POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report

Bello, Julio Plata; Garcı́a-Marı́n, Vı́ctor

doi:10.1186/1752-1947-7-245

Cited by 4 publications

(3 citation statements)

References 13 publications

(17 reference statements)

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“…Studies have shown a satisfactory role of surgical removal of the tumor in combination with post-operative radiotherapy and chemotherapy for systemic involvement. [ 4 , 6 ]…”

Section: Brief Review Of the Topicmentioning

confidence: 99%

Cytodiagnosis of scalp swelling: An uncommon presentation of a common neoplasm

et al. 2020

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“…Studies have shown a satisfactory role of surgical removal of the tumor in combination with post-operative radiotherapy and chemotherapy for systemic involvement. [ 4 , 6 ]…”

Section: Brief Review Of the Topicmentioning

confidence: 99%

Cytodiagnosis of scalp swelling: An uncommon presentation of a common neoplasm

et al. 2020

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“…Cases of POEMS syndrome have been reported in Japan ( 3 – 5 ), France, the United States, China and India ( 6 – 9 ). However, the incidence and prevalence of POEMS syndrome is unknown ( 10 ). Though the mechanism by which plasma cells cause POEMS syndrome remains to be fully understood, increased levels of vascular endothelial growth factor (VEGF) may be a factor ( 11 ).…”

Section: Introductionmentioning

confidence: 99%

POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report

et al. 2017

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Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss. Following the diagnosis of POEMS syndrome, the patient was treated only with pain-alleviating corticosteroids. Respiratory failure-induced mortality occurred 24 months after the patient first experienced difficulty walking and numbness in her lower extremities. The present study suggests that abnormal symptoms in cases of POEMS syndrome should be further evaluated during the diagnosis and treatment.

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“…Previous studies have shown that radiotherapy and chemotherapy are effective treatments for solitary and multiple lesions, respectively [ 10 - 13 ]. Sometimes, surgery can be an option for patients with cranial vault plasmacytoma [ 14 ]. However, the role of radiotherapy in patients with multiple lesions remains unclear, and the treatment outcomes vary according to the underlying plasma cell disorders [ 15 ].…”

Section: Introductionmentioning

confidence: 99%

The role of radiotherapy in the management of POEMS syndrome

et al. 2014

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BackgroundPOEMS syndrome is a paraneoplastic syndrome caused by an underlying plasma cell proliferative disease. In this study, we examined the treatment outcomes and role of radiotherapy in the management of POEMS syndrome.MethodsIn total, 33 patients diagnosed with POEMS syndrome were analyzed. These patients presented with osteosclerotic myeloma (OSM, n = 13), Castleman’s disease (CD, n = 4), OSM with CD (n = 10), and vascular endothelial growth factor elevation without gross lesions (VEGFe, n = 6), respectively. The patients were treated by radiotherapy alone (n = 4), chemotherapy alone (n = 16), or a combination thereof (n = 9).ResultsThe clinical response rates of radiotherapy, chemotherapy, and radiotherapy plus chemotherapy were 75%, 69%, and 89%, respectively. In addition, the hematologic response rates were 50%, 69%, and 71%, respectively. Among the six patients with limited multiple lesions who underwent radiotherapy, the clinical symptoms were improved in five patients after radiotherapy. The median progression-free survival (PFS) was 51 months, and the median overall survival (OS) was 65 months. In univariate analysis, the administration of chemotherapy was significantly associated with better PFS (p = 0.007) and OS (p = 0.020). In contrast, underlying VEGFe was a significant factor worsening PFS (p = 0.035) and OS (p = 0.008).ConclusionsRadiotherapy produces a reliable clinical response and is effective in improving POEMS-associated symptoms that are refractory to chemotherapy in selected patients with clustered or limited multiple lesions that can be covered by single radiation field.

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report

Cited by 4 publications

References 13 publications

Cytodiagnosis of scalp swelling: An uncommon presentation of a common neoplasm

Cytodiagnosis of scalp swelling: An uncommon presentation of a common neoplasm

POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report

The role of radiotherapy in the management of POEMS syndrome

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