Ultrasonography showing the subtle isoechoic periaortic lesion (arrow)
Abdominal imagingCase Type
Dr Rumita Kayastha, Dr. Sunil Pradhan, Dr. Riwaz Acharya,
42 years, male
A 42-year-old male patient, a known case of adenocarcinoma of gastric antrum following partial gastrectomy with gastrojejunostomy, jejunojenustomy and 2 cycles of chemotherapy came to the Outpatient department for follow up CT scan after 6 months of surgery. He gives history of feeling lethargy following chemotherapy with normal bowel and bladder habit.
Ultrasonography (USG) showed mild left hydroureteronephrosis. Small hypoechoic lesion noted in the right lobe of liver with dilated Common Bile Duct (CBD) with mild dilatation of Intrahepatic biliary ducts (IHBDS).
Contrast-Enhanced Computed tomography (CECT) showed mildly enhancing periaortic soft tissue density lesion causing encasement of origin of coeliac trunk, right hepatic artery. The lesion extended up to the level of aortic bifurcation. It caused encasement and narrowing of main portal vein. Smooth narrowing of distal CBD and mild dilatation of IHBDs was noted. Narrowing of left upper ureter was noted with mild hydroureteronephrosis with delayed excretion of contrast material of left kidney after 24 hours. Hypodense mildly enhancing lesions noted in the right lobe of liver in segment IV, segment V and in the lower segments of bilateral lungs.
Magnetic Resonance Imaging (MRI) showed subtle T1 and T2 isointense periaortic lesion surrounding the abdominal aorta encasing the coeliac trunk, hepatic trunk as described in CT.
Retroperitoneal fibrosis (RPF) covers a range of diseases characterized by proliferation of fibroinflammatory tissue, surrounding the infrarenal portion of abdominal aorta, inferior vena cava (IVC), iliac vessels [1,2]. The idiopathic form of RPF is more common . Drugs (ergot alkaloids), neoplasms (lymphoma, sarcoma, carcinoid, metastases from stomach, colon, breast, lung, genitourinary tract cancer), infections (histoplasmosis, tuberculosis), radiation, trauma, surgery, hematoma, proliferative diseases, asbestos exposure are secondary causes [4, 5, 6]. Idiopathic RPF is included under chronic periaortitis, along with inflammatory abdominal aortic aneurysms which is associated with atherosclerotic aortic disease [1, 7, 8].
Histologically, active cellular stage shows capillary proliferation with abundant inflammatory cells and chronic stage shows hyalinized collagen. [9, 10, 11]
RPF manifests with symptoms such as anorexia, weight loss, fever, flank pain [12, 13]. Ureteral involvement is bilateral in most cases. Some patients present with nonfunctioning kidneys , lower extremity oedema, hydrocele .
RPF is seen as a hypoechoic or isoechoic periaortic mass in Ultrasonography (US) [16, 17]. Intravenous urography demonstrates the triad of medial deviation of the middle third of the ureters, tapering of the lumen of ureters in the lower lumbar spine and hydroureteronephrosis with delayed excretion of contrast material .
RPF appear as an irregular mildly enhancing soft-tissue periaortic mass, extending from the renal arteries to the iliac vessels and often progresses through the retroperitoneum to envelop the ureters and IVC on CT scan . RPF can spread inferiorly to the pelvis, renal hila, retroperitoneal structures like duodenum, renal pelvis and kidney [20, 21].
Magnetic Resonance Imaging (MRI) has low T1 and high T2 signal intensity and early contrast enhancement in active phase [20, 21]. The inactive stage demonstrates low T2 signal intensity and little contrast enhancement [22, 23].
Fluorine-Fluorodeoxyglucose (18F-FDG ) Positron Emission Tomography (PET) allows detection of metabolic activity of retroperitoneal lesions irrespective of a benign or malignant underlying cause [23, 24].
First description of the extrahepatic biliary obstruction secondary to RPF was made in 1964. Since then 13 cases have been reported in the medical literature .
Corticosteroids are considered the first-line treatment for patients with newly diagnosed idiopathic RPF .In addition to corticosteroid therapy, drainage of the upper urinary tract by temporary placement of nephrostomy tubes or ureteral stent may be necessary for ureteral obstruction [1,26]. Surgery is reserved for refractory cases
RPF secondary to malignancy has a poor prognosis . Anterior displacement of the aorta and IVC is seen in malignant RPF mainly due to enlargement of lymph nodes [5, 6]. The soft-tissue mass usually spares the posterior aspect of the great vessels and does not cause vascular displacement in benign cases [8,10].
Written informed patient consent for publication has been obtained.
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