Management of isolated recurrence of extramedullary myeloid tumor at a single site

Extramedullary myeloid tumors (EMMT) can precede, occur with or follow AML. Rarely, they can present as isolated relapses. We present a 9-year-old child with t (8, 21) positive AML who was treated with induction regimen and achieved remission. While on high-dose cytarabine consolidation, he had isolated relapse in a single cervical lymph node with uninvolved marrow. He was treated with salvage chemotherapy alone. There are no clear guidelines for treatment of isolated extramedullary relapse. Chemotherapy, radiotherapy followed by stem cell transplant is the usual option. Our patient is unique for the unusual site of relapse and prolonged remission with cladribine-based chemotherapy, mitoxantrone-based consolidation, and oral maintenance therapy.