Characterizing POEMS Syndrome with <sup>18</sup>F-FDG PET/CT

Pan, Qingqing; Li, Jian; Li, Fang; Zhou, Daobin; Zhu, Zhaohui

doi:10.2967/jnumed.115.160507

Cited by 38 publications

(23 citation statements)

References 14 publications

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“…However, in the well‐described Mayo series, only 26% were eligible to receive radiation as their primary therapy. Moreover, with the advance of imaging modality, extensive bone involvement has been identified in majority of the cases, limiting the use of radiation . In terms of autotransplant, 41% and 36% cases from Mayo and our institute received it, respectively.…”

Section: Discussionmentioning

confidence: 96%

“…Systolic pulmonary arterial pressure (sPAP) was estimated based on echocardiography, and pulmonary arterial hypertension (PAH) was defined as a sPAP ≥ 50 mm Hg . All patients underwent 18 F‐FDG Positron emission tomography–computed tomography (PET‐CT) at baseline . Pleural effusion and ascites were detected by the mediastinal window of computed tomography.…”

Section: Methodsmentioning

confidence: 99%

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A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

Huang

Cai

et al. 2018

American J Hematol

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma dyscrasia without standard treatment. This phase II prospective trial evaluates the safety and response of 12 cycles of low dose lenalidomide (10 mg) plus dexamethasone (Rdex) in patients with newly diagnosed POEMS syndrome. Forty-one patients (28 men) were enrolled and the median age at diagnosis was 49 years (range, 21-70 years). Twenty-one patients (46%) achieved complete hematologic response and the neurologic response rate was 95%. The median serum vascular endothelial growth factor (VEGF) declined from 5155 pg/mL (range, 534-14 328 pg/mL) to 832 pg/mL (95-6254 pg/mL) after therapy. The overall VEGF response rate was 83%, and the median time to response was 2 months, with a mean VEGF reduction of 43% at the first month. In terms of clinical response, Rdex substantially relieved extravascular volume overload, organomegaly, and pulmonary hypertension. No treatment-related deaths occurred and no patients suffered from lenalidomide-related grade 3 or above adverse events. After a median follow-up of 34 months, median overall survival (OS) and progression-free survival (PFS) were not reached, with an estimated 3-year OS and PFS of 90% and 75%, respectively. In conclusion, Rdex was active with high hematologic, VEGF and organ response rate and well tolerated for patients with newly diagnosed POEMS syndrome. This trial was registered at www.clinicaltrials.gov as #NCT01816620.

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Section: Discussionmentioning

confidence: 96%

Section: Methodsmentioning

confidence: 99%

A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

Huang

Cai

et al. 2018

American J Hematol

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“…The role of 18flourodeoxyglucose PET/CT has already been described in POEMS. FDG uptake is more pronounced in the context of a lytic component in the bone lesion 7. Several other small lesions were only demonstrable on MRI because of their small size, this helped us opt for a systemic approach rather than radiotherapy to the plasmacytoma in the acetabulum.…”

Section: Discussionmentioning

confidence: 99%

POEMS syndrome: a unique presentation and a diagnostic challenge

et al. 2019

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We present the case of a previously well, 70-year-old lady who presented with gait deterioration and a clinicoradiological picture of myositis with T2-enhancement on MRI of the distal lower limb muscles. A muscle biopsy pointed towards neurogenic amyotrophy and a mixed demyelinating and axonal sensorimotor polyneuropathy was confirmed on nerve conduction studies. This was initially thought to be secondary to a myeloproliferative disorder but a positron emission tomography scan showed uptake in the left hip which corresponded to what was previously reported as a subchondral cyst on CT. A biopsy showed this to be a plasmacytoma in the context of a normal serum protein electrophoresis and a polyclonal increase in light chains on serum-free light chain estimation. The patient was started on treatment 6 months after the presentation for a diagnosis of POEMS syndrome, by which time, the patient had become bed-bound and fully dependent.

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“…Interestingly enough, lesions´ appearance in fifteen patients at three months following treatment showed lesion captation reduction but tomographic images identified no changes. This imaging mode further allows for follow up of adenomegaly that are identified at diagnosis, as well as that for hepatomegaly and splenomegaly, which tend to have abnormal captation [14].…”

Section: Discussionmentioning

confidence: 99%

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

Enciso¹,

Aponte²,

Rodríguez³

et al. 2017

J Clin Case Rep

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POEMS syndrome is a paraneoplastic phenomenon associated with polyneuropathy and paraproteinemia that arises out of the clonal expansion of plasma tumor cells. Its other distinguishing features include sclerotic bone lesions, increased vascular endothelial growth factor levels, Castleman disease alterations in lymph node biopsy and association with organomegaly, endocrinopathy, cutaneous lesions, papilledema, extravascular volume overload and thrombocytosis. Although established diagnostic criteria exist, the fact that the disease is rare and shares similar clinical manifestations with non-neoplastic conditions means that in most cases patients are diagnosed at latestage, when multiple complications have appeared and treatments for other conditions have already been given. Therefore, a high degree of suspicion combined with a multidisciplinary approach are requisites for attaining precise diagnosis and providing timely treatment. Due to the wide differential diagnosis that the syndrome´s clinical features encompass as well as its subsequent favourable responses to bone marrow transplant and diverse chemotherapy treatments, clinicians from diverse fields should include the POEMS syndrome within polyneuropathy differential diagnoses that require specific diagnostic tests. Herein, we present our case study in which we emphasize diagnosis and treatment of patients treated at our institution.

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Characterizing POEMS Syndrome with ¹⁸F-FDG PET/CT

Cited by 38 publications

References 14 publications

A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

POEMS syndrome: a unique presentation and a diagnostic challenge

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

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Characterizing POEMS Syndrome with 18F-FDG PET/CT

Cited by 38 publications

References 14 publications

A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

A prospective phase II study of low dose lenalidomide plus dexamethasone in patients with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome

POEMS syndrome: a unique presentation and a diagnostic challenge

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

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Characterizing POEMS Syndrome with ¹⁸F-FDG PET/CT