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This case report details a rare presentation of unicentric Castleman disease (UCD), hyaline vascular type in a 22-year-old woman. The patient presented with a large, well-circumscribed mass in the paravertebral region causing back pain and shortness of breath. Diagnostic imaging and biopsy confirmed the diagnosis, and surgical excision led to a favorable outcome.

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POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy.

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Castleman disease variant of POEMS syndrome without M protein: a case report.

Front Oncol

December 2024

Department of Hematology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is a paraneoplastic syndrome associated with an underlying plasma cell neoplasm. According to the current diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy and clonal plasma cell disorder are required for diagnosis. We report a case of a Castleman disease variant of POEMS syndrome without monoclonal protein (M protein) expression, which presented with polyneuropathy, organomegaly, endocrinopathy, skin lesions, and sclerotic bone lesions.

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Castleman disease is a lymphadenopathy of unknown cause at a single site, which is designated as unicentric Castleman disease, or at multiple sites designated as multicentric Castleman disease. We present a patient who showed axillary reactive lymphoid hyperplasia, likely due to unicentric Castleman disease, and orbital extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma in a six-year follow-up. A 76-year-old man had a painless left axillary mass for an unknown period and also left complete blepharoptosis with no other systemic symptoms.

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Introduction: Castleman's disease (CD) represents a rare polyclonal lymphoproliferative disorder characterized by atypical lymph node hyperplasia, the precise etiology of which remains undefined. Pancreatic involvement of CD is particularly uncommon and often misdiagnosed due to its nonspecific clinical features, making it difficult to distinguish from tumors with abundant blood supply such as solid pseudopapillary tumors and neuroendocrine tumors. Multimodal imaging plays a crucial role in diagnosing pancreatic CD and determining the extent of lymph node involvement.

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