Unicentric Hyaline Vascular Variant Castleman Disease

Unicentric Hyaline Vascular Variant Castleman Disease

Pei Lin, MD

Castleman disease, hyaline-vascular variant (HV-CD). Regressed germinal centers image are surrounded by expanded mantle zones image. There is also marked interfollicular blood vessel proliferation image.

Positive CT scan shows a 4.5 cm mass in the low left paraspinal region image. The site of disease in this patient is unusual but is otherwise consistent with unifocal HV-CD.



  • Castleman disease, hyaline-vascular variant (HV-CD)


  • Angiofollicular lymph node hyperplasia

  • Giant lymph node hyperplasia

  • Angiomatous lymphoid hamartoma


  • Typically unicentric and reactive lymphoproliferation of unknown etiology involving lymph nodes



  • Possible factors in pathogenesis

    • Dysregulation of vascular endothelial growth factor

    • Follicular dendritic cell (FDC) dysplasia may be precursor



  • Incidence

    • Rare

  • Age

    • Young adults; median age: 4th decade

  • Gender

    • No preference


  • Patients present with localized mass

    • Mass often detected incidentally

  • Rarely symptoms related to compression of adjacent tissues by enlarged lymph nodes

  • Lymphadenopathy, localized

    • Mediastinal or thoracic lymph nodes most commonly involved

    • Other sites: Cervical and retroperitoneal lymph nodes

    • Extranodal sites of involvement are rare

  • Patients may develop secondary amyloidosis


  • Surgical approaches

    • Surgery to remove enlarged lymph nodes

  • Adjuvant therapy

    • Usually not necessary

    • Radiation therapy has been used to alleviate compression symptoms


  • Excellent

  • Surgical removal is usually curative; relapse can occur uncommonly

  • Malignant neoplasms can arise in association with HV-CD

    • Follicular dendritic cell sarcomas are most common

    • Vascular neoplasms

    • Secondary neoplasms in HV-CD are often low grade but metastases are reported


Radiographic Findings

  • Enlarged lymph node or group of lymph nodes, but any site can be involved

    • Mediastinal or thoracic lymph nodes most common


Histologic Features

  • Numerous follicles in cortex and medulla of lymph node

  • Obliteration of subcapsular sinuses

  • 2 or more germinal centers in a follicle (“twinning”)

  • Follicles typically large with regressed (or involuted) germinal centers

    • Germinal centers are composed mostly of FDC with few lymphocytes

    • FDCs often hyperplastic and can show dysplasia

  • Many follicles show so-called “lollipop” features characterized by

    • Concentric rings of the mantle zone lymphocytes (“onion skin” appearance)

    • Sclerotic blood vessels radially traversing into germinal center

  • Interfollicular or stromal component is also important

    • Increased number of high endothelial venules with hyalinized walls

    • Stromal component can predominate with only few hyaline-vascular follicles

  • Clusters of plasmacytoid dendritic cells (plasmacytoid monocytes) can be prominent

  • Plasma cells and immunoblasts are not abundant in HV-CD

    • Much more common in plasma cell variant of CD



  • Human herpes virus-8 (HHV8) is absent

  • Polytypic B cells and T cells

  • Increased FDCs in involuted germinal centers often positive for CD21, CD23, CD35, or EGFR

    • Dysplastic FDCs often stain variably for FDC markers

  • Plasma cells are polytypic

Flow Cytometry

  • Polytypic B cells and T cells with normal immunophenotype


  • Rare cases reported with chromosomal translocations or other clonal abnormalities

    • No consistent cytogenetic findings

  • Del(12q13-15) resulted in intragenic HMGIC gene rearrangement reported in 1 case


  • No evidence of monoclonal immunoglobulin (Ig) or Tcell receptor (TCR) gene rearrangements

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Unicentric Hyaline Vascular Variant Castleman Disease

Full access? Get Clinical Tree

Get Clinical Tree app for offline access