mixed option.
Nevus pigmentosus more than 0,5 cm and plexiform neurofibromas arise since the birth or by the end of the 1st year of life. Knotty elements develop in an interval of 10 — 20 years.

Neurofibroma reasons:

The neurofibromatosis of the I type is caused by the mutant autosomal and dominant gene localized in a chromosome 17, having 100% penetrance. The neurofibromatosis of the II type is caused by a mutant gene in an autosome 22.

Treatment of the Neurofibroma:

Solitary tumors depending on the size subject to electroexcision, surgical excision.
At multiple tumors appoint synthetic retinoids:
Atsitretin in 50 — 75 mg 1 р / days, 4 — 11 weeks.

Efficiency of treatment is confirmed by data of a clinical and laboratory trial, including data on growth and the sizes of a tumor, an involvement of other fabrics and existence of metastasises (at malignant tumors), recurrent tumors after its removal.