Bone and Soft-Tissue Disorders
= CHLOROMA = Myeloid sarcoma = MYELOBLASTOMA
= uncommon extramedullary solid tumor consisting of primitive precursors of the granulocytic series of WBCs (myeloblasts, promyelocytes, myelocytes)
Peak age: 78 years; child >adult; M=F
Myeloid sarcoma is a rare extramedullary proliferation of immature myeloid cells occurring in 35% of AML patients.
Clinical setting:
- patient with acute myelogenous leukemia (in 38%)
- harbinger of AML in nonleukemic patient: usually developing within 1 year (rare)
- indicator of impending blast crisis in CML (in 1%) / leukemic transformation in myelodysplastic syndromes (polycythemia rubra vera, myelofibrosis with myeloid metaplasia, hypereosinophilic syndrome)
- during remission of hematologic malignancy (up to 20%)
- isolated event
Associated with: chronic myeloid leukemia, myelodysplastic syndrome, essential thrombocythemia, polycythemia vera
- 60% are of green color (chloroma) ← high levels of myeloperoxidase (30% are white / gray / brown depending on preponderance of cell type + oxidative state of myeloperoxidase)
Location: often multifocal
- common: bone (skull, orbit, paranasal sinus, periosteum); lymph nodes; soft tissues; skin; breast
- less common: GU tract, GI system, head & neck, chest
Site: propensity for bone marrow (arises from bone marrow traversing haversian canal + reaching the periosteum), perineural + epidural tissue
Radiography:
- osteolysis with ill-defined margins
CT:
- soft-tissue mass infrequently with bone erosion / demineralization / periosteal reaction
- generally homogeneously isoattenuating to slightly hyperattenuating relative to muscle / brain
- homogeneous enhancement on CT / MR (DDx to hematoma / abscess)
MR:
- iso- to hypointense relative to gray matter / bone marrow / muscle on T1WI
- heterogeneously iso- to slightly hyperintense on T2WI
Prognosis: poor outcome; resolution under chemotherapy ± radiation therapy; recurrence rate of 23%
DDx: osteomyelitis, histiocytosis X, neuroblastoma, lymphoma, multiple myeloma