Treatment of ectomesenchymoma

Treatment options for ectomesenchymoma include the following:

The Cooperative Weichteilsarkom Studiengruppe (CWS) reported on six patients (aged 0.2–13.5 years) registered over 14 years.[3][Level of evidence C1] The tumors were located in various sites, including the extremities, abdomen, and orbit. All six patients were treated with surgery and chemotherapy directed at rhabdomyosarcoma. Two patients received radiation therapy. Three patients experienced tumor recurrences with rhabdomyosarcoma features. Although data are scant, it appears that the tumor may respond to chemotherapy.[3]

The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) identified ten patients with ectomesenchymoma in a prospectively recorded database.[4] Of the ten cases, seven had an initial local diagnosis of rhabdomyosarcoma. All patients received chemotherapy according to rhabdomyosarcoma strategy, and four patients received radiation therapy. Overall, six patients were alive in first remission, two in second remission, and one after treatment for a second primary cancer. Only the patient with a metastatic tumor at diagnosis died of their disease.