(Chloroma, primary extramedullary leukemia)
DEFINITION AND PATHOGENESIS
- Extramedullary tumor of malignant myeloid precursor cells
IMPORTANCE
- Associated with AML in 3-8% of cases at presentation or relapse (heralds a blastic crisis)
- Dx is commonly missed (up to 75% reported) (prompt dx and tx improves prognosis)
- May also be associated with another myeloproliferative disorder
CLINICAL FEATURES
- Pain
- ± eosinophilia and leukocytosis
- Wide age distribution
RADIOLOGIC FEATURES
- Lytic permeative metaphyseal lesion (sclerosis is rare) characteristic on plain radiographs
- Solitary bone involvement reported but rare, ± soft tissue involvement (more common)
- CT may show mixed lysis and sclerosis
GROSS PATHOLOGY
- A minority of tumors will have a green hue, probably due to myeloperoxidase within the cells
- Dense fibrous tissue
HISTOLOGIC FEATURES
- Large, neoplastic cells, moderate to abundant cytoplasm, irregular nuclear bodrders, vesicular nuclei, ? 1 nucleoli
- ± eosinophilic myelocytes (~50%)
- “Starry sky pattern” often present
- + CD 45
- + CD 30
- + myelopreroxidase
- + naphthol chloraetate esterase
- S-100
- EMA
- EM:
- Cytoplasmic membrane-bound vesicles
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Large cell lymphoma
- Metastatic neuroblastom
- Ewing sarcoma
- Metastasis
- Plasmacytoma
DISEASE COURSE AND TREATMENT
- Progression to AML within 6-7 months often occurs
- Bone marrow aspirate to R/O AML
- Bone scan, skeletal survey to R/O other sites of involvement
- Median survival 13-14 months with surgery + XRT
- Chemotx with 2-yr survival > 50%
REFERENCES
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