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Tumor-like Lesions in Patients with Granulomatosis with Polyangiitis: A Case Series
Omer Gendelman1,2,3, Yuval Kuntzman1,2,3, Ora Shovman1,2,3
1Department of Medicine B, Sheba Medical Center, Tel Hashomer, Israel.
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.
Insights
Granulomatosis with polyangiitis (GPA) can rarely manifest as tumor-like lesions. This rare presentation, known as Tumefaction Wegener's granulomatosis, was successfully treated with rituximab in three unique cases.
Area of Science:
- Rheumatology
- Pathology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis affecting small blood vessels.
- Typically, GPA involves the respiratory tract and kidneys.
- Uncommonly, GPA can present with inflammatory changes resembling tumors.
Observation:
- Three distinct cases of GPA exhibiting tumor-like lesions across various organs were analyzed.
- Case 1: Mediastinal mass with upper respiratory symptoms.
- Case 2: Retroperitoneal mass and skin lesions with low back pain.
- Case 3: Paravertebral inflammatory mass with epigastric pain.
Findings:
- All three patients diagnosed with GPA and tumor-like lesions achieved successful treatment outcomes.
- Rituximab was the therapeutic agent employed for all cases.
Implications:
- Highlights the importance of recognizing "Tumefaction Wegener's granulomatosis" as a rare clinical presentation of GPA.
- Clinicians should consider GPA in the differential diagnosis of unexplained tumor-like masses, particularly with concurrent systemic symptoms.
- Successful treatment with rituximab suggests its efficacy in managing this unusual manifestation of GPA.
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