Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.

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Publication Details

Journal
Journal of Child Neurology
Published
2025-09-26
DOI
https://doi.org/10.1177/08830738251369737
Primary Topic
Peripheral Neuropathies and Disorders
Type
article
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article

Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report

Eduardo López‐Corella, Francisco Rivas‐Larrauri, Selma Scheffler‐Mendoza, Ana Luisa Rodríguez‐Lozano et al.
Journal of Child Neurology
Peripheral Neuropathies and Disorders
article

Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report

Eduardo López‐Corella, Francisco Rivas‐Larrauri, Selma Scheffler‐Mendoza, Ana Luisa Rodríguez‐Lozano, Hiromi Onuma‐Zamayoa, Marco Antonio Yamazaki‐Nakashimada, Melissa Espinosa‐Navarro, Magda Delgado-Landa
article en

Abstract

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.

Journal of Child NeurologyVol. 41(4)
Instituto Nacional de Salud (PE), Instituto Nacional de Pediatria (MX), Instituto Nacional de Cancerología (MX)
Openalex Percentile: Top 62%
Peripheral Neuropathies and Disorders
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