Ann R Coll Surg Engl 2010; 92:
Idiopathic retroperitoneal fibrosis of the pancreas is a very
rare and unusual progressive illness characterised by
chronic inflammation of connective tissue, for which con-
sistent therapeutic recommendations have not been
devised. About two-thirds of all cases are thought to be idio-
pathic whereas the remaining one-third of cases is second-
ary to chronic inflammation, trauma or malignancy.
A 43-year-old woman was referred to our institution with an
epigastric pain, occasional vomiting and weight loss. She
denied smoking or alcohol consumption and there was no sig-
nificant medical, surgical or trauma history. Physical exami-
nation was unremarkable and laboratory results including
complete blood count, kidney and liver function tests, amy-
lase, lipase, CEA and CA19-9 all were normal. The initial
chest, abdominal and pelvic computed tomography (CT) scan
revealed an ill-defined soft tissue density mass in the area of
the body of the pancreas measuring 3.8 × 5 cm partially encas-
ing the celiac trunk and the splenic artery in addition para-
aortic lymph node enlargement and no distant metastasis
(Fig. 1). A CT scan-guided fine-needle aspiration of the mass
was performed twice but the biopsies yielded no malignant
cells. Patient underwent exploratory laparatomy for sufficient
tissue biopsy and for celiac block; the operative findings were
consistent with the CT scan findings. Multiple incisional biop-
sies and celiac plexus block were performed. The histopatho-
logical analysis revealed fibrosis, chronic inflammation, no
malignant cells and reactive lymph node. A follow-up CT scan
was repeated 2 months later and revealed an increase in the
size of the mass from 3.8 × 5 cm to 5 × 6cm and encasement
of the three major branches of the celiac trunk (Fig. 2). There
was no detected increase in the levels of the tumour markers
CA19-9 and CEA. ESR was found to be elevated (75 mm/h).
PET/CT scan was done and showed patchy areas of uptake
in the pancreatic mass with a standardised uptake value
(SUV) of 1.9 which is related to post-surgical or active
inflammation. Tests for antinuclear antibodies and IgG4
levels were conducted, to rule out autoimmune pancreatitis
and results were normal.
ON-LINE CASE REPORT
Ann R Coll Surg Engl 2010; 92:
Idiopathic retroperitoneal fibrosis – Pancreas – Pancreatic carcinoma
Accepted 20 July 2010; published online
Faiez Daoud, King Hussein Cancer Center, Queen Rania Al Abdullah Street, Amman 11941, Jordan
T: +962 795565431; F: +9626 5353001; E: email@example.com
Idiopathic retroperitoneal fibrosis of the pancreas
versus pancreatic carcinoma
Bilal Baker, Habeeb Salameh, Faiez Daoud
Department of Surgery, King Hussein Cancer Center, Amman, Jordan
We report one of few cases of idiopathic retroperitoneal fibrosis of the pancreas, which is different from the classical retroperi-
toneal fibrosis that affects ureters and vessels that mimicking locally advanced pancreatic carcinoma at presentation.
Initial chest, abdominal and pelvic CT scan.
BAKER SALAMEH DAOUD Download full-text
Ann R Coll Surg Engl 2010; 92:
Idiopathic retroperitoneal fibrosis of the pancreas is an uncom-
mon disease characterised by chronic inflammation of the
retroperitoneal connective tissue that envelops the aorta and
tory and extends laterally to entrap one or both ureters.1
The most common presentation is ill-defined back pain
in obtaining sufficient tissue biopsy of the inoperable pancreatic
mass has made exploratory laparatomy an acceptable modality
for obtaining histological diagnoses for further management.
The differential diagnosis should include pancreatic carcinoma
in particular, and then autoimmune pancreatitis. Pancreatic car-
cinoma in our case was excluded by absence of malignant cells,
normal tumour markers and absence of distant metastasis.
Autoimmune pancreatitis is a new entity characterised by
diffuse irregular enlargement of the pancreas and diffuse irreg-
ular narrowing of the main pancreatic duct. In most cases,
autoimmune pancreatitis is mistaken for pancreatic cancer. A
number of laboratory tests such as serum IgG4, ANA and
steroid therapy.2In our patient, IgG4, ANA and rheumatoid fac-
tor levels were normal. There was no radiological evidence of
autoimmune pancreatitis, no lymphoplasmacytic infiltration
and no steroid remarkable response; therefore, a diagnosis of
autoimmune pancreatitis was excluded.
Our case demonstrates the typical clinical features of idio-
pathic retroperitoneal fibrosis affecting the pancreas including
pain, elevated ESR, normal tumour markers, absence of malig-
nant cells on histopathology and absence of metastasis.
Subsequently, we managed our case with prednisolone (1
case on regular CT scan follow-up. Following therapy, the
patient remained symptom-free and her ESR fluctuated
between 5–45 mm/h. At last follow-up, a CT scan was done 28
no evidence of metastasis (Fig. 3).
Many forms of treatment have been suggested in the man-
agement of retroperitoneal fibrosis. Options include the use of
steroids, tamoxifen or immunosuppressive drugs. Surgical
treatment is usually difficult because of the delay in diagnosis
and the tissue is extremely hard and may not easily separate
from the organs and vessels, making complete surgical removal
often impossible. Steroids have been claimed to decrease the
progression of fibrosis and to improve symptoms but there is no
consensus on the dose and the duration of steroids; however,
clinical or radiological progression of the disease after discon-
tinuation of steroids has been reported.3There are also several
other reports on the use of tamoxifen and immunosuppressive
drugs in the management of retroperitoneal fibrosis.4
The diagnosis idiopathic retroperitoneal fibrosis is usually
confirmed by demonstrating the absence of malignant cells
on biopsy, absence of metastasis, normal tumour marker
levels, and elevated levels of ESR.
1. Kaya M, Aydin F. Pancreatic mass lesion mimicking carcinoma: initial presentation
of retroperitoneal fibrosis. Turk J Gastroenterol 2005; 1 16 6: 156–9.
2. Ohtsubo K, Watanabe H, Tsuchiyama T, Mouri H, Yamaguchi Y, Motoo Y, et al. A
case of autoimmune pancreatitis associated with retroperitoneal fibrosis. JOP J
Pancreas (Online) 2007; 8 8: 320–5.
3. Kardar AH, Kattan S, Lindstedt E, Hanash K. Steroid therapy for idiopathic
retroperitoneal fibrosis: dose and duration. J Urol 2002; 1 16 68 8: 550–5.
4. Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal fibrosis to
tamoxifen. Surgery 1991; 1 10 09 9: 502–6.
increase in the size of the mass and encasement of the three major
branches of the celiac trunk.
Follow-up CT scan repeated 2 months later showing an
Figure 3 Final CT scan at 28 months after presentation showing a
stable tumour size and no evidence of metastasis.