POEMS syndrome: radiographic appearance with MRI correlation

Chong, Suzanne T.; Beasley, H. Scott; Daffner, Richard H.

doi:10.1007/s00256-005-0941-8

Cited by 40 publications

(23 citation statements)

References 10 publications

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“…The MRI imaging in POEMS syndrome is quite rare as the diagnosis is usually based on clinical symptoms, and the MRI scans are mostly performed in situations of diagnostic uncertainty. On the other hand, the character on T1 and T2-weighted sequences strongly illustrates the different types of bone involvement 5 . The contribution of angiography is dubious as the lesions are usually not of such an extent as seen in case report 3.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

“…There have been just a few reports on the contribution of MRI in the diagnostics of POEMS syndrome [5][6][7][8][9] . The MRI imaging in POEMS syndrome is quite rare as the diagnosis is usually based on clinical symptoms, and the MRI scans are mostly performed in situations of diagnostic uncertainty.…”

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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“…Osteosclerotic lesions occur in 95% of patients, and can be confused with benign bone islands, aneurysmal bone cysts, non-ossifying fibromas, and fibrous dysplasia [3,48,76,77] Some lesions are densely sclerotic, while others are lytic with a sclerotic rim, while still others have a mixed soap-bubble appearance. Bone windows of CT body images are often very informative, often even more so than FDGuptake, which can be variable [41,42].…”

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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“…Osteosclerotic lesions occur in approximately 95% of patients and can be confused with benign bone islands, aneurysmal bone cysts, nonossifying fibromas, and fibrous dysplasia. 7,9,87,88 Some lesions are densely sclerotic, whereas others are lytic with a sclerotic rim ( Figure 2D) and still others have a mixed soap-bubble appearance. FDG-PET/CT is a useful tool for screening for POEMS syndrome 34 as is (99m)Tc-HMDP bone scintigraphy.…”

Section: Targeting Other Features Of the Diseasementioning

confidence: 99%

How I treat POEMS syndrome

Dispenzieri

2012

Blood

101

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POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated VEGF, and abnormal pulmonary function. The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. The diagnosis is often delayed with intervening incorrect diagnoses of chronic inflammatory demyelinating polyradiculoneuropathy, myeloproliferative disorder, and monoclonal gammopathy of undetermined significance. Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, highdose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation. More universal recognition of and more scientific inquiry into the underpinnings of the disease will provide direction toward the best treatment strategies in the future. (Blood. 2012;119(24):5650-5658) Introduction POEMS syndrome, 1 also known as osteosclerotic myeloma, Takatsuki syndrome, 2 and Crow-Fukase syndrome, 3,4 is a rare paraneoplastic syndrome resulting from an underlying plasma cell disorder. There acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. There are 3 important points that relate to this memorable acronym. First, not all of the features within the acronym are required to make the diagnosis (Table 1). Second, there are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, elevated VEGF levels, abnormal pulmonary function tests, and a predisposition toward thrombosis. Lastly, there is a Castleman disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder. 5,6 Table 1 outlines the range of expected frequencies of each of the features based on the largest published series. 2,[7][8][9][10][11] The pathogenesis of the syndrome is not well understood. To date, VEGF is the cytokine that correlates best with disease activity, [12][13][14][15][16][17][18][19][20] although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy. 5,21-29 VEGF, which is expressed by osteoblasts, macrophages, tumor cells 30 (including plasma cells), 31,32 and megakaryocytes/pla...

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POEMS syndrome: radiographic appearance with MRI correlation

Cited by 40 publications

References 10 publications

Comparison of imaging methods in POEMS syndrome

Comparison of imaging methods in POEMS syndrome

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

How I treat POEMS syndrome

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