18F-FDG PET/CT in the evaluation of POEMS syndrome

Alberti, M.; Martínez-Yélamos, Sergio; Fernández, A.; Vidaller, Antonio; Narváez, José Antonio; Cano, Luis; Gámez, Cristina; Martı́nez-Matos, Juan Antonio

doi:10.1016/j.ejrad.2009.06.004

Cited by 60 publications

(42 citation statements)

References 8 publications

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“…This technique is considered essential for initial staging and evaluation in most malignancies, especially lymphoma. The use of 18-FDG-PET/CT in multiple myeloma is still under evaluation, and few data are available in the POEMS syndrome [20][21][22]. Twelve of our 19 patients had 18-FDG-PET/CT at diagnosis, revealing multiple lesions in 11 cases.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of lenalidomide in POEMS syndrome: A retrospective study of 20 patients

et al. 2013

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POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18‐FDG‐PET/CT at diagnosis (11 with positive findings), and nine patients during follow‐up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow‐up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses. Am. J. Hematol. 88:207–212, 2013. © 2012 Wiley Periodicals, Inc.

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Section: Discussionmentioning

confidence: 99%

Efficacy of lenalidomide in POEMS syndrome: A retrospective study of 20 patients

et al. 2013

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“…We found just two recent papers which demonstrated the potential of FDG-PET/CT scan in the evaluation of POEMS (ref. 17,18 ). Although quite expensive, PET/CT seems to be a very convenient method for detection of the activity as well as the extent and localization of bone and/or lymph node lesions.…”

Section: Discussionmentioning

confidence: 99%

Comparison of imaging methods in POEMS syndrome

Minařík¹,

Ščudla²,

Bačovský³

et al. 2012

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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“…The requirements set forth in Table I are designed to retain both sensitivity and specificity, potentially erring on the side of specificity. Making the diagnosis can be a challenge, but a good history and physical examination followed by appropriate testing-most notably radiographic assessment of bones [40][41][42] measurement of VEGF [13,17,20,43,44], and careful analysis of a bone marrow biopsy [39]-can differentiate this syndrome from other conditions like CIDP, monoclonal gammopathy of undetermined significance (MGUS) neuropathy, and immunoglobulin light chain amyloid neuropathy. A high platelet count is seen in 54% of POEMS patients as compared to 1.5% of patients with CIDP [45].…”

Section: Diagnosismentioning

confidence: 99%

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

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Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

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18F-FDG PET/CT in the evaluation of POEMS syndrome

Cited by 60 publications

References 8 publications

Efficacy of lenalidomide in POEMS syndrome: A retrospective study of 20 patients

Efficacy of lenalidomide in POEMS syndrome: A retrospective study of 20 patients

Comparison of imaging methods in POEMS syndrome

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

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