Recommendations

Recommendations

Confirmation of the diagnosis is absolutely required. Central pathology review is performed for all cases as part of enrollment on the Registry.

Once the diagnosis is established, treatment decisions must be made by patients and treating providers taking into account the individual clinical circumstances. If feasible, complete resection is suggested. For unresectable disease, radiation therapy and chemotherapy are often used. Although initial responses are not uncommon, durable responses are rare, however we are recommending that ifosfamide-based regimens be used upfront when feasible, and evaluation for PDL1 expression be considered for possible addition of an immune checkpoint inhibitor. Registry personnel are available for consultation on an individual case basis.