Unicentric Castleman Disease

Hematol Oncol Clin North Am. 2018 Feb;32(1):65-73. doi: 10.1016/j.hoc.2017.09.006.

Abstract

Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder that manifests typically as proliferation of a single lymph node or region of lymph nodes. Histologically, hyaline vascular variant is found in a majority of UCDs. UCD commonly presents in younger patient populations. Patients with UCD may be asymptomatic or present with symptoms related to mass effects on surrounding structures. It is difficult to achieve a definitive diagnosis by imaging alone. Histologic examination of the lesion remains the gold standard for diagnosis. Complete surgical resection is the best primary treatment modality for UCD resulting in excellent long-term survival and low recurrence rates.

Keywords: Castleman disease; Hyaline vascular variant; Interleukin 6; Plasma cell variant; Unicentric.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Castleman Disease / diagnosis*
  • Castleman Disease / mortality
  • Castleman Disease / pathology*
  • Castleman Disease / therapy
  • Disease-Free Survival
  • Humans
  • Survival Rate