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Radical nephrectomy for retroperitoneal fibrosis: Case report.
Matheus Miranda Paiva1, Eloi Guilherme Provinciali Moccellin2, Alessandro Vengjer2
1Santa Casa de Misericordia de Santos, Divisão de Urologia, Departamento de Cirurgia, Santos, São Paulo, Brazil.
International Journal of Surgery Case Reports
|November 7, 2024
Summary
Retroperitoneal fibrosis (RPF) is a rare fibroblast proliferation disease. This case highlights RPF mimicking malignancy, emphasizing the need for thorough diagnosis even with negative IgG4 levels.
Area of Science:
- Nephrology
- Oncology
- Pathology
Background:
- Retroperitoneal fibrosis (RPF) is a rare proliferative fibroblast disease with unclear etiology and nonspecific, late-onset symptoms.
- Elevated serum IgG4 levels are observed in up to 60% of patients, but RPF can occur with negative IgG4.
- The primary treatment goal is preserving kidney function.
Observation:
- A 34-year-old asymptomatic man presented with a poorly defined retroperitoneal mass initially suspected to be malignant.
- Initial biopsies led to a diagnosis of low-grade follicular lymphoma, treated with chemotherapy.
- Recurrence after one year necessitated a radical nephrectomy.
Findings:
- Histopathological examination revealed advanced retroperitoneal fibrosis (RPF) despite negative IgG4 levels.
- This case underscores that RPF can present atypically and mimic malignancy.
- RPF diagnosis relies on imaging and histopathology, with treatment tailored to disease invasiveness.
Implications:
- RPF diagnosis requires careful consideration, especially in cases with negative IgG4 levels.
- This case highlights the importance of comprehensive pathological evaluation to differentiate RPF from other retroperitoneal masses.
- Further research into RPF etiology and diagnostic markers is warranted.
