Tumor-like Lesions in Patients with Granulomatosis with Polyangiitis: A Case Series

Isr Med Assoc J. 2021 Jun;23(6):350-352.

Abstract

Background: Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis. It usually involves the respiratory tract and kidney. Rarely, tumor-resembling inflammatory changes ensue.

Objectives: To report three unique cases of GPA presenting with tumor-like lesions in various organs.

Methods: We presented three cases of GPA. Case 1 presented with typical upper respiratory symptoms of GPA and a mediastinal mass. Case 2 presented with low back pain, a large retroperitoneal mass, and nodular skin lesions. Case 3 presented with epigastric pain and a paravertebral inflammatory mass.

Results: The patients were treated successfully with rituximab.

Conclusions: Clinicians should be aware of this presentation of granulomatosis with polyangiitis, which is known as Tumefaction Wegener's granulomatosis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Biomarkers / blood
  • Biopsy / methods
  • Diagnosis, Differential
  • Female
  • Granuloma / pathology
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / drug therapy
  • Granulomatosis with Polyangiitis* / pathology
  • Granulomatosis with Polyangiitis* / physiopathology
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Kidney Neoplasms / diagnosis*
  • Magnetic Resonance Imaging / methods
  • Male
  • Mediastinal Neoplasms / diagnosis*
  • Middle Aged
  • Myeloblastin / immunology
  • Retroperitoneal Neoplasms / diagnosis*
  • Rituximab / administration & dosage*
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Biomarkers
  • Immunosuppressive Agents
  • Rituximab
  • Myeloblastin