title: Features of extramedullary myeloma relapse: high proliferation, minimal marrow involvement, adverse cytogenetics: a retrospective single-center study of 24 cases.
abstract: Extramedullary (e) relapse in multiple myeloma(MM) has an adverse prognosis, but knowledge concerning biological features and preferred treatment is scarce. We screened the myeloma registry of our institution for eMM relapses and identified 24 cases among 357 patients (pts).Only 8% of eMM relapses occurred after initial therapy, but 54% occurred after third-line or subsequent therapy. Baseline molecular cytogenetics revealed high-risk features in 10 of 19 evaluable patients. Most frequently, eMM presented as soft tissue (67%) and organ involvement (25%) or malignant effusion (12.5%). Incidence of leptomeningeal/CNS involvement was 21%. At eMM relapse, bone marrow infiltration was absent in 46% and low in 21%. Ten eMM biopsies were available showing increased proliferation, i.e., Ki-67 of 67%(range, 30–90%) of all cancer cells. Pts received radiation therapy, dose-intense chemotherapy, novel agents, and allogeneic SCT resulting in an overall response rate of 54%. Median progression-free survival was 2 (95% CI 0.08–3.92) and median overall survival 7 months (95% CI 3.56–10.43), respectively,with only three patients being alive at 12 months from diagnosis. EMM relapse may present at any anatomical site with frequent CNS involvement. Biological features include increased proliferation and low rate of marrow involvement.Prognosis remains poor despite intensive treatment.
authors.affiliation: Department of Hematology and Oncology, University Hospital Wuerzburg, Oberdürrbacher Str. 6, 97080 Wuerzburg, Germany. rasche_l@medizin.uni-wuerzburg.de
authors.forename: Leo
authors.initials: L
authors.lastname: Rasche
authors.forename: Corinna
authors.initials: C
authors.lastname: Bernard
authors.forename: Max S
authors.initials: MS
authors.lastname: Topp
authors.forename: Markus
authors.initials: M
authors.lastname: Kapp
authors.forename: Johannes
authors.initials: J
authors.lastname: Duell
authors.forename: Carmen
authors.initials: C
authors.lastname: Wesemeier
authors.forename: Eugenia
authors.initials: E
authors.lastname: Haralambieva
authors.forename: Uwe
authors.initials: U
authors.lastname: Maeder
authors.forename: Hermann
authors.initials: H
authors.lastname: Einsele
authors.forename: Stefan
authors.initials: S
authors.lastname: Knop
mesh_terms: D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology*; D049109:Cell Proliferation; D015331:Cohort Studies; D020732:Cytogenetic Analysis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D012008:Recurrence; D012042:Registries; D012189:Retrospective Studies; D016019:Survival Analysis