A 70 year old gentleman diagnosed with metastatic squamous cell carcinoma
- A 70-year-old gentleman was diagnosed with metastatic squamous cell carcinoma (metastases to left adrenal gland) in February 2017; completed chemotherapy (Paclitaxel + Carboplatin) on May 2017. No radiotherapy.
- The disease was stable on the last follow-up scan (April 2021).
- Atherosclerotic changes in the abdominal aorta. No periaortic soft tissue
- Significant non-enhancing peri-aortic soft tissue thickening and fat stranding seen adjacent the infrarenal aorta extending upto aortic bifurcation and abutting the inferior vena cava laterally with no significant luminal narrowing or thrombosis.
DIAGNOSIS:
RETROPERITONEAL FIBROSIS IN A KNOWN CASE OF LUNG MALIGNANCY
DISCUSSION:
- Retroperitoneal fibrosis (RPF) encompasses a range of diseases characterized by proliferation of aberrant fibroinflammatory tissue, which usually surrounds the infrarenal portion of the abdominal aorta, inferior vena cava, and iliac vessels.
- Risk factors include asbestos exposure, smoking, tumor, infection, trauma, radiotherapy, surgery, and use of certain drugs.
- The idiopathic form of RPF accounts for more than two-thirds of cases; the rest are secondary to factors such as drug use, malignancies, or infections. If promptly diagnosed and treated, idiopathic and most other benign forms of RPF have a good prognosis.
- In contrast, malignant RPF, which accounts for up to 10% of cases, has a poor prognosis.
- In early or active stages, variable enhancement can be seen with intravenous contrast while no enhancement may be seen in quiescent disease.
- Therefore, the most important diagnostic challenge is differentiation of benign from malignant RPF.
- The patient underwent a CT guided biopsy of the retroperitoneal periaortic soft tissue using coaxial needle system.
- The differentials considered were idiopathic retroperitoneal fibrosis and malignant RPF.
- Biopsy of periaortic soft tissue revealed features consistent with retroperitoneal fibrosis. Negative for malignancy.
- IHC confirmed IgG4 related retroperitoneal fibrosis.
References:
- https://rarediseases.info.nih.gov/diseases/9568/retroperitoneal-fibrosis
- Caiafa RO, Vinuesa AS, Izquierdo RS, Brufau BP, Ayuso Colella JR, Molina CN. Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics. 2013 Mar;33(2):535-52.
- Ranschaert, E., Bell, D. Retroperitoneal fibrosis. Reference article, Radiopaedia.org. (accessed on 13 Jan 2022) https://doi.org/10.53347/rID-14537
Dr Rajesh V Helavar
Consultant Interventional and Diagnostic Radiologist
Manipal Hospital, Yeshwanthpur, Bengaluru
Dr Vikas H P
Radiology Resident
Manipal Hospital, Yeshwanthpur, Bengaluru