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POEMS
Behrang Amini, MD, PhD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Osteosclerotic myeloma, Crow-Fukase syndrome, plasma cell dyscrasia, endocrinopathy, polyneuropathy (PEP) syndrome, Takatsuki syndrome
        • Definitions

          • Rare paraneoplastic syndrome due to underlying plasma cell disorder
          • Old acronym defines some features: polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS)
          • Diagnosis requires following
            • Both mandatory criteria
              • Polyneuropathy
              • Monoclonal plasma cell proliferative disorder
            • At least 1 major criterion
              • Sclerotic or mixed sclerotic/lytic myelomatous bone lesion on radiography or CT
              • Castleman disease (giant cell lymph node hyperplasia, angiofollicular lymph node hyperplasia)
              • Elevated serum or plasma vascular endothelial growth factor (VEGF) levels (≥ 3x upper limit of normal)
            • At least 1 minor criterion
              • Organomegaly (lymphadenopathy > splenomegaly, hepatomegaly)
              • Extravascular volume overload (peripheral edema, ascites, or pleural effusion)
              • Endocrinopathy (excluding diabetes mellitus or hypothyroidism)
                • Hypogonadism is most common endocrine abnormality
                • Diabetes mellitus and hypothyroidism excluded due to high prevalence in general population
              • Skin changes
              • Papilledema
              • Thrombocytosis or polycythemia

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Nuclear Medicine Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              • Role of Radiologist

                                Selected References

                                1. Khouri J et al: Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome: a review. JAMA Oncol. 7(9):1383-91, 2021
                                2. Dispenzieri A: POEMS syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol. 94(7):812-27, 2019
                                3. Messiou C et al: Guidelines for acquisition, interpretation, and reporting of whole-body MRI in myeloma: myeloma response assessment and diagnosis system (MY-RADS). Radiology. 291(1):5-3, 2019
                                4. Glazebrook K et al: Computed tomography assessment of bone lesions in patients with POEMS syndrome. Eur Radiol. 25(2):497-504, 2015
                                5. Pan Q et al: Characterizing POEMS syndrome with 18F-FDG PET/CT. J Nucl Med. 56(9):1334-7, 2015
                                6. Shi X et al: CT characteristics in 24 patients with POEMS syndrome. Acta Radiol. 57(1):51-7, 2015
                                7. Montoriol PF et al: Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. Eur J Radiol. 80(3):861-4, 2011
                                8. Albertí MA et al: 18F-FDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol. 76(2):180-2, 2010
                                9. Ohwada C et al: Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome. Bone Marrow Transplant. 43(9):739-40, 2009
                                10. Allam JS et al: Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest. 133(4):969-74, 2008
                                11. Kuwabara S et al: Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology. 71(21):1691-5, 2008
                                12. Silberman J et al: Review of peripheral neuropathy in plasma cell disorders. Hematol Oncol. 26(2):55-65, 2008
                                13. Dispenzieri A et al: POEMS syndrome: definitions and long-term outcome. Blood. 101(7):2496-506, 2003
                                14. Ishikawa O et al: The skin changes of POEMS syndrome. Br J Dermatol. 117(4):523-6, 1987
                                15. Resnick D et al: Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. Radiology. 140(1):17-22, 1981
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Clinical Issues

                                      TERMINOLOGY

                                      • Synonyms

                                        • Osteosclerotic myeloma, Crow-Fukase syndrome, plasma cell dyscrasia, endocrinopathy, polyneuropathy (PEP) syndrome, Takatsuki syndrome
                                      • Definitions

                                        • Rare paraneoplastic syndrome due to underlying plasma cell disorder
                                        • Old acronym defines some features: polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS)
                                        • Diagnosis requires following
                                          • Both mandatory criteria
                                            • Polyneuropathy
                                            • Monoclonal plasma cell proliferative disorder
                                          • At least 1 major criterion
                                            • Sclerotic or mixed sclerotic/lytic myelomatous bone lesion on radiography or CT
                                            • Castleman disease (giant cell lymph node hyperplasia, angiofollicular lymph node hyperplasia)
                                            • Elevated serum or plasma vascular endothelial growth factor (VEGF) levels (≥ 3x upper limit of normal)
                                          • At least 1 minor criterion
                                            • Organomegaly (lymphadenopathy > splenomegaly, hepatomegaly)
                                            • Extravascular volume overload (peripheral edema, ascites, or pleural effusion)
                                            • Endocrinopathy (excluding diabetes mellitus or hypothyroidism)
                                              • Hypogonadism is most common endocrine abnormality
                                              • Diabetes mellitus and hypothyroidism excluded due to high prevalence in general population
                                            • Skin changes
                                            • Papilledema
                                            • Thrombocytosis or polycythemia

                                      IMAGING

                                      • General Features

                                        • Radiographic Findings

                                          • CT Findings

                                            • MR Findings

                                              • Nuclear Medicine Findings

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            • Role of Radiologist

                                                              Selected References

                                                              1. Khouri J et al: Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome: a review. JAMA Oncol. 7(9):1383-91, 2021
                                                              2. Dispenzieri A: POEMS syndrome: 2019 update on diagnosis, risk-stratification, and management. Am J Hematol. 94(7):812-27, 2019
                                                              3. Messiou C et al: Guidelines for acquisition, interpretation, and reporting of whole-body MRI in myeloma: myeloma response assessment and diagnosis system (MY-RADS). Radiology. 291(1):5-3, 2019
                                                              4. Glazebrook K et al: Computed tomography assessment of bone lesions in patients with POEMS syndrome. Eur Radiol. 25(2):497-504, 2015
                                                              5. Pan Q et al: Characterizing POEMS syndrome with 18F-FDG PET/CT. J Nucl Med. 56(9):1334-7, 2015
                                                              6. Shi X et al: CT characteristics in 24 patients with POEMS syndrome. Acta Radiol. 57(1):51-7, 2015
                                                              7. Montoriol PF et al: Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. Eur J Radiol. 80(3):861-4, 2011
                                                              8. Albertí MA et al: 18F-FDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol. 76(2):180-2, 2010
                                                              9. Ohwada C et al: Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome. Bone Marrow Transplant. 43(9):739-40, 2009
                                                              10. Allam JS et al: Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest. 133(4):969-74, 2008
                                                              11. Kuwabara S et al: Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology. 71(21):1691-5, 2008
                                                              12. Silberman J et al: Review of peripheral neuropathy in plasma cell disorders. Hematol Oncol. 26(2):55-65, 2008
                                                              13. Dispenzieri A et al: POEMS syndrome: definitions and long-term outcome. Blood. 101(7):2496-506, 2003
                                                              14. Ishikawa O et al: The skin changes of POEMS syndrome. Br J Dermatol. 117(4):523-6, 1987
                                                              15. Resnick D et al: Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. Radiology. 140(1):17-22, 1981