title: Outcomes of patients with POEMS syndrome treated initially with radiation.
abstract: POEMS syndrome is a rare plasma cell dyscrasia presenting with polyneuropathy and other systemic findings. Patients with 1 to 3 bone lesions and negative bone marrows are often treated with involved field radiation therapy as the initial and potentially definitive therapy. Long-term outcomes of patients treated with this approach have not been systematically studied. Of the 146 patients with POEMS syndrome seen at the Mayo Clinic between January 1999 and September 2011, 38 (26%) were given targeted radiation as their initial primary therapy and are the ones studied here. The median number of bone lesions was 1 (range: 1-6). The median dose of radiation administered was 45 Gy (range: 35-54 Gy). Complete or partial hematologic, vascular endothelial growth factor, fluorodeoxyglucose-positron emission tomography, and clinical responses were documented in 31%, 14%, 22%, and 47%, respectively. With median follow-up of 43 months, the 4-year overall survival is 97% and event-free survival is 52%. Risk factors for needing salvage therapy included reduced pulmonary diffusing capacity of carbon monoxide and increased urinary total protein. The presence of 3 lesions compared with 1 or 2 did not increase risk for treatment failure. Among selected patients with POEMS syndrome, radiation produces durable, meaningful responses.
authors.affiliation: Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Humeniuk
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Witzig
authors.forename: Shaji K
authors.initials: SK
authors.lastname: Kumar
authors.forename: Prashant
authors.initials: P
authors.lastname: Kapoor
authors.forename: John A
authors.initials: JA
authors.lastname: Lust
authors.forename: Suzanne R
authors.initials: SR
authors.lastname: Hayman
authors.forename: Francis K
authors.initials: FK
authors.lastname: Buadi
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
authors.forename: Steven R
authors.initials: SR
authors.lastname: Zeldenrust
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Russell
authors.forename: David
authors.initials: D
authors.lastname: Dingli
authors.forename: Yi
authors.initials: Y
authors.lastname: Lin
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D059248:Chemoradiotherapy; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D019650:Hematopoietic Stem Cell Mobilization / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000401:mortality* / Q000532:radiotherapy*; D011878:Radiotherapy / Q000379:methods*; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A