Right Atrial Invasion by Metastatic Esophageal Adenocarcinoma

CASE REPORT
Right Atrial Invasion by Metastatic Esophageal
Adenocarcinoma with Direct Connection to Liver
Ali Zohair Nomani and Kaleem Ullah Toori
ABSTRACT
Common causes of right sided intra-cardiac atrial masses include primary cardiac tumors (atrial myxoma), atrial thrombus,
tumor thrombus with hepatocellular or other thoracoabdominal cancers and metastatic lesions. Invasion of atria by
gastrointestinal tumors is rare and that with esophageal ones seldom observed. Esophageal cancers rather present with
dysphagia, odynophagia or systemic symptoms. Due to the lack of a serosal layer, esophageal tumors usually spread
early in their course. Typical sites of spread include liver, gut, mediastinum, lungs and draining lymph nodes. We report a
case of metastatic esophageal adenocarcinoma presenting with direct extension of metastatic tumor thrombus from liver
to right atrium via inferior vena cava.
Key Words: Metastasis. Liver. Right atrium. Esophageal cancer. Adenocarcinoma. Tumor thrombus.
INTRODUCTION
Esophageal cancer is the eighth most common cancer
in the world and ranks sixth among all cancers in
mortality comprising 7% of all gastrointestinal cancers.
Historically reliable records marking the discovery of
esophageal carcinoma date well back to the beginning
of the 19th century.1-4 Lack of a serosa layer in the
esophagus and the location of this conduit in a very
narrow mediastinal space allows early tumor invasion
into neighboring organs such as trachea, bronchus,
lung, liver and aorta.3 Metastasis of esophageal
carcinomas into the liver has been well recognized but to
our observation, direct extension of metastatic lesions
from liver into the right atrium has never been explained
before.
We report a case of metastatic esophageal adenocarcinoma presenting with direct extension of metastatic
tumor thrombus from liver to right atrium via inferior vena
cava (IVC).
CASE REPORT
A 42 years gentleman presented to outpatient department of KRL Hospital with complaints of dyspepsia,
epigastric discomfort, nausea, grade 1 dysphagia and
more than 10 kg weight loss in the last 2 months. He had
been a chain smoker with more than 40 pack years
history of cigarette smoking but non-alcoholic. He was a
driver by profession. His family history was significant
only for ischemic heart disease. Upon examination, he
Department of Medicine, KRL Hospital, Islamabad.
Correspondence: Dr. Ali Zohair Nomani, House No. 783,
Block-B, Street 4, Sector O-9, National Police Foundation,
Loi Bher, Islamabad.
E-mail: [email protected]
Received: April 18, 2013; Accepted: December 09, 2013.
was cachexic, pale, dehydrated and had a weight of 54
kg. His systemic examination did not reveal any
significant clinical signs. Upon initial investigations, he
had hemoglobin of 9.8 g/dl and had normocytic normochromic anemia. He had a raised ESR (erythrocyte
sedimentation rate) of 70 mm in first hour. Rest of
the laboratory tests including liver function tests,
renal functions tests, neutrophil and platelet count,
coagulation profile, serum albumin, serum electrolytes,
chest radiograph and ultrasound abdomen and pelvis
were all normal. He was advised a diagnostic upper GI
(gastrointestinal) endoscopy as part of further workup.
It revealed an irregular pedunculated friable growth at
the gastroesophageal junction commencing at 35 cm
and extending up to 39 cm from the upper central
incisors (Figure 1A). A punch biopsy from the lesion
revealed moderately differentiated adenocarcinoma with
basal palisading (Figure 1B) and accompanying areas of
necrotic tissue (Figure 1C). A subsequent endoscopic
ultrasound showed extramural extension of the mass
lesion. In order to stage the disease, non-contrast and
contrast enhanced abdominal CT (computed tomography) were performed. The CT showed hypodense
lesions on non-contrast imaging. Contrast CT showed
early arterial enhancement after intravenous contrast
and characteristic delayed (portal) venous washout
suggesting metastasis (Figure 1D). On CT, a welldefined polypoidal mass was also delineated arising
from the liver. The mass was followed and seen to be
extending into right atrium of heart via IVC (Figure 1E).
The mass lesion was seen to be extending half way
across into the right atrium on transthoracic echocardiography. It was a mobile circular structure
measuring 5.3 x 5.6 cm2 in size and mimicked atrial
myxoma. Transesophageal echocardiography confirmed
the presence of mass inside the right atrium but the
patient did not have any cardiovascular signs or
Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (Special Supplement 2): S109-S111
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Ali Zohair Nomani and Kaleem Ullah Toori
DISCUSSION
Esophageal cancer is the eighth most common cancer
in the world. Although significant advancements have
been made in the treatment of esophageal cancer, this
aggressive malignancy commonly presents as locally
advanced disease with a dismal prognosis.1,4 Despite
improvements in the detection of premalignant
pathology, newer preventive strategies, and the
development of more effective combination therapies,
the overall incidence of esophageal carcinomas has
risen.5 The most common histological types are
squamous cell carcinoma and adenocarcinoma, which
together constitute more than 90% of esophageal
malignancies.5 Progressive dysphagia or odynophagia
are the most common presenting complaints of patients
with esophageal cancer.5 The lack of a serosa layer in
the esophagus and the location of this conduit in a very
narrow mediastinal space allows early tumor invasion
into neighboring organs such as trachea, bronchus,
lung, and aorta.3
Figure 1: Esophageal cancer with hepatic metastasis and right atrial
invasion by tumor thrombus.
(1A) Endoscopic view of esophageal mass showing an irregular,
pedunculated lesion at the lower end of esophagus.
(1B) Histopathological view of esophageal mass showing basal palisading
(white arrow head), glandular appearance with intervening fibrotic tissue
(black arrow head) typical of adenocarcinoma (hematoxylin and eosin
staining - 40 x objective).
(1C) Histopathological view of esophageal mass showing necrotic tissue
(black arrow head) and cellular infiltrate (white arrow head), (hematoxylin
and eosin staining - 40 x objective).
(1D) CT Abdomen showing hepatic metastatic lesion with early arterial
enhancement (white arrow head). The lesion showed delayed venous
washout in portal phase.
(1E) CT abdomen showing tumor thrombus extending from left lobe of liver
(black arrow head) into the inferior vena cava (white arrow head).
(1F) Trans-esophageal echocardiography showing circular mass lesion in
right atrium of heart (white arrow head).
symptoms. The lesion was traced back up to the left lobe
of liver with IVC as conduit and defined as tumor
thrombus (Figure 1F). The patient did not show any
cardiac or respiratory symptoms afterwards as well as
on follow-up.
Keeping in view the grave prognosis of stage IV
esophageal carcinoma and essentially non-curable
stage of disease, the patient was immediately put on
combination chemotherapy with epirubicin, cisplatin and
5-fluorouracil (ECF) with the aim to improve survival and
achieve palliation of symptoms. Upon consensus, it was
decided to conservatively treat the intra-cardiac lesion
rather than surgery in the light of otherwise poor
prognosis of the primary tumor. The patient was started
on prophylactic dose of heparin to reduce the risk of
pulmonary embolism secondary to tumor thrombus. He
did well with palliation of symptoms and presented in
reasonably good health on follow-up after 60 days.
S110
Computed tomography is presently the standard
procedure for the detection of distant metastases in
patients with esophageal cancer.6 For inoperable
tumors, cisplatin with fluorouracil remains the most
commonly used regimen, and the combination has been
shown to be an integral part of primary management of
patients with locally defined disease, as well as
palliation.5 High response rates have been reported in
the treatment of advanced gastric cancer with epirubicin,
cisplatin and continuous infusion 5-fluorouracil, including
instances of unresectable disease being rendered
operable by chemotherapy.7
Common causes of right sided intra-cardiac atrial
masses include primary cardiac tumors (atrial myxoma),
atrial thrombus, tumor thrombus with hepatocellular or
other thoracoabdominal cancers and metastatic
lesions.8 Invasion of atria by GI tumors is rare and
particularly that with esophageal carcinoma are seldom
observed.6 Metastasis of esophageal carcinomas into
the liver has been well recognized but to our
observation, direct extension of metastatic lesions from
liver into the right atrium has never been explained
before. We report a case of metastatic esophageal
adenocarcinoma presenting with direct extension of
metastatic tumor thrombus from liver to right atrium
via IVC.
Treatment of choice in patients with mobile thrombi in
the right heart chambers is still controversial owing to
the increased risk of recurrent pulmonary embolism.
Thrombolysis and surgical or catheter embolectomy are
the preferred options.9 However, anticoagulation with
heparin has been shown to be a successful treatment
option with the aim to reduce the risk of pulmonary
embolism and can be the treatment of choice in
otherwise inoperable cases.9,10 This report further
Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (Special Supplement 2): S109-S111
Right atrial invasion by metastatic esophageal adenocarcinoma
supports the conservative approach towards treatment
of intra-atrial tumor thrombus in selective patients.
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