RHABDOMYOSARCOMA
Category: Pediatric Surgery
Abstract : Rhabdomyosarcoma : Most common soft tissue sarcoma in infants and children and represents about 5-15% of all solid malignant lesions. It has a peak incidence at age 2-5 years. Second surge between 10-15 years of age. Tumors of the pelvic organs and head and neck are more prevalent in infancy and early childhood, while the paratesticular rhabdomyosarcomas are largely a disease of adolescents and yo
Rhabdomyosarcoma : Most common soft tissue sarcoma in infants and children and represents about 5-15% of all solid malignant lesions. It has a peak incidence at age 2-5 years. Second surge between 10-15 years of age. Tumors of the pelvic organs and head and neck are more prevalent in infancy and early childhood, while the paratesticular rhabdomyosarcomas are largely a disease of adolescents and young adults.
Although classically described as occurring in striated muscle, rhabdomyosarcomas arise from a primitive cell type and occur in mesenchymal tissue at almost any body site (possibly excluding the brain), including many organs that normally do not have striated muscle. The predominant histologic type in infants and small children is embryonal. The botryoid rhabdomyosarcoma is a subtype of the embryonal variety, which ordinarily extends into body cavities such as the bladder, nasopharynx, vagina, or bile duct. The alveolar cell type, named for a superficial resemblance to the pulmonary alveoli, is the most common form found on the muscle masses of the trunk and extremities, and is seen more frequently as age advances.
The clinical findings, diagnostic evaluation and therapy employed are dependent upon the location of the primary tumor and is beyond the scope of this review. In brief, head and neck tumors are most common and occur in the orbit, nasopharynx, cheek, neck, middle ear, larynx and paranasal sinuses. Most are treated by simple biopsy followed by combined therapy or preoperative chemotherapy and radiation followed by conservative resection. Operations for extremity lesions include wide local excision to remove as much of the gross tumor as possible. Rhabdomyosarcomas can arise from the bladder, prostate, uterus, or vagina. The trend in treatment is more chemotherapy and conservative surgical management.
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