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Hyaline-Vascular Castleman Disease : Clinical & Gross

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HemePath_CastlemanDz_HV35_Adrenal_resized.jpg

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Clinical Features of Hyaline-vascular Castleman Disease (HVCD): HVCD can occur at any age but typically affects young adults. It usually presents as a localized mass-like enlargement of mediastinal or cervical lymph nodes.

Other sites of involvement include: axilla, retroperitoneum, mesentery, broad ligament, nasopharynx, meninges, and soft tissues of extremities. Besides a mass lesion, there are no systemic symptoms. There is also no association with HIV infection. Surgical resection is adequate treatment. Systemic therapy is not required.

HVCD was considered to be a reactive or hyperplastic process. However, evidence of monoclonality in lymph node stromal cells in recent studies has led to the suggestion that it may be a neoplasm.

Grossly, HVCD presents as a large well-circumscribed mass with a solid gray-white cut surface.This specimen photograph shows HVCD involving retroperitoneum. Image copyright: pathorama.ch.

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