Friday, June 16, 2023

Infantile fibromatoses.. Increased growth of fibrous tissue forming a belief in subcutaneous tissue and bones, especially the head, neck and trunk



Infantile fibromatoses:

Fibroblastoma is a very rare connective tissue disease that affects infants and may be present at birth and continues to appear for several years.

Clinical manifestations:

The lesions result from the growth of the fibrous tissue forming a belief in subcutaneous and bone tissue, especially the head, neck and trunk.
Visceral nodules may affect the liver, heart, and lungs. The nodules may be rubbery or stiff and may be superficial or deep, but if the gut is damaged, the baby will usually die within several months.

Treatment:

The combination of the chemical treatment of Fincristin and dicitomycin d and cyclophosphamide may yield good results.

In infants, fibroblastic fibrosis is often congenital (often referred to as congenital fibroblastic sarcoma) and usually occurs in babies in their first two years of life.

On the cellular side, congenital fibroblastic sarcoma is characterized by a majority of cases in which a transition occurs between chromosomes 12 and 15, leading to the formation of the ETV6-NTRK3 fusion gene, with the chromosome trigonometry of 8, 11, 17 or 20 in some individual cases.

Histology is similar to ETV6-NRTK3, in addition to some chromosomal trigonometry, and the distribution of markers to the cell type within the tumor, with what is present in the cytoplasmic cytoskeletal morphology.

Indeed, they appear to be the same disease except that the erythrocyte lymphoma originates in the kidney while the congenital fibroblastic sarcoma arises in the renal tissues.