Fibrous Tumors

Lipomatous Tumors

Outcomes

Treatment

Treatment for rhabdomyosarcoma consists of chemotherapy, radiation therapy and sometimes surgery. Surgery to remove the tumor may be difficult or impossible depending on the location of the tumor.

Unresectable tumors should be treated preoperatively with chemotherapy. Reduction of the tumor burden may permit adequate resection thus allowing reduction in radiation therapy.

If there is no evidence of metastasis, surgery combined with chemotherapy and radiation offers the best prognosis. Patients whose tumors have not metastasized usually have a good chance for long-term survival, depending on the subtype of the tumor.

All children with rhabdomyosarcoma are assumed to have microscopic metastasis at the time of diagnosis because hematogenous spread occurs early. This is the rationale for use of chemotherapy in all patients.

Use of vincristine, actinomycin and cylophosphamide (VAC) has proven efficacious in low and intermediate risk tumors. Other drug combinations are being evaluated in patients with poor prognostic categories and include the use of etoposide and ifosfamide.

Radiation therapy plays an important role in local tumor control and its dosage is based upon the site, clinical group and histology of the tumor.

The overall survival rate of children with RMS depends upon site of origin and histology.

About one-third of newly diagnosed patients falls into the low risk group and have a cure rate of 85 to 95%.

The intermediate risk group, which incorporates all patients with gross residual tumors except orbital tumors, has a cure rate of 70 to 80%.

About one-fifth of patients have metastatic disease at the time of diagnosis. Their 5-year survival rate is only 20%.

Benign lipomas, so often seen in adults, are uncommon in children. Infiltrating lipomas and lipoblastomas are the more common benign fatty tumors in children. Complete resection is necessary to prevent recurrence but it may not be feasible in all locations. Liposarcomas are very rare in children and wide resection is essential for cure.

There is a wide spectrum of lesions represented in the category of fibrous tumors. Differentiating benign from malignant can be very difficult. Benign fibroma is a rare small lesion in subcutaneous tissues that is treated with wide local excision. Fibromatoses may appear benign under the microscope but behave aggressively because of their location or invasiveness. They can only be cured by wide local excision; however, mutilating surgery is not recommended unless the tumor becomes "aggressive."

Fibrosarcoma can present at any age. The most common locations are the trunk, extremities, face and neck. The tumor presents as a rapidly growing, firm, painless mass. The primary site is imaged with CT or M RI, combined with metastatic evaluation with chest radiography and chest CT scanning. Complete surgical excision is essential for cure. Chemotherapy and radiation therapy are ineffective.

Lesson 9