PURPOSE: Perivascular cuffing of the celiac and SMA as the only manifestation of pancreatic ductal adenocarcinoma (PDAC) is an under-recognized entity. This study aimed to report this rare entity and differentiate it from retroperitoneal fibrosis and vasculitis.
MATERIALS AND METHODS: Three groups of CT studies were retrospectively retrieved (12/11-9/17): A) three patients presented with celiac and SMA perivascular cuffing as the only manifestation of surgically proven PDAC; B) four retroperitoneal fibrosis patients; C) seven abdominal vasculitis patients.
Studies were evaluated for the following features: morphologic appearance, vascular anatomic site, wall thickness, extent of vasculature involvement and extra-vascular findings.
RESULTS: Circumferential soft tissue perivascular cuffing was seen in all three groups.
In the PDAC group, involvement of both the celiac and proximal-SMA was seen in one patient, while celiac or mid-SMA involvement was noted in each of the two.
Retroperitoneal fibrosis involved the aorta (4/4), common iliac arteries (4/4) and renal arteries (2/4). Hydronephrosis was present in 3/4 of patients.
Vasculitis patients showed aortitis accompanied by iliac or SMA involvement (2/7), isolated involvement of celiac (2/7), isolated aortitis (1/7), mid-SMA (1/7) or celiac and proximal-SMA (1/7).
PDAC and vasculitis groups demonstrated lower wall thickness in in comparison to retroperitoneal fibrosis (PDAC: 1.0±0.2cm, RF: 2.4±0.4cm, vasculitis: 1.2±0.5cm; p=0.002).
There was no statistically difference in the length of vascular involvement (PDAC: 6.3±2.1cm, retroperitoneal fibrosis: 8.7±0.5cm, vasculitis: 7.1±2.6cm).
CONCLUSION:
Celiac and SMA perivascular cuffing can be the sole manifestation of PDAC. This entity differs from retroperitoneal fibrosis, which involves the aorta, iliac and renal arteries, demonstrates thicker walls and often hydronephrosis. PDAC however, may be indistinguishable from vasculitis. Clinicians and radiologists alike should be aware of this rare, atypical PDAC appearance, as prompt investigation and diagnosis could have an impact over the clinical outcome.
