A 32-year-old woman presented with a dull aching abdominal pain of 2 months duration.
She had similar complaints 18 months earlier and on evaluation, she was found to have
a 4-cm cystic lesion near the head of the pancreas ([Fig. 1]). A repeat contrast enhanced computed tomography scan of the abdomen revealed a
complex thick-walled cystic lesion measuring ~ 5 cm in size, located near the head
of the pancreas and invading the medial wall of the inferior vena cava and involving
the left renal vein ([Fig. 2]). Subsequent 18-fluorodeoxyglucose positron emission tomography showed intense uptake
in the lesion with a maximum standardized uptake value of 9.0 ([Fig. 3]). Endoscopic ultrasound (EUS) revealed a 5-cm complex septated cystic lesion in
relation to the head of the pancreas abutting the inferior vena cava ([Fig. 4]). EUS guided aspiration of the cyst revealed a serosanguinous fluid that was rich
in lymphocytes and no malignant cells were seen. The cyst was completely emptied and
fluid cancer antigen 19-9, carcinoembryonic antigen, and triglyceride were within
normal limits, whereas fluid amylase and lipase were elevated.
Fig. 1 A 32-year-old woman presented with a dull aching abdominal pain of 2 months duration.
A computed tomography (CT) abdominal scan revealed a 4-cm cystic lesion near the head
of the pancreas.
Fig. 2 A repeat contrast enhanced CT abdominal scan showed a complex thick-walled cystic
lesion measuring ~ 5 cm in size, located near the head of the pancreas and invading the medial wall
of the inferior vena cava (IVC).
Fig. 3 Subsequent 18-fluorodeoxyglucose positron emission tomography revealed intense 18-fluorodeoxyglucose
uptake in the lesion.
Fig. 4 Endoscopic ultrasound (EUS) revealed a 5-cm complex septated cystic lesion located
near the head of the pancreas and abutting the inferior vena cava.
The patient underwent laparotomy and a 6-cm hard lesion arising from the infrahepatic
inferior vena cava was observed. There was extensive desmoplastic reaction surrounding
the lesion involving the aorta, and superior mesenteric artery. The lesion was removed
and reconstruction of the inferior vena cava and left renal vein was performed using
a polytetrafluoroethylene vascular graft. The biopsy from the resected specimen revealed
a lymphoplasmacytic infiltrate with storiform fibrosis and obliterative phlebitis
suggesting a diagnosis of idiopathic retroperitoneal fibrosis.
Retroperitoneal fibrosis is a rare entity characterized by nonspecific chronic inflammation
of the retroperitoneum that can be caused by trauma, radiation or drugs such as methylsergide
[1]
[2]. Treatment is usually medical in the form of immunosuppression with or without surgical
intervention depending upon the stage of the disease and the type of organ involved
in the disease process [3]. Formation of a pseudocyst is an unusual complication of retroperitoneal fibrosis
[4].
Endoscopy_UCTN_Code_CCL_1AF_2AG_3AB
