Glomeruloid hemangioma associated with TAFRO syndrome

Hum Pathol. 2018 Dec:82:172-176. doi: 10.1016/j.humpath.2018.04.011. Epub 2018 Apr 25.


Glomeruloid hemangioma is a rare cutaneous lesion that has been considered as a specific cutaneous marker of POEMS syndrome. Herein, we present the first case of glomeruloid hemangioma associated with TAFRO syndrome, a unique variant of idiopathic multicentric Castleman disease. The patient is a 74-year-old woman presented with fever, cervical lymphadenopathy, thrombocytopenia, bilateral pleural effusions and ascites. Biopsy of the lymph node revealed multicentric Castleman disease-like histology and bone marrow biopsy showed mild reticulin fibrosis, consistent with TAFRO syndrome. The patient simultaneously developed multiple skin lesions, which were histologically confirmed as glomeruloid hemangioma. Multiple immunoglobulin-positive granules were detected in the proliferating endothelial cells. Glomeruloid hemangioma is not specific to POEMS syndrome and can be a manifestation of TAFRO syndrome.

Keywords: Glomeruloid hemangioma; Immunoglobulin; Multicentric Castleman disease; POEMS syndrome; TAFRO syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antineoplastic Agents, Immunological / therapeutic use
  • Biopsy
  • Castleman Disease / drug therapy
  • Castleman Disease / immunology
  • Castleman Disease / pathology*
  • Female
  • Hemangioma / drug therapy
  • Hemangioma / immunology
  • Hemangioma / pathology*
  • Humans
  • Immunoglobulins / analysis
  • Immunohistochemistry
  • Lymph Nodes / drug effects
  • Lymph Nodes / immunology
  • Lymph Nodes / pathology*
  • Rituximab / therapeutic use
  • Skin Neoplasms / drug therapy
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology*
  • Steroids / therapeutic use
  • Treatment Outcome


  • Antineoplastic Agents, Immunological
  • Immunoglobulins
  • Steroids
  • Rituximab

Supplementary concepts

  • Multi-centric Castleman's Disease