Relapse/refractory rhabdomyosarcoma

Abstract

Although the outcome of rhabdomyosarcoma has improved, about one-third of localized rhabdomyosarcoma patients and more than two-thirds of metastatic rhabdomyosarcoma patients still suffer a relapse. In general, the prognosis of recurrent rhabdomyosarcoma is poor and no standard treatment has been established. When deciding on a treatment plan for a recurrent disease, it is important to evaluate the patient with risk factors in mind. The stage and group (primary site, regional lymph node metastasis, tumor size, and distant metastasis) at the time of the first occurrence, the details of the treatment history (chemotherapy, surgery and its curative effect, and radiation dose), and the time of recurrence (during treatment, within 18 months after the start of treatment, or after more than 18 months) should be confirmed. Accurate evaluation of the mode of recurrence (local recurrence, regional recurrence, or metastatic (disseminated) recurrence) is very important, and multiple imaging studies should be performed. Biopsy should be performed aggressively when there are atypical findings of imaging or when a second cancer is suspected. If the recurrent disease is localized, whether it is local or metastatic, an aggressive and multifaceted approach to local therapy should be taken to ensure long-term survival. In contrast, long-term survival in cases of disseminated or multiple recurrence is extremely rare. Chemotherapy is usually administered, but oncogene panel testing and participation in early phase trials may be considered. Minimally invasive treatment and palliative treatment to improve QOL should be considered.