Imaging revealed a large retroperitoneal mass and an enlarged periaortic
lymph node measuring 3 cm.
sup] These lesions involve not only the periaortic
retroperitoneum but also the aortic wall.
Familial PHEO/PGL syndrome was suspected and a staged, minimally invasive approach was planned with the first attempt to spare the right adrenal to prevent adrenal insufficiency, followed by removal of the left peri-aortic nodule with completion periaortic
lymph node dissection.
Imaging findings include eccentric, saccular shaped, rapidly growing aneurysms with periaortic
soft tissue stranding, free fluid, and gas.
CT images showed lung scarring, splenomegaly, pericardial effusion, and multifocal adenopathy of the left axilla, mediastinum, celiac, periaortic
, retroperitoneal, and mesenteric regions.
On CT RPF appears as periaortic
soft tissue density extending from the level of renal artery to iliac vessels with frequent medial deviation and obstruction of ureters.
The patient then underwent left radical nephrectomy with periaortic
lymphadenectomy and extended right hemi-colectomy.
Involvement of the ureters in the periaortic
inflammatory process is common, documented to be present in 53 percent of CT scans, (3) with progression to ureteral obstruction in 20 percent of cases.
In the early course of disease, a periaortic
soft-tissue mass with or without rim enhancement (depending on the degree of necrosis) may be the only finding before development of the aneurysm.
Lymphatic spread via lymphatic channels that communicate with axillary, inguinal and periaortic
lymph nodes is also possible.
Central retroperitoneal lymphadenopathy with a few prominent lymph nodes in the left periaortic
window was also noted.
Granulomatosis with polyangiitis (Wegener's) presenting as a periaortic