12.2.12Prognosis

•General event-free survival is 20–80%, depending on stage, in comparison with the mean of 24% before the chemotherapy and radiotherapy

•Before multidisciplinary treatment approaches, disease spread became evident in the majority of affected children within the 1st year after diagnosis

•Prognosis according to stage (see above)

•Prognostic factors include: tumor site, as well as size and extension after surgery

•Favorable prognosis in tumors of orbit or genitourinary tract (exception is the prostate): early stage

•Moderate to poor prognosis:

–Extremities: early metastases, frequently histological signs in the alveoli

–Retroperitoneal site – often late diagnosis and large tumor

•Variable prognosis in rhabdomyosarcoma of the head and neck area

•Poor prognosis with involvement of CNS

•Unfavorable prognosis in children with alveolar and pleomorphic histology; both are associated with a high rate of local relapse and metastases

•Age:

–More favorable prognosis in children less than 7 years old

–In patients more than 7 years of age, disease is more frequently of advanced stage and alveolar in histology

12.2.13Therapy and Prognosis in Nonresponding or Relapsing Rhabdomyosarcoma

•In nonresponding rhabdomyosarcoma, combination therapy with topotecan, vinorelbine, taxol, and irinotecan has been used

•The majority of relapse occurs within 2–3 years after diagnosis

•In patients with local relapse, standard chemotherapy plus ifosfamide, doxorubicin, etoposide, or other cytotoxic agents (see above)

•In children with disseminated relapse, poor prognosis

•Experimental procedure:

–Double high-dose therapy with autologous stem-cell transplantation

–Allogeneic stem-cell transplantation with the possibility of antitumor effects caused by the donor’s immune response (graft-versus-tumor effect)