Treatment of rhabdomyosarcoma

  Rhabdomyosarcoma is a common soft tissue malignancy in children and adolescents, but can also occur in adults. There are four types of rhabdomyosarcoma histopathologically: embryonal, spindle cell, glandular alveolar and pleomorphic rhabdomyosarcoma.  1. Embryonal rhabdomyosarcoma: It is the most common type of rhabdomyosarcoma, accounting for more than 50-60% of rhabdomyosarcoma, mostly occurring in infants and young children, preferably in the head and neck, genitourinary tract and retroperitoneum. The prognosis of early stage patients of this type is good, and the 5-year survival rate can reach 80%, while the prognosis of late stage is poor.  2. Adenoid rhabdomyosarcoma: It is common in adolescents aged 10 to 20 years old, and is more likely to occur in the extremities, followed by the trunk, perirectum and perineum. It is highly malignant, and the 5-year survival rate is less than 20%.  3.Spindle cell rhabdomyosarcoma: Prevalent in the parametrium and uterus, followed by the head and neck, more males than females, good prognosis, high local recurrence rate after resection, low metastasis rate.  4. Rhabdomyosarcoma multiforme: It is rare and mainly occurs in middle-aged and elderly people, especially in the limbs and thighs. It is highly malignant, prone to recurrence and metastasis after surgery, and has a poor prognosis.  Treatment: Surgical resection, chemotherapy and radiotherapy are the main treatments for rhabdomyosarcoma. Radical surgery alone has a cure rate of less than 25%. Chemotherapy is a systemic treatment for both primary and metastatic lesions, and radiotherapy can result in better control of local lesions. Therefore, comprehensive treatment should be adopted for rhabdomyosarcoma.  1.Surgical treatment Radical surgery is an important means to cure rhabdomyosarcoma, and for early stage (stage I and II), radical surgery should be pursued as early as possible.  Chemotherapy is very important in the treatment of rhabdomyosarcoma, and preoperative chemotherapy and postoperative adjuvant chemotherapy are of great value in preventing recurrence, prolonging life and improving long-term survival rate. In recent years, the long-term survival rate of rhabdomyosarcoma has increased from 10-40% to 60-80% due to the use of chemotherapy.  The VAC regimen (vincristine + actinomycin D + cyclophosphamide) is the basic regimen of chemotherapy for rhabdomyosarcoma, and some other chemotherapy drugs such as adriamycin, isocyclophosphamide, cisplatin, VP16, etc. also have good effect on rhabdomyosarcoma .  Some postoperative recurrent and advanced rhabdomyosarcoma can obtain long-term survival through chemotherapy.  3.Radiotherapy Radiotherapy is a local treatment for rhabdomyosarcoma. For areas where residual tumors cannot be completely removed, radiotherapy can further kill local residual tumor cells after surgery and prevent tumor recurrence.