Tumors of the Musculoskeletal System

Malignancies may present with musculoskeletal symptoms in the context of primary tumors of bone, fibrous or soft tissue, metastases to bone or leukemia. In the younger child leukemia must always be considered and excluded, however, this is generally not pertinent to the adolescent group. Fortunately metastatic bone disease is very rare. It occurs most commonly in younger children with neuroblastoma (121).

"Red flags" that should alert the clinician to consider this group of disorders include pain quality (e.g., night pain), swelling and tenderness in the absence of trauma, and the presence of systemic features. A brief synopsis of this group of disorders follows. If the reader is considering any of these diagnoses the support of colleagues in radiology and orthopedics with an expertise in this field should be sought.

Benign Tumors

Benign musculoskeletal tumors may occur in the juvenile population. Osteoid osteoma is a tumor of bone that is most common in the second decade of life. It is more common in boys and typically occurs in the neck and intertrochanteric area of the femur. Diagnosis is achieved radio-graphically and treatment is tailored to achieve pain relief. Osteochondroma is the most common tumor of cartilaginous origin. It has an equal sex distribution and occurs more frequently in adolescents (122). It presents as a painless exostotic mass with symptoms from secondary pressure effects. Common sites include the knee and elbow. Other benign musculoskeletal tumors include chondroma, chondroblastoma and chondromyxoid fibroma, juvenile fibromatosis, ossifying fibroma, fibrous dysplasia, and nonossifying fibroma. The latter is relatively common in adolescence and may be confused with malignant pathology.

Benign soft tissue tumors that may present in adolescents include pigmented villonodular synovitis, synovial hemangioma, and synovial chondromatosis. Clinical suspicion of the former is raised by obtaining a bloody knee aspirate.

Malignant Tumors

Malignant musculoskeletal tumors occur relatively commonly in the second decade of life. Osteosarcoma accounts for 60% of all bone tumors in the juvenile population with 75% of cases occurring between 8 and 25 years of age (122). Tumors arise in the metaphyses (60% around the knee) and metastasize early. Even in expert hands the prognosis may be poor with five-year disease free survival of 65% (123). Rhabdomyosarcoma is the most common soft tissue sarcoma in children and is relatively uncommon in adolescents. Tumors arise in striated muscle most commonly in the head and neck area. They may arise in the extremities in about 20% and this tends to be more prevalent in adolescents (122). Ewing's sarcoma is the most malignant of bone tumors. It occurs most frequently in white males in their second decade. Tumors arise in the diaphysis of long bones with a characteristic radiographic appearance. The 5-year survival varies from 50% to 80% depending on the site of the primary tumor and the presence of metastases.

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