Rhabdomyosarcoma

Rhabdomyosarcoma– a malignant tumor originating from the skeletal (striated) muscle; in adults, it is characterized by poorly differentiated oblong or round, bizarrely shaped cells with large brightly colored nuclei; more common in babies. There are embryonic (develops up to 15 years) and adult types of rhabdomyosarcoma. Frequency. Takes 3rd place among malignant soft tissue neoplasms. Pathomorphology. The tumor consists of spindle-shaped or rounded cells, in the cytoplasm of which longitudinal and transverse striation is determined. Genetic aspects. In the development of rhabdomyosarcomas, the participation of several genes located on chromosomes 1,2,11, 13 and 22 is assumed; the possible role of genomic imprinting or duplication of individual genes is considered (for example, the gene for insulin-like growth factor-2 IGF2[147470],

Clinical picture

- Most often, tumors are localized in three anatomical regions of the body; limbs, head and neck, small pelvis
- Tumors grow rapidly, without pain and

dysfunction

- It is not uncommon to sprout the skin with the formation of exophytic bleeding tumors.

Treatment is surgical; for large tumors, preoperative radiation therapy is advisable.

- When conducting combined (surgical, chemotherapy) treatment of localized forms of embryonic rhabdomyosarcoma, an increase in 5-year survival up to 70% is noted. In the presence of metastases, the 5-year survival rate is 40%
- For pleomorphic rhabdomyosarcoma (adult tumor), the 5-year survival rate is 30%.

Synonyms

- rhabdosarcoma
- Rhabdomyoblastoma
- Malignant rhabdomyoma

MSH

- Tumor rhabdoid (601607)
- Alveolar rhabdomyosarcoma (268220, FKHR gene, 268220 PAX3 homeotic gene; 268220 PAX gene
- Rhabdomyosarcoma (268210)