Case Report

Journal of International Medical Research

48(9) 1–6
Primary congenital ! The Author(s) 2020
Article reuse guidelines:
choledochal cyst with sagepub.com/journals-permissions
DOI: 10.1177/0300060520946871
squamous cell carcinoma: journals.sagepub.com/home/imr

a case report

Lili Wang1,* , Xiaomei Ma1,*,

Dongdong Chen2, Jialiang Ren3, Hua Cheng4,
Gang Huang1 , Rong Wang1 and
Jiarong Cheng5

Abstract
Cases of extrahepatic bile duct carcinoma are mostly adenocarcinomas and extrahepatic bile duct
squamous cell carcinomas are rare. We report here a case of choledochal squamous cell carci-
noma in a young woman who underwent surgery and chemotherapy. The woman presented with
abdominal discomfort. A physical examination showed tenderness in the upper abdomen.
Laboratory tests showed elevated direct bilirubin, total bilirubin, and C-reactive protein levels.
Abdominal computed tomography and magnetic resonance imaging showed a cystic-solid mixed
soft tissue mass in the common bile duct. Pain symptoms in the patient were not relieved and
surgical treatment was performed. Postoperative pathological results showed a choledochal cyst
complicated by squamous cell carcinoma. The patient was treated by biliary intestinal anastomo-
sis followed by chemotherapy. However, the patient developed liver metastasis and recurrence at
a 6-month follow-up. Primary congenital bile duct cysts with squamous cell carcinoma are
extremely rare. Surgical resection is the main treatment option for choledochal squamous cell
carcinoma. Postoperative chemoradiotherapy can be used, but the efficacy is poor and chemo-
therapy does not significantly prolong the patient’s survival.

1
Department of Radiology, Gansu Provincial Hospital,
Lanzhou, China
2
Department of Surgery, Gansu Provincial Hospital,
Lanzhou, China *These authors contributed equally to this work.
3
GE Healthcare China, Daxing District, Beijing, China Corresponding author:
4
Department of Radiology, Beijing Children’s Hospital Gang Huang, Department of Radiology, Gansu Provincial
Affiliated to Capital Medical University, Beijing, China Hospital, No. 204 Donggang West Road, Lanzhou
5
Gansu University of Traditional Chinese Medicine, 730000, China.
Lanzhou, China Email: keen0999@163.com

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2 Journal of International Medical Research

Keywords
Bile duct, carcinoma, squamous cell, chemotherapy, choledochal cyst, liver metastasis
Date received: 14 January 2020; accepted: 8 July 2020

Introduction a soft abdomen overall, no rebound pain,

and an obvious abdominal mass. Upon
Congenital choledochal cysts are a type of
admission, liver function and blood tests
choledochal cyst or fusiform ectasia, and it
showed the following: serum alanine amino-
is a common congenital anomaly.
transferase level, 5 IU/L (normal range, 5–40
Congenital choledochal cysts mainly occur
IU/L); aspartate aminotransferase level, 28
in children, where 80% of cysts are usually
IU/L (8–40 IU/L); alkaline phosphatase
diagnosed before the age of 10 years.1 As a
level, 750 IU/L (40–110 IU/L); direct bilirubin
result of increasing awareness of this rare
level, 21.3 lmol/L (1.71–7 lmol/L); total bili-
disease, the number of adult cases has
rubin level, 35.1 lmol/L (1.71–17.1 mmol/L);
increased. The prevalence rates of choledo-
C-reactive protein level, 172.78 mg/L (0.4–5.2
chal cyst in Western and Asian populations
are 1/13,000 and 1/100,000, respectively.2,3 mg/L); blood routine hemoglobin level, 104 g/
In this report, we describe an extremely rare L (110–150 g/L); lymphocyte count, 1.3  109
case of a primary congenital choledochal cells/L (1.1–3.2  109 cells/L); white blood cell
cyst with squamous cell carcinoma. This count, 10  109 cells/L); and international
patient underwent exploratory laparotomy, normalized ratio, 1.19 mm/hour (0–15 mm/
choledochoenterostomy, and enteroenter- hour). The levels of the tumor antigens carci-
ostomy, followed by chemotherapy, but noembryonic antigen and alpha-fetoprotein
had recurrence and metastasis within 6 were within the normal range. The patient
months, which indicated that chemotherapy provided signed informed consent for proce-
had no effect on this disease. dures to be performed.
Abdominal ultrasonography showed a
tumor of the common bile duct cyst,
Case report which was located in the left wall of the
A 32-year-old woman of Han ethnicity common bile duct. Multiphase computed
developed abdominal discomfort 6 months tomography (CT) showed common bile
before admission to our hospital, with duct dilatation with a diameter of 7 cm.
the symptoms worsening after eating. The Enhanced CT showed that the soft tissue
patient presented with discomfort of the tumor was unevenly enhanced during the
back, nausea, and abdominal pain after arterial phase (Figure 1a). Magnetic
eating. She experienced no chills, fever, resonance imaging (MRI) displayed a
vomiting, jaundice, or weight loss, and vis- solid lesion, which was located in the left
ited a local secondary clinic. A large chol- lateral wall of the cyst, and showed
edochal cyst was detected by endoscopic uniform low- and high-signal intensities on
retrograde cholangiopancreatography. She T1- (T1WI) and T2- (T2WI) weighted
was subsequently referred to our hospital images, respectively (Figure 1b).
for surgery. A physical examination Moreover, MRI showed high-signal inten-
showed tenderness in the upper abdomen, sity on diffusion-weighted imaging, and
Wang et al. 3

Figure 1. a: Magnetic resonance imaging shows a soft tissue tumor mass that is enhanced during the
arterial phase. Blood vessels can be seen in the lesion (orange arrow). b: A solid lesion is located in the
lateral wall of the cyst, showing equal signal intensity on the anti-lipid sequence. The cystic component
(indicated by the orange arrow) is hyperintense. c: The orange arrow indicates an iso-T2 signal in the cystic
lesion. The extrahepatic bile duct shows dilatation. d: The focus recurred, and new lesions can be seen in the
caudate lobe of the liver (orange arrow).

low-signal intensity on the apparent diffu- but the patient’s symptoms were not
sion coefficient map. Additionally, ribbons relieved. Therefore, an operation was car-
of high- and low-signal intensities were ried out. Three days after admission, three
found on T1WI and T2WI, respectively, surgeons performed exploratory laparoto-
in the cyst (Figure 1c). The case was diag- my, choledochoenterostomy, and enteroen-
nosed and analyzed by two deputy chief terostomy under general anesthesia.
doctors. The diagnosis was considered as Perioperatively, common bile duct expan-
a choledochal cyst that was complicated sion was observed in the upper section,
by a tumor. No metastatic signs were with a solid, qualitatively hard tissue mass
found when craniocerebral MRI, chest X- in the lower section and it was tightly
ray, abdominal CT, MRI, and other exami- adherent to the surrounding tissue.
nations were performed. A biopsy from the tumor was pathological-
Oxycodone and acetaminophen tablets ly analyzed. The common bile duct was
were administered orally before surgery, resected, and the jejunum with a distance
4 Journal of International Medical Research

treatment was 21 days, with an interval of

3 weeks after the next course of treatment,
and this lasted for six courses.
The patient was followed up for 6
months and abdominal CT showed recur-
rence. There were new lesions in the caudate
lobe of the liver, which indicated liver
metastasis (Figure 1d).

Discussion
Squamous cell carcinoma is also referred to
as epidermoid carcinoma. The majority of
cases of cholangiocarcinoma are hepatic
hilar cholangiocarcinomas. More than
Figure 2. Histopathology shows abnormal cells 90% of extrahepatic cancers involve adeno-
with large nuclei, deep staining, and irregular nuclei carcinomas.4 However, primary squamous
in the stained section. There is also small nucleoli, cell carcinoma of the extrahepatic bile
red staining of the cytoplasm, and inflammatory cell duct is rare, and only 2% of biliary carci-
infiltration (hematoxylin and eosin staining; original nomas are of squamous cell origin,5 with
magnification,  100). only a few cases reported in approximately
100 years.6–8
Kohno et al.9 suggested that squamous
of 50 cm from the Treitz ligament was cut at
cell carcinoma is derived from adenocarci-
the proximal end. Choledochal jejunostomy noma. However, Cabot et al.10 developed a
was then performed and the biliary tract theory suggesting that inflammatory stimu-
was reconstructed. No obvious enlarged lation is the main cause of metaplasia in
lymph nodes were found. A pathological epithelial mucous membranes. Sewkani
examination of the excised tissue showed et al.11 proposed that squamous cell carci-
that the squamous cell carcinoma had scat- noma is caused by choledochal cysts and
tered distribution of heteromorphic epithe- sclerosing cholangitis. In previously
lial cells. These cells had an irregular reported cases of a primary congenital bile
karyotype, and were clearly split with duct cyst with squamous cell carcinoma,
overt nucleoli, visible pathological fission, some patients had coexisting congenital
an abundant cytoplasm, and visible inter- choledochal cysts and/or bile duct stones.
cellular bridges, but no keratin pearls Therefore, “inflammation–cancer” trans-
(Figure 2). The patient was diagnosed formation might occur in patients with con-
with squamous cell carcinoma of the bile genital choledochal cysts or stones, and a
duct. There were no lesions in the patient’s few cases could develop primary squamous
lungs, gastrointestinal tract, or uterus. cell carcinoma of the bile duct.12
Therefore, primary squamous cell carcino- MRI features depend on the composi-
ma of the bile duct was diagnosed by histo- tion of the tumor. After injection of gado-
pathology. The patient received linium diethylenetriaminepentaacetic acid
postoperative chemotherapy with gemcita- contrast agent, most tumors show low-
bine (1000 mg/m2) and cisplatin and high-signal intensities on T1WI and
2
(75 mg/m ). Based on the patient’s body T2WI, respectively. Additionally, some
surface area (1.8 m2), the first course of masses display low-signal intensity on
Wang et al. 5

T2WI, high-signal intensity mainly on Our findings suggest that this type of
T1WI, or mixed signal intensities on both cancer can originate from congenital cystic
T1WI and T2WI. The main points of diag- disease. This type of cancer shows a high
nosis of congenital choledochal cyst with degree of malignancy, and chemotherapy
squamous cell carcinoma include the fol- does not significantly prolong the patient’s
lowing. First, this disease is more common survival.
in children and young individuals. Second,
if a choledochal cyst is complicated by a Declaration of conflicting interest
solid mass and the patient is younger than
The authors declare that there is no conflict of
20 years, carcinogenesis is highly likely.
Third, a solid mass is seen on the wall of interest.
the choledochal cyst, and shows high-signal
intensity on DWI. Fourth, a choledochal Ethics statement
cyst complicated by stones easily becomes Written informed consent was obtained from the
cancerous. patient for publication of this case report and its
This type of carcinoma shows a high accompanying images. All procedures performed
degree of malignancy, widespread destruc- were in accordance with the ethical standards of
tion, and lymph node involvement through the institutional and national research commit-
lymphatic metastasis around the bile duct, tee. This study was approved by the Ethics
Committee of Gansu Provincial Hospital (No.
which may lead to systemic metastasis. CT
2019-086).
and MRI scans are helpful for determining
relevant characteristics of these tumors,
including nidus number, position, and inva- Funding
sion of surrounding tissues. In case a lesion This study was supported by a grant from Gansu
is suspected to be malignant, a biopsy or Province People’s Hospital of China (Number:
even surgical resection should be per- 16GSSY1-7).
formed. Although prognosis of choledochal
squamous cell carcinoma may be unfavor- ORCID iDs
able, these approaches are considered the Lili Wang https://orcid.org/0000-0003-4576-
best treatment options. The degree of 3776
malignancy of this disease is high, and the Gang Huang https://orcid.org/0000-0001-
prognosis of patients with incomplete oper- 6057-5349
ation or metastasis is poor. Recurrence and
metastasis occurred 6 months after the References
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