Download Abstract A 54-year-old female was seen at another hospital because

Abstract
A 54-year-old female was seen at another hospital because of jaundice. CT showed an unclear
boundary and a poorly enhanced mass lesion in the pancreatic body, measuring 28 mm in
diameter. MRCP showed stenosis of the lower bile duct and the main pancreatic duct in the
pancreatic body and slight dilatation of the main pancreatic duct in the pancreatic tail. According
to these findings, the preoperative diagnosis was synchronous double cancers of primary lower
bile duct cancer and pancreatic body cancer. We performed pylorus-preserving
pancreaticoduodenectomy with splenic artery and vein resection. A histopathological
examination revealed that the lower bile duct tumor was moderately differentiated tubular
adenocarcinoma, and the pancreatic body tumor was moderately differentiated tubular
adenocarcinoma. These two tumors showed no histopathological continuity. According to these
pathological findings, we diagnosed the patient with synchronous double cancers of primary
lower bile duct cancer and pancreatic body cancer. The patient was discharged from the hospital
on the 48th day after surgery. However, she died of multiple organ failure due to cancer
recurrence 22 months after surgery.
Key words: Double cancer, Bile duct cancer, Pancreatic cancer, Synchronous
Introduction
In view of the recent progress in imaging techniques and aging of the population, synchronous
double cancers are being reported with increasing frequency.1 However, the development of
synchronous double primary cancers of the bile duct and pancreas is rare, and there are only 6
other reported cases of synchronous double primary cancers consisting of bile duct cancer and
pancreatic cancer. 2-7 We herein report a surgical case of synchronous double cancers in a patient
with primary bile duct cancer and pancreatic cancer, and review the literature.
Case report
A 54-year-old female was seen at another hospital because of jaundice. Computed tomography
(CT) revealed intrahepatic bile duct dilatation, lower bile duct stenosis and stenosis of the main
pancreatic duct (MPD). She underwent endoscopic retrograde cholangiopancreatography
(ERCP) and an endoscopic nasobiliary drainage (ENBD) tube was placed in the common bile
duct. She was diagnosed autoimmune pancreatitis and sclerosing cholangitis by image findings,
and steroid treatment was started at another hospital. But improvement of the lower bile duct
and MPD stenosis were not showed, and she was then referred to our hospital for close
inspection and treatment. On laboratory examination, the serum levels of total bilirubin, alkaline
phosphatase (ALP) and γ-glutamyl transpeptidase (γ-GTP) were elevated to 1.5 mg/dl (normal
range 0.2-1.2), 767 U/l (normal range: 115-359 U/l) and 361 U/l (normal range: 11-58 U/l),
respectively. The level of tumor markers carcinoembryonic antigen (CEA), carbohydrate
antigen 19-9 (CA19-9), S-pancreas-1 antigen (SPan-1) and DUPAN-2 were in the normal
ranges. An immunological examination revealed that the levels of serum IgG and IgG4 were in
the normal ranges. Contrast-enhanced CT showed an unclear boundary mass lesion in the
pancreatic body, measuring 28 mm in diameter. The mass lesion revealed a low density in the
early phase (Fig. 1). Magnetic resonance cholangiopancreatography (MRCP) showed stenosis
of the lower bile duct and the MPD in the pancreatic body and a slight dilatation of the MPD in
the pancreatic tail (Fig. 2).
According to these findings, the preoperative diagnosis was synchronous double cancers of
primary lower bile duct cancer and pancreatic body cancer. After 144 days passed from ENBD
tube was placed, we performed pylorus-preserving pancreaticoduodenectomy with splenic
artery and vein resection.
The cut surface of the resected specimen from the bile duct showed an irregular and hard tumor,
measuring 30×10 mm in size (Fig. 3A). The cut surface of the resected specimen from the
pancreatic body showed a hard and solid tumor, measuring 30×13 mm (Fig. 3B). A
histopathological examination revealed that the lower bile duct tumor was moderately
differentiated tubular adenocarcinoma, and the pancreatic body tumor was moderately
differentiated tubular adenocarcinoma (Fig. 4A,B). These two tumors showed no
histopathological continuity.
According to these pathological findings, we diagnosed the patient with synchronous double
cancers of primary lower bile duct cancer and pancreatic body cancer. According to the UICC
classification, the stage of the lower bile duct cancer was classified as T3N0M0, StageⅡA, and
the pancreatic body cancer was classified as T3N0M0, StageⅡA. Exacerbation of diabetes
mellitus developed in postoperative period, and it was slightly difficult to control the erratic
blood sugar level. The patient was discharged from the hospital on the 48th day after surgery.
However, 18 months later, a recurrence of peritoneal dissemination was observed. The patient
died of multiple organ failure due to cancer recurrence 22 months after surgery.
Discussion
The frequency of multiple primary tumors among all cases of malignancy ranges from 1-3%.8
The frequency of pancreatic cancer in association with cancer of other organs is estimated to
range from 7.3-16.7%.9
According to the criteria presented by Warren and Gates, double cancers are defined as follows:
(1) each of the tumors must present a definite picture of malignancy, (2) each must be
histologically distinct, and (3) the probability that one is a metastatic lesion from the other must
be ruled out.10 If the time interval between the secondary cancer and primary cancer diagnoses is
less than 6 months, then patients are considered to have synchronous tumors, whereas if the time
interval is more than 6 months, then patients are considered to have metachronous tumors.11 In
our case, the malignant features of each tumor were synchronously confirmed by the
postoperative pathological diagnosis, and the possibility that one was a metastasis of the other
was excluded. Therefore, we diagnosed the patient with synchronous double cancers of primary
lower bile duct cancer and pancreatic body cancer.
There are only 7 reports of double cancer consisting of bile duct cancer and pancreatic cancer,
including our case (Table 1).2-7 The patients in these cases were from 54-79 years of age (mean:
68 years). The patients consisted of 3 males and 4 females. The most frequent symptom was
jaundice. The most common site of bile duct cancer was the inferior bile duct, and the most
common histology was tubular adenocarcinoma, followed by papillary adenocarcinoma. The
most common site of pancreatic cancer was the pancreatic head, and the most common
histology was tubular adenocarcinoma.
Prognostic factors for bile duct cancer and pancreatic cancer include surgical margin (curative
resection) and lymph node metastasis.12-16 One case did not undergo curative resection, and the
patient died 8 months after surgery. Six cases underwent curative resection and two died within
one year; however, three cases including our case obtained a relatively long-term survival.
Hosokawa et al.6 reported a patient who was still alive without recurrence for 84 months after
surgery.
In summary, we herein reported a surgical case of double primary cancers of the bile duct and
pancreas. Synchronous double cancers of the bile duct and pancreas are rare, with only 7
reported cases in the literature, including the present case. Double cancers of the bile duct cancer
and pancreatic cancer have a poor prognosis. However, our findings suggest that curative
resection is an effective treatment for double cancer consisting of bile duct cancer and pancreatic
cancer and it may provide a long-term survival.
References
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Captions
Fig. 1: CT showed an unclear boundary and poorly enhanced mass lesion in the
pancreatic body, measuring 28 mm in diameter (arrows).
Fig. 2: MRCP showed stenosis of the lower bile duct (arrows) and the main pancreatic duct in
the pancreatic body (arrowheads) and slight dilatation of the main pancreatic duct in the
pancreatic tail.
Fig. 3: (A) The cut surface of the resected specimen from the bile duct showed an irregular and
hard tumor, measuring 30×10 mm (arrows).
(B) The cut surface of the resected specimen from the pancreatic body showed a hard and
solid tumor, measuring 30×13 mm (arrows).
Fig. 4: (A) A histopathological examination revealed that the lower bile duct tumor was
moderately differentiated tubular adenocarcinoma (H＆E, 40×).
(B) A histopathological examination revealed that the pancreatic body tumor was
moderately differentiated tubular adenocarcinoma (H＆E, 40×).
Table 1: Reported surgical cases of synchronous double primary cancers of the bile duct and
pancreas.